Unrecognized Incomplete Cervical Spinal Cord Injury: Review of Nine New and 28 Previously Reported Cases JOSEPH M. BICKNELL,
MD,* KURT FIELDER, MU-
Nine patients with unrecognized incomplete cervical spinal cord injury are discussed. Four were sent home as normal, three were called hys terieal, and two went undiagnosed during stupor or coma. A literature search revealed 28 additlonal cases. Eighty percent of these were males, two thirds were over 50 years old, and most had central or posterior cord syndromes. Falls with hyperextension, spondylosls, or disc disease, and motor vehicle accidents were the most common causes. Only one of the 37 had a cervical fracture. In some the neurologic problem was missed altogether: in others it was attributed to hysteria, intoxication, or to other neurologic or systemic diseases. Minor injuries without cervical fracture or dislocation, advanced age, unusual or changeable neurologic deficits, intoxication, and psychiatric problems all contributed to the confusion. (Am J Emerg Med 1992; 10:336-343. This is a US government work. There are no restrictions on its use. One having dislocation in a vertebra of his neck, while he is unconscious of his two legs and his two arms, and his urine dribblesSmith Surgical Papyrus, an ailment not to be treated .-Edwin Egypt, 2,500-3,000 BC’
The poor prognosis in severe spinal cord injury (SCI) known 5,000 years ago still holds true; if there is no motor or sensory function 48 hours after injury the diagnosis is easy, but there is almost never any meaningful recovery.’ In 25% to 30% of cases, however, there is incomplete cervical SC1 (IC-SCI),3*4 with deficits ranging from nearly complete to barely perceptib1e5; in these cases the prognosis is good, but the diagnosis is easily missed. We have seen nine patients with IC-SC1 whose deficits went unrecognized, and we have identified 28 similar cases from earlier reports. G” We present the details of these cases, review the features of IC-SCI, discuss the factors involved in the diagnostic difficulty, and offer suggestions for the recognizing and managing patients with this reversible form of SCI.
The first two were seen several years earlier; the last seven were seen within the past year. We reviewed our nine cases of unrecognized SCI, looking at the age and sex of the patients, the inciting event, the mechanism of spinal injury, the radiographic findings, the clinical syndrome of neurologic damage, the factors contributing to the diagnostic error, and the types of incorrect diagnosis or management that ensued. In addition, we reviewed previously published reports of IC-SCI, identified 28 similar cases in which the neurologic deficit was overlooked or misdiagnosed, and subjected these case reports to the same analysis. RESULTS The findings in our nine cases are presented below, along with the features of 28 similar cases identified in a review of earlier publications. The highlights of these 37 cases are summarized in Table 1. Case Reports Patient no. I A Sf-year-old man fell while intoxicated, striking his head against a car and hyperextending his neck. Though he complained of weakness and needed help to walk, he was sent home from the emergency department as “intoxicated”. The following day he returned, and was again dismissed, this time as “hysterical”. Evaluation in another hospital 3 days later revealed weakness of moderate degree in all extremities. Tendon reflexes were reduced in the arms and increased in the legs, and the plantar responses were extensor. Sensory and bladder functions were normal. Spine films showed cervical spondylosis and stenosis, but no fracture or dislocation. He was treated conservatively and made an essentially full recovery over the next month.
METHODS
Patient no. 2
Our cases were seen in the course of day to day patient care and do not reflect a systematic review of hospital files.
A 57-year-old alcoholic had transient quadriparesis from a diving accident at age 37, and anterior cervical fusion for radiculopathy at age 41. He fell while drinking and complained he could not move. He was seen to move at the shoulders and elbows, was diagnosed as “hysterical”, and was admitted to the psychiatric department, where he was found to be quadriplegic with a CS motor and sensory level, sacral sensory sparing, and urinary retention. Radiographic studies showed severe spondylosis, a U-6 fusion, and a partial myelographic block at the C4-5 level with an anteriorposterior (AP) diameter of 9 mm. After 6 months of conservative treatment he could walk with a walker, but his hand muscles never improved.
From the ‘Neurology Service and Zia Spinal Cord Injury Center, Veterans Affairs Medical Center, Albuquerque, NW; and the tDeDartment of Neurology, University of New Mexico School of Medicine, Albuquerque, NM. ManuscriDt received November 26, 1991; revision accepted Februarv 24,1992. Presented in part at the annual meeting, Society of Clinical Neuroloaists. Point Clear, AL, October 1990. Address reprint requests to Dr Bicknell, Neurology Service, Veterans Affairs Medical Center, Albuquerque, NW 87108. Key Words: Spinal cord injuries, cervical vertebrae, alcoholism, aging, paraplegia, traumatology. This is a US government work. There are no restrictions on its use. 07356757/92/1004-0015$0.00/0 336
Patient no. 3 A 54-year-old “street person” was hit by a car, sustaining facial abrasions. He said he was too weak to walk or feed himself, and his skin was so painful he could not be touched. He was grandiose and
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paranoid. The trauma service noted no deficits, and cervical spine films were normal. He was transferred to the psychiatric department with the statements: “refuses to use arms”, and “no radiographic findings compatible with paralysis”. A neurologic consultant found weak arms, Medical Research Council (MRC) grade 2/5 (see Table 2) but no leg weakness. Pinprick was perceived poorly below the C4 level. Tendon reflexes were absent in the arms and normal in the legs; plantar responses were extensor. Cervical magnetic resonance imaging (MRI) showed spurring, disc protrusion, and cord compression at C4-5 with an AP diameter of 8 mm. After 7 weeks of physical therapy the patient was independent in living skills, but his arms and hands remained weak.
Patient no. 4 A 58year-old alcoholic fell down three steps and complained of weakness. “No abnormality” was seen in the emergency department and he was sent home. He was admitted to another hospital 2 days later and found to have weak arms (MRC grade 3/S proximally and l/5 distally) with reduced or absent reflexes and weak legs (MRC grade 4/5) with hyperactive reflexes and extensor plantar responses; there was a sensory deficit to pinprick below the upper chest. Cervical MRI showed congenital fusion of C4 and 5 with osteophytes, bulging discs, and canal stenosis at the C3-4 and C5-6 levels. After several months of supportive care he was discharged, ambulating with the aid of canes.
Patient no. 5 A 37-year-old man wrecked his truck while intoxicated. His cervical spine films were read as normal. He was admitted to a trauma center complaining of total paralysis and anesthesia. A physician noted a C5 sensory deficit, weakness of MRC grade O/5 in deltoids and 3/5 elsewhere in arms and legs, and reflexes which were reduced in the upper extremities and brisk in lower extremities. He described his findings as “mixed exam, inconsistent with normal radiographic studies”, and requested psychiatric and neurologic consultation. The neurologist found weakness at MRC grade 3/5 in the arms, and 4/5 in the legs, a C6-8 deficit to pinprick, and hyperactive reflexes with extensor plantar responses. The psychiatric examination revealed no evidence of conversion. Cervical MRI showed spinal stenosis from C4 to C7. A week later the patient could walk but used his hands poorly. He was transferred to a rehabilitation facility.
Patient no. 6 A 43-year-old carpenter fell from a step ladder and bruised his forehead, shoulder, and hands. He complained in the emergency department of pain and weakness in his hands, but no neurologic problem was noted and he was told his hands were “only bruised”. The pain subsided, but weakness persisted; 4 months later an orthopedic surgeon noted weak hand muscles and diagnosed “peripheral neuropathy”. X-rays of the cervical spine and hands were normal, and MRI of the cervical spine showed only mild spondylosis. A neurologic consultant noted atrophy of hand muscles, analgesia to pinprick in the C5 to C8 dermatomes, impaired vibration and position sense in the feet, areflexia in the arms, and hyperreflexia in the legs. Nerve conduction velocity studies were normal. A year and a half later the patient remained weak in all extremities with atrophy of hand muscles, unable to manipulate small objects, and hyperreflexic with a right extensor plantar response.
Patient no. 7 A lo-year-old boy was ill for 10 days with earache, fever, and vomiting. He fell but got up and went to bed, where he was found
comatose 15 hours later, lying prone with his neck extended, and rotated. He had lost 14 pounds and had pressure sores on his knees, ear, and mandible. He had spontaneous movement. Lower extremity reflexes were hyperactive with clonus. A computed tomographic (CT) scan of the head showed no abnormality. He was ketoacidotic with a serum glucose level of 627 mg/dL and was treated with insulin. He developed renal failure and was dialyzed. On the seventh hospital day he was found to be weak in both arms, MRC grade 2/5 proximally and 3/5 distally, and hyperreflexic in all extremities. He complained of painful dysesthesia in the arms. Electromyography revealed fibrillation potentials in the right arm and cervical paraspinous muscles. Cervical MRI revealed prevertebral soft tissue swelling consistent with edema or hematoma. His neck was immobilized, and he received physical therapy, but his neurologic deficits persisted, and he was discharged to outpatient physical therapy.
Patient no. 8 A 22-year-old man wrecked his car and was thrown from the vehicle. He was found obtunded with a frontal scalp laceration and a blood alcohol level of 1% mg/dL, but he moved all extremities, and his head CT and lateral cervical spine films were read as normal. Left hemiparesis was noted 12 hours later, and two further head CT scans were normal. On the ninth day after his injury he had bilateral Babinski responses, “poor coordination” on the right, and weakness on the left, and 15 days postinjury bilateral weakness and atrophy of the hand and forearm was noted. Cervical MRI revealed multiple abnormalities at the C5-6 level, including subluxation, canal narrowing, disc hemiation, and a fracture of the right articular complex. The C5 and C6 vertebrae were fused posteriorly 3 weeks after the initial injury. He improved in physical therapy, and was discharged to outpatient treatment.
Patient no. 9 A 64-year-old man was seen for transient left monocular blindness, and was noted to have weakness (MRC grade 2/5) and atrophy of hand and forearm muscles bilaterally, which came on after neck extension for anesthesia 10 years previously. It had never been explained. He could not handle buttons, and he needed pliers to grip objects firmly. Strength in proximal arm muscles and in the legs was normal, and reflexes were normal. He had slight impairment of light touch and two-point discrimination in the hands, but all other sensory functions were normal. He had a history of transient weakness in the right arm and leg at age 12 after he fell while carrying his brother on his shoulders and “twisted his neck”. Electromyogram showed fibrillation and reduced recruitment in CS-Tl muscles of the hands and forearms bilaterally. Magnetic resonance imaging of the cervical spine showed spinal stenosis at the C5-6 level with a lo-mm AP diameter, posterior osteophytes, and disc bulging.
Cases Previously Reported Hopkins and Rudge6 described seven patients with hyperextension injuries and damage to the center of the spinal cord, six of whom had severe pain in the shoulders and arms. One complained bitterly of a sunburn sensation, refused to wear his pajama top, and left the hospital after an altercation with the nurses. His SC1 was not recognized until a followup visit 11 weeks later. A second patient was briefly quadriplegic after a fall but regained motor power and complained of pain in his shoulders. These were radiographically normal
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TABLE1. Features of Cases with Unrecognized
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Spinal Cord Injury
Spinal
Cord Syndrome
Contributing Factor
Diagnostic Error
Author
Year
Age/Sex
Etiology
Hopkins and Rudge’
1973
32/M
Fall, ? hyperflexion (occipital laceration)
No fracture
CCP with hyperpathia and Lhermitte sign
Hopkins and Rudge6
1973
45/M
Fall on back
No fracture
Hardy’
1977
No details
No fracture
Alcohol
Sent home as intoxicated
Maxted and Dowda
1982
No details, 6 cases 52/M
Transient quadriparesis, CCP with hyperpathia, and Lhermitte sign ?CCS?
No fracture
ccs
Alcohol
Deficits
Maxted and DowdB
1982
72/M
No fracture; spondylosis
ccs
1982
78/F
No fracture; spondylosis
Anterior
Elderly, changing clinical picture Elderly, hip pain
Sent home, deficit unrecognized, diagnosed cerebral concussion
Maxted and Dowd’
Sche?
1983
48lM
Fall. ? hyperextension (bruised forehead) Fall, ? hyperextension (bruised forehead) Fall, ? hyperextension (bruised face) Fall, ? hyperextension (temporal abrasions)
ccs
Delayed symptoms, stiff neck
Good et aI”
1984
No details 9 cases
No fracture; spondylosis, t prevertebral soft tissue No fracture
CCP, cervical spondylotic myelopathy
Complaints “numb, clumsy hands”
Crawford and Shepherd”
1989
81/F
No fracture, spondylosis
ccs
Crawford and Shepherd”
1989
60/M
No fracture; spondylosis
ccs
Delayed symptoms Elderly Elderly
No fracture; spondylosis
ccs
Elderly
Diagnoses: multiple sclerosis, peripheral neuropathy, hysteria
No fracture; spondylosis
ccs
Elderly
Diagnoses:
stroke, TIA
Elderly
Diagnosed:
hysteria
ccs
Elderly
myocardial
ccs
Elderly
Diagnoses: stroke Diagnosed:
ccs
Alcohol
Sent home as intoxicated, home as hysteria
Subtotal
Alcohol
Diagnosed:
CCS, CCP with hyperpathia
Psychiatric disorder, extreme sensory complaints
Diagnosed: conversion or malingering “no radiographic findings compatible with paralysis.”
Crawford and Shepherd”
70/M
Crawford and Shepherd”
72/F
Crawford and Shepherd”
Crawford and Shepherd” Crawford and Shepherd”
1989
60/M
75/M
Spondylosis and/or disc, high cervical levels Fall, hyperextension Fall, ? hyperextension (facial laceration, fractured jaw) Fall, ? hyperextension (facial injury) Fall, ? hyperextension (bruised forehead) Fall, ? hyperextension (forehead laceration) Fall
1989
70/F
Current series
1991
52/M
Current series
1991
57/M
Fall
Current series
1991
54/M
Hit by car, ? hyperextension (facial abrasion, frontal hematoma)
Fall, fractured pelvis and humerus Fall, hyperextension
IlljUry
No fracture; spondylosis
No fracture; spondylosis No fracture; spondylosis
No fracture; spondylosis; stenosis No fracture: spondylosis, stenosis, old fusion No fracture: spondylosis, stenosis
(TABLE 1 continued on next page)
Alcohol, extreme sensory complaints, no major deficit Only sensory complaints
Neurologic deficit unrecognized. Conflict with nurses over symptoms. Went home.
Sent home, deficit
of
attributed
unrecognized
to intoxication
Diagnosed
hip fracture
Diagnosed
meningitis
or hysteria
Diagnoses: peripheral neuropathy, carpal tunnel syndrome, subacute combined degeneration, myopathy, alcoholic cerebellar degeneration Diagnosed Guillain-Barre syndrome Diagnosed
hysteria
infarct,
stroke
sent
hysteria
BICKNELL AND FIELDER n CERVICAL
TABLE 1.
Features of Cases with Unrecognized
Author
Year
Age/Sex
Etiology
Current
series
1991
66/M
Fall
Current
series
1991
37/M
Motor vehicle accident
Current
series
1991
43/M
Current series
1991
10/M
Current
series
1991
22/M
Current
series
1991
64/M
Fall, ? hyperextension (abrasions: forehead, shoulder, and hands) Fall; prolonged coma with neck extension and rotation Motor vehicle accident, ? hyperextension, (frontal laceration) Neck extension for anesthesia
ABBREVIATION:CCS, central
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Cervical Spinal Cord Injury (Cont’d) Diagnostic Error
Alcohol
Sent home, deficit
ccs
Alcohol
No fracture: spondylosis
CCS, CCP with hyperpathia
Hand injuries, clinical picture
Diagnosed: conversion reaction, “Exam inconsistent with normal studies.” Sent home, deficit unrecognized; diagnosed: bruised hands; diagnosed: motor neuropathy
No fracture; prevertebral soft tissue injury
CCS, CCP with hyperpathia
Coma from diabetic ketoacidosis
Spinal injury unrecognized for 7 days, until coma cleared
C6 facet fracture
ccs
Stupor from head injury
Spinal cord deficits head injury
attributed
No fracture: spondylosis, stenosis
ccs
Uncertain
Spinal cord deficits for 10 years
unrecognized
unrecognized
to
cord syndrome.
Research Council Grading of
Grade 5 4 3 2 1 0
Contributing Factor
ccs
No fracture; spondylosis, stenosis, congenital fusion No fracture: stenosis
and he was sent home, where he sat immobile for 3 weeks to avoid the pain of his shirt rubbing his skin. His SC1 was first recognized 2 months later when he developed Lhermitte’s sign (an electrical sensation radiating down the spine and limbs) with neck flexion and was found to be hyperreflexic. Hardy’ reported on 141 patients who had sustained SC1 without demonstrable injury to the spinal column. In six who had been drinking at the time of their accidents, the inability to move was attributed to intoxication; they were sent home to “sleep it off”, only to be readmitted later with obvious paralysis of extremities and loss of sphincter control. Maxted and Dowd* described an additional three patients with acute central cord syndrome without bony injury in whom the spinal cord problem was initially overlooked. The first was transiently unable to move his limbs after his fall, but improved rapidly, and was sent home after skull and spine films proved negative. When he returned complaining of neck pain and weak arms he was admitted with a diagnosis of cerebral concussion, but was later found to be quadriparetic with a complete block on cervical myelography. A second patient, unable to move his limbs or pass his urine after a fall while drinking, was admitted for observation with TABLE 2. Medical Muscle Strength
Cord Syndrome
Spinal Injury
Normal strength Active movement against gravity and resistance Active movement against gravity Active movement when gravity is eliminated Only a trace of movement No movement
the deficits attributed to intoxication until he was found the next day to be genuinely quadriparetic. The third complained of hip pain after a fall and could not stand. Her problems were attributed to a fractured hip, but x-rays proved negative, and neurologic examination revealed a spastic quadriparesis. A patient described by Scher’ fell from a truck and hit his head, but had no neurologic deficit until 2 days later, when he complained of neck pain, weak limbs, and urinary retention. Meningitis was suspected, but spinal fluid was normal. His neurologic deficits were considered hysterical until spine films showed prevertebral swelling, and neurologic examination showed deficits typical of “central cord syndrome”. Good et al” reported a syndrome of “numb, clumsy hands” in 13 patients with high cervical myelopathy associated with cervical spondylosis at the C3 to C5 level. Only four were correctly diagnosed initially. The other nine had their deficits attributed not to spinal cord compression but to peripheral neuropathy, carpal tunnel syndrome, vitamin B,, deficiency, myopathy, or cerebellar degeneration, and the spinal cord origin of the deficits was not recognized until myelography demonstrated cord compression. Finally, Crawford and Shepherd” reported the cases of seven elderly patients with hyperextension injuries from falls. Three were called hysterical, three were thought to have suffered strokes, and one was diagnosed with GuillainBarre syndrome. Correct neurologic diagnosis was not made until 2 to 12 days after the initial injury. Features of Cases Eighty percent of these patients were male, and two-thirds were over 50 years old. The injuries resulted from falls in 18 patients, cervical spondylosis or disc disease in 10, and mo-
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tor vehicle accidents in three. Hyperextension, as manifested by frontal or facial injuries, was a factor in 16 cases. Only one patient had a fracture, but 25 had spondylosis, six had stenosis, and two had fused vertebrae. The clinical picture was a central cord syndrome” in 24 patients, contusio cervicalis posterior’3 in 14, and subtotal and anterior syndromes were seen in one case each.4 Alcohol intoxication contributed to the confusion in 12 cases. Old age, and the syndrome of “numb, clumsy hands” were factors in nine patients each. Three patients had severe dysesthesia, three had delayed, changing, or minimal neurologic deficits, and in three the neurologic problems were overshadowed by severe hand, neck, or hip pain. In two the deficits were not evident until recovery from coma, and psychiatric problems complicated one case. The diagnostic errors included attributing the patient’s problems to other peripheral or central nervous system disorders in 12 and six cases, respectively, or to systemic disorders in four patients. Eleven patients were sent home, seven with their cord injury unrecognized: eight were wrongly called intoxicated, and eight were mistakenly considered hysterical. DISCUSSION When the spine appears intact or there is no anesthesia, when the paralysis of motion is limited or incomplete, or the sphincters are Pearce Bainot affected, the problem becomes more difficult.ley14
Complete traumatic paraplegia and quadriplegia are relatively stereotyped and easily recognized. On the other hand IC-SC1 is much more variable. The injury may be mild and the spine films normal. The lower extremities may be completely spared, and the upper limbs only partly involved. There may be motor deficits without sensory loss, or the reverse. It is no wonder these cases cause confusion.
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This clinical diversity was well characterized in Schneider and coworkers’ report on the syndrome of acute central cervical spinal cord injury,12 which others have also described, 4.h*8,‘5-22and which we will refer to as “central cord syndrome” (CCS). Central cord syndrome is “characterized by disproportionately more motor impairment of the upper than the lower extremities, bladder dysfunction, usually urinary retention, and varying degrees of sensory loss below the level of the lesion”.‘* Schneider stressed (1) the laminated segmental organization of the spinal cord tracts, (2) the central location of the lesion, (3) the role of hyperextension and spondylosis in producing cord damage without bony injury, (4) the reversible nature of the deficits, and (5) the favorable prognosis with conservation care. ” It is important to note some exceptions to Schneider’s description: (1) IC-SC1 is not always acute, (2) the lesion is not always exactly central, and (3) though the cord may be “injured” by cervical spondylosis or disc, there is not always a history of a traumatic event. Anatomy The classic representation of motor and sensory pathways in the spinal cord has the sacral, lumbar, thoracic, and cervical fibers laminated progressively from the peripheral to the central portion of the cord, surrounding the sensory and motor neurons of the posterior and anterior horns (Figure 1). Pathology Injury to the spinal cord usually produces lesions which are most severe centrally in the grey matter of the posterior and anterior horns, and extend into the more centrally located segments of the posterior columns, lateral spinothalamic, and lateral corticospinal tracts. The lesions are hemorrhagic initially, becoming cavitary and gliotic with time, and they become larger as the severity of injury increases.23 Motor
Sensory t Posterior
Column: Impaired vibratnn. pen ll0” and llne tactlIe sense
1,
1
FIGURE 1. Cervical spinal cord anatomy and origin of deficits in incomplete cervical spinal cord injury.
01
upper
2 Lumbosacral Segment3 -of Lateral Corticospinal Tract: Spastic. hype+reflexic lower hmb paralysis wth Sablnskl Flaccid arellexlc paraiyszs wlh splnal shoch E-ladder dysfunctlan wth bilateral qury
Posterior nom: Loss of pam and tempera ture sensabon wth hyperesthesla r affected SZgml?“ts
3 Lateral Spinothalamic Tract: Loss of pam and temperature sense on opposte stde below lesnn
Cervical segments Of
Spastic. hyperreflexlc limb paralysis
P
4 Cervical Segments Posterior Column: “Numb. clumsy hands’
4 n July 1992
1
3. Anterior
Horn: tiypoton~c. arellextc, atrophic paraiys~s 01 upper limb
BICKNELL
AND FIELDER
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Varying spinal artery distributions size of the lesions.24
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CORD INJURY
may influence the site and
Signs and Symptoms The deficits in IC-SC1 reflect the interplay of pathology with anatomy. They are usually present immediately after injury and last days, weeks, or longer. The initial deficits may clear promptly, reflecting spinal cord concussion, or may not appear until hours or days after the injury, as a result of edema or ischemia.” The arms are often weak. If this is from corticospinal tract injury, there is spasticity and hyperreflexia, and recovery is often excellent. If the anterior horn cells are injured, there is hypotonia, areflexia, early atrophy, and poor recovery, especially in forearm and hand muscles.i4 Lower extremity weakness reflects corticospinal tract injury. A small lesion spares these tracts. A lesion which is large but mild causes spastic weakness and hyperreflexia, while severe corticospinal injury produces spinal shock with flaccid, areflexic legs.14 Bilateral corticospinal tract injury can impair bladder function.‘* Sensory deficits are common as well. Dorsal horn injury impairs pain and temperature sense in the cervical segments, often with pain and dysesthesia, which can be so severe that the patient is x-rayed for fractures, and will not let anything touch the skin.6*‘3 Lateral spinothalamic tract injury impairs pain and temperature sensation in the lower body, but sacral sensation is often spared. Damage to posterior columns impairs vibration, position, and tactile sense, often with sacral sparing, and if only cervical fibers are affected the patient may have “numb, clumsy hands”.” The anatomic basis of the deficits in IC-SC1 is shown in Figure 1. Syndromes These deficits are combined in syndromes which reflect the level, location, and severity of injury, but not all investigators group their patients accordingly. Guttmann26 divided all SC1 patients into only two groups-those with complete lesions who do not recover, and those with incomplete lesions who do. Electrophysiologic evidence for preserved spinal pathway function has been demonstrated in patients who have complete loss of function by clinical examination, blurring the distinction between complete and incomplete. Dimitrijevic*’ calls these patients “discomplete”. Schneider’* and others4V6*8V1s-22 called all of their IC-SC1 cases CCS, despite variations in the severity or the pattern of clinical deficits. This gives the misleading impression that these patients all have similar clinical presentations. Braakman and Penning5 subdivide the incomplete injuries into the following syndromes: (1) subtotal, with no motor function, and no sensory function except sacral sparing; (2) anterior, with complete loss of motor and spinothalamic function, but preserved posterior column function; (3) central, as classically described by Schneider; (4) Brown-Sequard, with motor and posterior column loss ipsilaterally and spinothalamic loss contralaterally; (5) contusio cervicalis posterior, with
sensory loss and hyperesthesia in the upper limbs; (6) brachial monoplegia, with lower motor neuron deficits in only one arm; and (7) root, with deficits in the territory of only one or two roots (sometimes indistinguishable from brachial monoplegia). Subtotal, anterior, and Brown-Sequard syndromes are the least common, each present in 5% or less of cases. Root or brachial monoplegia syndromes are common with fracture or dislocation, involving 19% of cases. Central cord syndrome and contusio cervicalis posterior, affecting 27% and 38% of patients, respectively, are the most frequent syndromes in patients without bony injury. We feel these syndromes are worth emphasizing because they differ so markedly from complete cervical SC1 and from one another, not only in signs and symptoms and site of injury, but also in therapeutic needs and prognosis. Mechanism of Injury The mechanism of injury in IC-SC1 varies with age. Motor vehicle or sporting accidents with fractures or dislocations are more common in children and young adults.25 Falls are more frequent in older patients,” and hyperextension, evidenced by facial injury, is a common mechanism, with cord compression between discs or osteophytes anteriorly and the infolded ligamentum flavum posteriorly. ‘****Cervical spondylosis or stenosis increase the risk of cord injury in the elderly, but severe cord injury can be seen without fracture or dislocation at any age.“‘25 Patients with cervical spondylosis or disc protrusion can also develop any of the syndromes seen in traumatic ICSCI, 10,29-31either “spontaneously”, or with minor injuries.32 Therapy In most cases supportive, nonoperative care is sufficient. Problems such as head, chest, or abdominal injury, shock, or infection may complicate the early management of ICSCI, 18,22,25and cervical fracture or dislocation require appropriate surgical care.2*6 Keeping the neck in a neutral position, providing physical and occupational therapy and bowel and bladder care, and progressively increasing physical activity are the mainstays of care.‘2~22~25 Bohlman’ found that steroid therapy increased the risk of gastrointestinal hemorrhage and did not improve outcome, but more recent reports suggest a possible role for steroids in some forms of SCI.33 Schneider” advised against surgery, citing deterioration after laminectomy, but Brodkey et alI6 and others2,‘5.‘8,34 report favorable results from anterior decompression in ICSCI, and similar benefits are described with surgery for myelopathy, spondylosis or disc.29,30 Prognosis The prognosis in IC-SC1 is generally good. In 183 patients with IC-SC1 of varying severity, Braakman and Penning’ report worsening or death in 7%, no change in 35%, and improvement in 58%. While improvement is possible even with severe loss of function35 the outcome is better in those with milder deficits5
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The recovery sequence noted by Schneider12 holds true in most cases: legs improve first, followed by return of bladder function, and improvement in the arms; the hands are the last to improve and the least likely to recover completely.‘4 Improvement is not always complete or permanent. Some patients who are recovering stabilize with persisting delicits.i6 Some patients worsen hours to days after their initial injury.’ Some make a good recovery but then deteriorate again months or years later.3.36 CONCLUSIONS A number of factors contribute to the difficulty in recognizing IC-SCI. Many patients, regardless of age, have no spinal fracture or dislocation,‘*7225 and many report only trivial injuries: a “whiplash”, a bump on the forehead, a leap onto a bed,“*” or a simple fa11.8V”The symptoms may be changeable, clearing quickly after injury,25 or coming on hours or days later. 17,2J The complaints may seem bizarre: weakness in the arms with none in the legs,12 sensory loss without weakness,‘0s’3 or painful dysesthesia.6.‘3 Patients with head injury,2 the very old,” or the very youngZS may be unable to give a history or cooperate for neurologic examination. Systemic problems such as long bone fractures, shock, or thoracoabdominal injury can overshadow the neurologic deficits.2 Patients with mental illness6~‘2~20*37 may be hard to interview or examine, and their symptoms can easily be blamed on their psychiatric disorders. Finally, alcohol intoxication both increases the risk of SC1 and impedes the neurologic evaluation.37 Bohlman2 found that the diagnosis of SC1 was delayed in one third of all cases, and Geisler et al38 reported neurologic deterioration as a result of faulty management in 3% of SC1 patients. To avoid these errors, the neck should be protected and SC1 suspected in every patient with face, head, or neck injury, every injured patient with neurologic signs or symptoms, and every patient who cannot cooperate for neurologic evaluation. Lateral films of the cervical spine should be obtained with special projections as needed to see all the vertebrae.39 Patients should be re-examined regularly for changes that would necessitate further imaging or surWhen these patients have neurologic deficits or gery. 6*15*16 complaints, they should be treated for SC1 even when the spine films are normal or the complaints seem bizarre. Understanding the anatomy, pathology, mechanisms, and neurologic deficits of IC-SCI, and observing these precautions, should improve the recognition and management of this potentially reversible neurologic disorder. The authors express their gratitude to Kirsten Shelstad for support in computerized literature research and Verna Lee for assistance in manuscript preparation.
REFERENCES 1. Howorth B, Petrie G: Injuries of the Spine. Baltimore, MD, Williams & Wilkins, 1964, pp 1-59 2. Bohlman HH: Acute fractures and dislocations of the cervical spine: An analysis of three hundred hospitalized patients and review of the literature. J Bone Joint Surg 1979;61-A:11191142 3. Bosch A, Stauffer ES, Nickel VL: Incomplete traumatic paraplegia: A ten-year review. JAMA 1971;216:473-478
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4. Shrosbee RD: Acute central cervical spinal cord syndrome: Aetiology, age incidence and relationship to the orthopaedic injury. Paraplegia 1977;14:251-258 5. Braakman R, Penning L: Injuries of the cervical spine. In Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology, vol 25. New York, NY, American Elsevier, 1976, pp 227-380 6. Hopkins A, Rudge P: Hyperpathia in the central cervical cord syndrome. J Neurol Neurosurg Psychiatry 1973;36:637-642 7. Hardy AG: Cervical spinal cord injury without bony injury. Paraplegia 1977;14:296-305 8. Maxted NJ, Dowd GSE: Acute central cord syndrome without bony injury. Injury 1982;14:103-106 9. Scher AT: Hyperextension trauma in the elderly: An easily overlooked spinal injury. J Trauma 1983;23:1068-1068 10. Good DC, Couch JR, Wacaser L: “Numb, clumsy hands” and high cervical spondylosis. Surg Neurol 1984;22:285-291 11, Crawford PM, Shepherd DI: Hyperextension injuries to the cervical cord in the elderly. Br Med J 1989;299:669-670 12. Schneider RC, Cherry G, Pantek H: The syndrome of acute central cervical spinal cord injury. J Neurosurg 1954;11;547-577 13. Biemond A: Contusio cervicalis posterior. Ned Tijdschr Geneeskd 1964;108:1333-1335 14. Bailey P: Traumatic hemorrhages into the spinal cord. Med Ret 1900;57:573-582 15. Bose B, Northrup BE, Osterholm JI, et al: Reanalysis of central cervical cord injury management. Neurosurgery 1984;15: 367-372 16. Brodkey JS, Miller CF, Harmody RM: The syndrome of acute central cervical spinal cord injury revisited. Surg Neurol 1989;14:251-257 17. Chen LS, Blaw ME: Acute central cervical cord syndrome caused by minor trauma. J Pediatr 1986;108:96-97 18. Merriam WF. Tavlor TKF. Ruff SJ. et al: A reaooraisal of acute traumatic cential cord.syndrome. J Bone Joint Surg 1986;68-B:708-713 19. Morse SD: Acute central cervical spinal cord syndrome. Ann Emerg Med 1982;11:436-439 20. Peterson DI, Altman K: Central cervical spinal cord syndrome due to minor hyperextension injury. West J Med 1989; 150:691-694 21. Rand RW, Crandall PH: Central spinal cord syndrome in hyperextension injuries of the cervical spine. J Bone Joint Surg 1962;44-A:1415-1422 22. Szymanski D, Collier R, Orr S: Central cord syndrome. Ann Emerg Med 1983;12:45-47 23. Ducker T: Experimental injury of the spinal cord. In Vinken PJ, Bruyn GW feds): Handbook of Clinical Neurology, vol 25. New York, NY, American Elsevier, 1976, pp 9-26 24. Turnbull IM, Briege A, Hassler 0: Blood supply of cervical spinal cord in man, a microangiographic cadaver study. J Neurosurg 1966;24:951-965 25. Pang D, Wilberger JN: Spinal cord injury without radiographic abnormalities in children. J Neurosurg 1982;57:114-129 26. Guttmann L: Initial treatment of traumatic paraplegia and tetraplegia. In Harris R (ed): Proceedings Symposium on Spinal Injuries. Edinburgh, Scotland, Royal College of Surgery, 1966, pp 80-92 27. Dimitrijevic MR: Clinical neurophysiological evaluation of motor and sensory functions in chronic spinal cord injury patients. In lllis LS (ed): Spinal Cord Dysfunction: Assessment. New York, NY, Oxford, 1988, pp 274-286 28. Taylor AR, Blackwood W: Paraplegia in hyperextension cervical injuries with normal radiographic appearances. J Bone Joint Surg 1948;30-B:245-248 29. Crandall PH, Batzdorf V: Cervical spondylotic myetopathy. J Neurosurg 1966;25:57-66 30. O’Connell JEA: Involvement of the spinal cord by intervertebral disc protrusions. Br J Surg 1955;43:225-247 31. O’Laoire SA, Thomas DGT: Spinal cord compression due to prolapse of cervical intervertebral disc (herniation of nucleus pulposus): Treatment in 26 cases by diskectomy without interbody bone graft. J Neurosurg 1983;59:847-853 32. Lees F, Turner JWA: Natural history and prognosis of cervical spondylosis. Br Mad J 1963;2:1607-1610
BICKNELL AND FIELDER n CERVICAL CORD INJURY
33. Bracken MB, Shepard MJ, Collins WF, et al: A randomized controlled trial of methylprednisolone or naloxone in the treatment of acute spinal cord injury. Results of the Second National Acute Spinal Cord Injury Study. N Engl J Med 1990;322:14051411 34. Young S, Tamas L, O’Laoire SA: Prolapse of a cervical disc in elderly patients with cervical spondylosis. Br Med J 1988; 293:749-750 35. Suwanwela C, Alexander E Jr, Davis CH: Prognosis in spinal cord injury with special reference to patients with motor paralysis and sensory preservation. J Neurosurg 1982;19:220227 38. Guttman L. In discussion following Shrosbee RD: Acute
343
central cervical spinal cord syndrome: Aetiology, age incidence and relationship to the orthopaedic injury. Paraplegia 1977;14: 251-258 37. Fullerton DT, Harvey RF, Klein MH, et al: Psychiatric disorders in patients with spinal cord injuries. Arch Gen Psychiatry 1981;38:1369-1371 38. Geisler WC, Wynne-Jones N, Jousse AT: Early management of the patient with trauma to the spinal cord. Med Serv J Can 1966;23:512-522 39. Scher AT: Cervical spinal cord injury without evidence of fracture or dislocation: An assessment of the radiological features. S Afr Med J 1976;50:962-965
MD,* KURT FIELDER, MU-
Nine patients with unrecognized incomplete cervical spinal cord injury are discussed. Four were sent home as normal, three were called hys terieal, and two went undiagnosed during stupor or coma. A literature search revealed 28 additlonal cases. Eighty percent of these were males, two thirds were over 50 years old, and most had central or posterior cord syndromes. Falls with hyperextension, spondylosls, or disc disease, and motor vehicle accidents were the most common causes. Only one of the 37 had a cervical fracture. In some the neurologic problem was missed altogether: in others it was attributed to hysteria, intoxication, or to other neurologic or systemic diseases. Minor injuries without cervical fracture or dislocation, advanced age, unusual or changeable neurologic deficits, intoxication, and psychiatric problems all contributed to the confusion. (Am J Emerg Med 1992; 10:336-343. This is a US government work. There are no restrictions on its use. One having dislocation in a vertebra of his neck, while he is unconscious of his two legs and his two arms, and his urine dribblesSmith Surgical Papyrus, an ailment not to be treated .-Edwin Egypt, 2,500-3,000 BC’
The poor prognosis in severe spinal cord injury (SCI) known 5,000 years ago still holds true; if there is no motor or sensory function 48 hours after injury the diagnosis is easy, but there is almost never any meaningful recovery.’ In 25% to 30% of cases, however, there is incomplete cervical SC1 (IC-SCI),3*4 with deficits ranging from nearly complete to barely perceptib1e5; in these cases the prognosis is good, but the diagnosis is easily missed. We have seen nine patients with IC-SC1 whose deficits went unrecognized, and we have identified 28 similar cases from earlier reports. G” We present the details of these cases, review the features of IC-SCI, discuss the factors involved in the diagnostic difficulty, and offer suggestions for the recognizing and managing patients with this reversible form of SCI.
The first two were seen several years earlier; the last seven were seen within the past year. We reviewed our nine cases of unrecognized SCI, looking at the age and sex of the patients, the inciting event, the mechanism of spinal injury, the radiographic findings, the clinical syndrome of neurologic damage, the factors contributing to the diagnostic error, and the types of incorrect diagnosis or management that ensued. In addition, we reviewed previously published reports of IC-SCI, identified 28 similar cases in which the neurologic deficit was overlooked or misdiagnosed, and subjected these case reports to the same analysis. RESULTS The findings in our nine cases are presented below, along with the features of 28 similar cases identified in a review of earlier publications. The highlights of these 37 cases are summarized in Table 1. Case Reports Patient no. I A Sf-year-old man fell while intoxicated, striking his head against a car and hyperextending his neck. Though he complained of weakness and needed help to walk, he was sent home from the emergency department as “intoxicated”. The following day he returned, and was again dismissed, this time as “hysterical”. Evaluation in another hospital 3 days later revealed weakness of moderate degree in all extremities. Tendon reflexes were reduced in the arms and increased in the legs, and the plantar responses were extensor. Sensory and bladder functions were normal. Spine films showed cervical spondylosis and stenosis, but no fracture or dislocation. He was treated conservatively and made an essentially full recovery over the next month.
METHODS
Patient no. 2
Our cases were seen in the course of day to day patient care and do not reflect a systematic review of hospital files.
A 57-year-old alcoholic had transient quadriparesis from a diving accident at age 37, and anterior cervical fusion for radiculopathy at age 41. He fell while drinking and complained he could not move. He was seen to move at the shoulders and elbows, was diagnosed as “hysterical”, and was admitted to the psychiatric department, where he was found to be quadriplegic with a CS motor and sensory level, sacral sensory sparing, and urinary retention. Radiographic studies showed severe spondylosis, a U-6 fusion, and a partial myelographic block at the C4-5 level with an anteriorposterior (AP) diameter of 9 mm. After 6 months of conservative treatment he could walk with a walker, but his hand muscles never improved.
From the ‘Neurology Service and Zia Spinal Cord Injury Center, Veterans Affairs Medical Center, Albuquerque, NW; and the tDeDartment of Neurology, University of New Mexico School of Medicine, Albuquerque, NM. ManuscriDt received November 26, 1991; revision accepted Februarv 24,1992. Presented in part at the annual meeting, Society of Clinical Neuroloaists. Point Clear, AL, October 1990. Address reprint requests to Dr Bicknell, Neurology Service, Veterans Affairs Medical Center, Albuquerque, NW 87108. Key Words: Spinal cord injuries, cervical vertebrae, alcoholism, aging, paraplegia, traumatology. This is a US government work. There are no restrictions on its use. 07356757/92/1004-0015$0.00/0 336
Patient no. 3 A 54-year-old “street person” was hit by a car, sustaining facial abrasions. He said he was too weak to walk or feed himself, and his skin was so painful he could not be touched. He was grandiose and
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paranoid. The trauma service noted no deficits, and cervical spine films were normal. He was transferred to the psychiatric department with the statements: “refuses to use arms”, and “no radiographic findings compatible with paralysis”. A neurologic consultant found weak arms, Medical Research Council (MRC) grade 2/5 (see Table 2) but no leg weakness. Pinprick was perceived poorly below the C4 level. Tendon reflexes were absent in the arms and normal in the legs; plantar responses were extensor. Cervical magnetic resonance imaging (MRI) showed spurring, disc protrusion, and cord compression at C4-5 with an AP diameter of 8 mm. After 7 weeks of physical therapy the patient was independent in living skills, but his arms and hands remained weak.
Patient no. 4 A 58year-old alcoholic fell down three steps and complained of weakness. “No abnormality” was seen in the emergency department and he was sent home. He was admitted to another hospital 2 days later and found to have weak arms (MRC grade 3/S proximally and l/5 distally) with reduced or absent reflexes and weak legs (MRC grade 4/5) with hyperactive reflexes and extensor plantar responses; there was a sensory deficit to pinprick below the upper chest. Cervical MRI showed congenital fusion of C4 and 5 with osteophytes, bulging discs, and canal stenosis at the C3-4 and C5-6 levels. After several months of supportive care he was discharged, ambulating with the aid of canes.
Patient no. 5 A 37-year-old man wrecked his truck while intoxicated. His cervical spine films were read as normal. He was admitted to a trauma center complaining of total paralysis and anesthesia. A physician noted a C5 sensory deficit, weakness of MRC grade O/5 in deltoids and 3/5 elsewhere in arms and legs, and reflexes which were reduced in the upper extremities and brisk in lower extremities. He described his findings as “mixed exam, inconsistent with normal radiographic studies”, and requested psychiatric and neurologic consultation. The neurologist found weakness at MRC grade 3/5 in the arms, and 4/5 in the legs, a C6-8 deficit to pinprick, and hyperactive reflexes with extensor plantar responses. The psychiatric examination revealed no evidence of conversion. Cervical MRI showed spinal stenosis from C4 to C7. A week later the patient could walk but used his hands poorly. He was transferred to a rehabilitation facility.
Patient no. 6 A 43-year-old carpenter fell from a step ladder and bruised his forehead, shoulder, and hands. He complained in the emergency department of pain and weakness in his hands, but no neurologic problem was noted and he was told his hands were “only bruised”. The pain subsided, but weakness persisted; 4 months later an orthopedic surgeon noted weak hand muscles and diagnosed “peripheral neuropathy”. X-rays of the cervical spine and hands were normal, and MRI of the cervical spine showed only mild spondylosis. A neurologic consultant noted atrophy of hand muscles, analgesia to pinprick in the C5 to C8 dermatomes, impaired vibration and position sense in the feet, areflexia in the arms, and hyperreflexia in the legs. Nerve conduction velocity studies were normal. A year and a half later the patient remained weak in all extremities with atrophy of hand muscles, unable to manipulate small objects, and hyperreflexic with a right extensor plantar response.
Patient no. 7 A lo-year-old boy was ill for 10 days with earache, fever, and vomiting. He fell but got up and went to bed, where he was found
comatose 15 hours later, lying prone with his neck extended, and rotated. He had lost 14 pounds and had pressure sores on his knees, ear, and mandible. He had spontaneous movement. Lower extremity reflexes were hyperactive with clonus. A computed tomographic (CT) scan of the head showed no abnormality. He was ketoacidotic with a serum glucose level of 627 mg/dL and was treated with insulin. He developed renal failure and was dialyzed. On the seventh hospital day he was found to be weak in both arms, MRC grade 2/5 proximally and 3/5 distally, and hyperreflexic in all extremities. He complained of painful dysesthesia in the arms. Electromyography revealed fibrillation potentials in the right arm and cervical paraspinous muscles. Cervical MRI revealed prevertebral soft tissue swelling consistent with edema or hematoma. His neck was immobilized, and he received physical therapy, but his neurologic deficits persisted, and he was discharged to outpatient physical therapy.
Patient no. 8 A 22-year-old man wrecked his car and was thrown from the vehicle. He was found obtunded with a frontal scalp laceration and a blood alcohol level of 1% mg/dL, but he moved all extremities, and his head CT and lateral cervical spine films were read as normal. Left hemiparesis was noted 12 hours later, and two further head CT scans were normal. On the ninth day after his injury he had bilateral Babinski responses, “poor coordination” on the right, and weakness on the left, and 15 days postinjury bilateral weakness and atrophy of the hand and forearm was noted. Cervical MRI revealed multiple abnormalities at the C5-6 level, including subluxation, canal narrowing, disc hemiation, and a fracture of the right articular complex. The C5 and C6 vertebrae were fused posteriorly 3 weeks after the initial injury. He improved in physical therapy, and was discharged to outpatient treatment.
Patient no. 9 A 64-year-old man was seen for transient left monocular blindness, and was noted to have weakness (MRC grade 2/5) and atrophy of hand and forearm muscles bilaterally, which came on after neck extension for anesthesia 10 years previously. It had never been explained. He could not handle buttons, and he needed pliers to grip objects firmly. Strength in proximal arm muscles and in the legs was normal, and reflexes were normal. He had slight impairment of light touch and two-point discrimination in the hands, but all other sensory functions were normal. He had a history of transient weakness in the right arm and leg at age 12 after he fell while carrying his brother on his shoulders and “twisted his neck”. Electromyogram showed fibrillation and reduced recruitment in CS-Tl muscles of the hands and forearms bilaterally. Magnetic resonance imaging of the cervical spine showed spinal stenosis at the C5-6 level with a lo-mm AP diameter, posterior osteophytes, and disc bulging.
Cases Previously Reported Hopkins and Rudge6 described seven patients with hyperextension injuries and damage to the center of the spinal cord, six of whom had severe pain in the shoulders and arms. One complained bitterly of a sunburn sensation, refused to wear his pajama top, and left the hospital after an altercation with the nurses. His SC1 was not recognized until a followup visit 11 weeks later. A second patient was briefly quadriplegic after a fall but regained motor power and complained of pain in his shoulders. These were radiographically normal
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TABLE1. Features of Cases with Unrecognized
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Spinal Cord Injury
Spinal
Cord Syndrome
Contributing Factor
Diagnostic Error
Author
Year
Age/Sex
Etiology
Hopkins and Rudge’
1973
32/M
Fall, ? hyperflexion (occipital laceration)
No fracture
CCP with hyperpathia and Lhermitte sign
Hopkins and Rudge6
1973
45/M
Fall on back
No fracture
Hardy’
1977
No details
No fracture
Alcohol
Sent home as intoxicated
Maxted and Dowda
1982
No details, 6 cases 52/M
Transient quadriparesis, CCP with hyperpathia, and Lhermitte sign ?CCS?
No fracture
ccs
Alcohol
Deficits
Maxted and DowdB
1982
72/M
No fracture; spondylosis
ccs
1982
78/F
No fracture; spondylosis
Anterior
Elderly, changing clinical picture Elderly, hip pain
Sent home, deficit unrecognized, diagnosed cerebral concussion
Maxted and Dowd’
Sche?
1983
48lM
Fall. ? hyperextension (bruised forehead) Fall, ? hyperextension (bruised forehead) Fall, ? hyperextension (bruised face) Fall, ? hyperextension (temporal abrasions)
ccs
Delayed symptoms, stiff neck
Good et aI”
1984
No details 9 cases
No fracture; spondylosis, t prevertebral soft tissue No fracture
CCP, cervical spondylotic myelopathy
Complaints “numb, clumsy hands”
Crawford and Shepherd”
1989
81/F
No fracture, spondylosis
ccs
Crawford and Shepherd”
1989
60/M
No fracture; spondylosis
ccs
Delayed symptoms Elderly Elderly
No fracture; spondylosis
ccs
Elderly
Diagnoses: multiple sclerosis, peripheral neuropathy, hysteria
No fracture; spondylosis
ccs
Elderly
Diagnoses:
stroke, TIA
Elderly
Diagnosed:
hysteria
ccs
Elderly
myocardial
ccs
Elderly
Diagnoses: stroke Diagnosed:
ccs
Alcohol
Sent home as intoxicated, home as hysteria
Subtotal
Alcohol
Diagnosed:
CCS, CCP with hyperpathia
Psychiatric disorder, extreme sensory complaints
Diagnosed: conversion or malingering “no radiographic findings compatible with paralysis.”
Crawford and Shepherd”
70/M
Crawford and Shepherd”
72/F
Crawford and Shepherd”
Crawford and Shepherd” Crawford and Shepherd”
1989
60/M
75/M
Spondylosis and/or disc, high cervical levels Fall, hyperextension Fall, ? hyperextension (facial laceration, fractured jaw) Fall, ? hyperextension (facial injury) Fall, ? hyperextension (bruised forehead) Fall, ? hyperextension (forehead laceration) Fall
1989
70/F
Current series
1991
52/M
Current series
1991
57/M
Fall
Current series
1991
54/M
Hit by car, ? hyperextension (facial abrasion, frontal hematoma)
Fall, fractured pelvis and humerus Fall, hyperextension
IlljUry
No fracture; spondylosis
No fracture; spondylosis No fracture; spondylosis
No fracture; spondylosis; stenosis No fracture: spondylosis, stenosis, old fusion No fracture: spondylosis, stenosis
(TABLE 1 continued on next page)
Alcohol, extreme sensory complaints, no major deficit Only sensory complaints
Neurologic deficit unrecognized. Conflict with nurses over symptoms. Went home.
Sent home, deficit
of
attributed
unrecognized
to intoxication
Diagnosed
hip fracture
Diagnosed
meningitis
or hysteria
Diagnoses: peripheral neuropathy, carpal tunnel syndrome, subacute combined degeneration, myopathy, alcoholic cerebellar degeneration Diagnosed Guillain-Barre syndrome Diagnosed
hysteria
infarct,
stroke
sent
hysteria
BICKNELL AND FIELDER n CERVICAL
TABLE 1.
Features of Cases with Unrecognized
Author
Year
Age/Sex
Etiology
Current
series
1991
66/M
Fall
Current
series
1991
37/M
Motor vehicle accident
Current
series
1991
43/M
Current series
1991
10/M
Current
series
1991
22/M
Current
series
1991
64/M
Fall, ? hyperextension (abrasions: forehead, shoulder, and hands) Fall; prolonged coma with neck extension and rotation Motor vehicle accident, ? hyperextension, (frontal laceration) Neck extension for anesthesia
ABBREVIATION:CCS, central
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Incomplete
Cervical Spinal Cord Injury (Cont’d) Diagnostic Error
Alcohol
Sent home, deficit
ccs
Alcohol
No fracture: spondylosis
CCS, CCP with hyperpathia
Hand injuries, clinical picture
Diagnosed: conversion reaction, “Exam inconsistent with normal studies.” Sent home, deficit unrecognized; diagnosed: bruised hands; diagnosed: motor neuropathy
No fracture; prevertebral soft tissue injury
CCS, CCP with hyperpathia
Coma from diabetic ketoacidosis
Spinal injury unrecognized for 7 days, until coma cleared
C6 facet fracture
ccs
Stupor from head injury
Spinal cord deficits head injury
attributed
No fracture: spondylosis, stenosis
ccs
Uncertain
Spinal cord deficits for 10 years
unrecognized
unrecognized
to
cord syndrome.
Research Council Grading of
Grade 5 4 3 2 1 0
Contributing Factor
ccs
No fracture; spondylosis, stenosis, congenital fusion No fracture: stenosis
and he was sent home, where he sat immobile for 3 weeks to avoid the pain of his shirt rubbing his skin. His SC1 was first recognized 2 months later when he developed Lhermitte’s sign (an electrical sensation radiating down the spine and limbs) with neck flexion and was found to be hyperreflexic. Hardy’ reported on 141 patients who had sustained SC1 without demonstrable injury to the spinal column. In six who had been drinking at the time of their accidents, the inability to move was attributed to intoxication; they were sent home to “sleep it off”, only to be readmitted later with obvious paralysis of extremities and loss of sphincter control. Maxted and Dowd* described an additional three patients with acute central cord syndrome without bony injury in whom the spinal cord problem was initially overlooked. The first was transiently unable to move his limbs after his fall, but improved rapidly, and was sent home after skull and spine films proved negative. When he returned complaining of neck pain and weak arms he was admitted with a diagnosis of cerebral concussion, but was later found to be quadriparetic with a complete block on cervical myelography. A second patient, unable to move his limbs or pass his urine after a fall while drinking, was admitted for observation with TABLE 2. Medical Muscle Strength
Cord Syndrome
Spinal Injury
Normal strength Active movement against gravity and resistance Active movement against gravity Active movement when gravity is eliminated Only a trace of movement No movement
the deficits attributed to intoxication until he was found the next day to be genuinely quadriparetic. The third complained of hip pain after a fall and could not stand. Her problems were attributed to a fractured hip, but x-rays proved negative, and neurologic examination revealed a spastic quadriparesis. A patient described by Scher’ fell from a truck and hit his head, but had no neurologic deficit until 2 days later, when he complained of neck pain, weak limbs, and urinary retention. Meningitis was suspected, but spinal fluid was normal. His neurologic deficits were considered hysterical until spine films showed prevertebral swelling, and neurologic examination showed deficits typical of “central cord syndrome”. Good et al” reported a syndrome of “numb, clumsy hands” in 13 patients with high cervical myelopathy associated with cervical spondylosis at the C3 to C5 level. Only four were correctly diagnosed initially. The other nine had their deficits attributed not to spinal cord compression but to peripheral neuropathy, carpal tunnel syndrome, vitamin B,, deficiency, myopathy, or cerebellar degeneration, and the spinal cord origin of the deficits was not recognized until myelography demonstrated cord compression. Finally, Crawford and Shepherd” reported the cases of seven elderly patients with hyperextension injuries from falls. Three were called hysterical, three were thought to have suffered strokes, and one was diagnosed with GuillainBarre syndrome. Correct neurologic diagnosis was not made until 2 to 12 days after the initial injury. Features of Cases Eighty percent of these patients were male, and two-thirds were over 50 years old. The injuries resulted from falls in 18 patients, cervical spondylosis or disc disease in 10, and mo-
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tor vehicle accidents in three. Hyperextension, as manifested by frontal or facial injuries, was a factor in 16 cases. Only one patient had a fracture, but 25 had spondylosis, six had stenosis, and two had fused vertebrae. The clinical picture was a central cord syndrome” in 24 patients, contusio cervicalis posterior’3 in 14, and subtotal and anterior syndromes were seen in one case each.4 Alcohol intoxication contributed to the confusion in 12 cases. Old age, and the syndrome of “numb, clumsy hands” were factors in nine patients each. Three patients had severe dysesthesia, three had delayed, changing, or minimal neurologic deficits, and in three the neurologic problems were overshadowed by severe hand, neck, or hip pain. In two the deficits were not evident until recovery from coma, and psychiatric problems complicated one case. The diagnostic errors included attributing the patient’s problems to other peripheral or central nervous system disorders in 12 and six cases, respectively, or to systemic disorders in four patients. Eleven patients were sent home, seven with their cord injury unrecognized: eight were wrongly called intoxicated, and eight were mistakenly considered hysterical. DISCUSSION When the spine appears intact or there is no anesthesia, when the paralysis of motion is limited or incomplete, or the sphincters are Pearce Bainot affected, the problem becomes more difficult.ley14
Complete traumatic paraplegia and quadriplegia are relatively stereotyped and easily recognized. On the other hand IC-SC1 is much more variable. The injury may be mild and the spine films normal. The lower extremities may be completely spared, and the upper limbs only partly involved. There may be motor deficits without sensory loss, or the reverse. It is no wonder these cases cause confusion.
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This clinical diversity was well characterized in Schneider and coworkers’ report on the syndrome of acute central cervical spinal cord injury,12 which others have also described, 4.h*8,‘5-22and which we will refer to as “central cord syndrome” (CCS). Central cord syndrome is “characterized by disproportionately more motor impairment of the upper than the lower extremities, bladder dysfunction, usually urinary retention, and varying degrees of sensory loss below the level of the lesion”.‘* Schneider stressed (1) the laminated segmental organization of the spinal cord tracts, (2) the central location of the lesion, (3) the role of hyperextension and spondylosis in producing cord damage without bony injury, (4) the reversible nature of the deficits, and (5) the favorable prognosis with conservation care. ” It is important to note some exceptions to Schneider’s description: (1) IC-SC1 is not always acute, (2) the lesion is not always exactly central, and (3) though the cord may be “injured” by cervical spondylosis or disc, there is not always a history of a traumatic event. Anatomy The classic representation of motor and sensory pathways in the spinal cord has the sacral, lumbar, thoracic, and cervical fibers laminated progressively from the peripheral to the central portion of the cord, surrounding the sensory and motor neurons of the posterior and anterior horns (Figure 1). Pathology Injury to the spinal cord usually produces lesions which are most severe centrally in the grey matter of the posterior and anterior horns, and extend into the more centrally located segments of the posterior columns, lateral spinothalamic, and lateral corticospinal tracts. The lesions are hemorrhagic initially, becoming cavitary and gliotic with time, and they become larger as the severity of injury increases.23 Motor
Sensory t Posterior
Column: Impaired vibratnn. pen ll0” and llne tactlIe sense
1,
1
FIGURE 1. Cervical spinal cord anatomy and origin of deficits in incomplete cervical spinal cord injury.
01
upper
2 Lumbosacral Segment3 -of Lateral Corticospinal Tract: Spastic. hype+reflexic lower hmb paralysis wth Sablnskl Flaccid arellexlc paraiyszs wlh splnal shoch E-ladder dysfunctlan wth bilateral qury
Posterior nom: Loss of pam and tempera ture sensabon wth hyperesthesla r affected SZgml?“ts
3 Lateral Spinothalamic Tract: Loss of pam and temperature sense on opposte stde below lesnn
Cervical segments Of
Spastic. hyperreflexlc limb paralysis
P
4 Cervical Segments Posterior Column: “Numb. clumsy hands’
4 n July 1992
1
3. Anterior
Horn: tiypoton~c. arellextc, atrophic paraiys~s 01 upper limb
BICKNELL
AND FIELDER
W CERVICAL
Varying spinal artery distributions size of the lesions.24
341
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may influence the site and
Signs and Symptoms The deficits in IC-SC1 reflect the interplay of pathology with anatomy. They are usually present immediately after injury and last days, weeks, or longer. The initial deficits may clear promptly, reflecting spinal cord concussion, or may not appear until hours or days after the injury, as a result of edema or ischemia.” The arms are often weak. If this is from corticospinal tract injury, there is spasticity and hyperreflexia, and recovery is often excellent. If the anterior horn cells are injured, there is hypotonia, areflexia, early atrophy, and poor recovery, especially in forearm and hand muscles.i4 Lower extremity weakness reflects corticospinal tract injury. A small lesion spares these tracts. A lesion which is large but mild causes spastic weakness and hyperreflexia, while severe corticospinal injury produces spinal shock with flaccid, areflexic legs.14 Bilateral corticospinal tract injury can impair bladder function.‘* Sensory deficits are common as well. Dorsal horn injury impairs pain and temperature sense in the cervical segments, often with pain and dysesthesia, which can be so severe that the patient is x-rayed for fractures, and will not let anything touch the skin.6*‘3 Lateral spinothalamic tract injury impairs pain and temperature sensation in the lower body, but sacral sensation is often spared. Damage to posterior columns impairs vibration, position, and tactile sense, often with sacral sparing, and if only cervical fibers are affected the patient may have “numb, clumsy hands”.” The anatomic basis of the deficits in IC-SC1 is shown in Figure 1. Syndromes These deficits are combined in syndromes which reflect the level, location, and severity of injury, but not all investigators group their patients accordingly. Guttmann26 divided all SC1 patients into only two groups-those with complete lesions who do not recover, and those with incomplete lesions who do. Electrophysiologic evidence for preserved spinal pathway function has been demonstrated in patients who have complete loss of function by clinical examination, blurring the distinction between complete and incomplete. Dimitrijevic*’ calls these patients “discomplete”. Schneider’* and others4V6*8V1s-22 called all of their IC-SC1 cases CCS, despite variations in the severity or the pattern of clinical deficits. This gives the misleading impression that these patients all have similar clinical presentations. Braakman and Penning5 subdivide the incomplete injuries into the following syndromes: (1) subtotal, with no motor function, and no sensory function except sacral sparing; (2) anterior, with complete loss of motor and spinothalamic function, but preserved posterior column function; (3) central, as classically described by Schneider; (4) Brown-Sequard, with motor and posterior column loss ipsilaterally and spinothalamic loss contralaterally; (5) contusio cervicalis posterior, with
sensory loss and hyperesthesia in the upper limbs; (6) brachial monoplegia, with lower motor neuron deficits in only one arm; and (7) root, with deficits in the territory of only one or two roots (sometimes indistinguishable from brachial monoplegia). Subtotal, anterior, and Brown-Sequard syndromes are the least common, each present in 5% or less of cases. Root or brachial monoplegia syndromes are common with fracture or dislocation, involving 19% of cases. Central cord syndrome and contusio cervicalis posterior, affecting 27% and 38% of patients, respectively, are the most frequent syndromes in patients without bony injury. We feel these syndromes are worth emphasizing because they differ so markedly from complete cervical SC1 and from one another, not only in signs and symptoms and site of injury, but also in therapeutic needs and prognosis. Mechanism of Injury The mechanism of injury in IC-SC1 varies with age. Motor vehicle or sporting accidents with fractures or dislocations are more common in children and young adults.25 Falls are more frequent in older patients,” and hyperextension, evidenced by facial injury, is a common mechanism, with cord compression between discs or osteophytes anteriorly and the infolded ligamentum flavum posteriorly. ‘****Cervical spondylosis or stenosis increase the risk of cord injury in the elderly, but severe cord injury can be seen without fracture or dislocation at any age.“‘25 Patients with cervical spondylosis or disc protrusion can also develop any of the syndromes seen in traumatic ICSCI, 10,29-31either “spontaneously”, or with minor injuries.32 Therapy In most cases supportive, nonoperative care is sufficient. Problems such as head, chest, or abdominal injury, shock, or infection may complicate the early management of ICSCI, 18,22,25and cervical fracture or dislocation require appropriate surgical care.2*6 Keeping the neck in a neutral position, providing physical and occupational therapy and bowel and bladder care, and progressively increasing physical activity are the mainstays of care.‘2~22~25 Bohlman’ found that steroid therapy increased the risk of gastrointestinal hemorrhage and did not improve outcome, but more recent reports suggest a possible role for steroids in some forms of SCI.33 Schneider” advised against surgery, citing deterioration after laminectomy, but Brodkey et alI6 and others2,‘5.‘8,34 report favorable results from anterior decompression in ICSCI, and similar benefits are described with surgery for myelopathy, spondylosis or disc.29,30 Prognosis The prognosis in IC-SC1 is generally good. In 183 patients with IC-SC1 of varying severity, Braakman and Penning’ report worsening or death in 7%, no change in 35%, and improvement in 58%. While improvement is possible even with severe loss of function35 the outcome is better in those with milder deficits5
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The recovery sequence noted by Schneider12 holds true in most cases: legs improve first, followed by return of bladder function, and improvement in the arms; the hands are the last to improve and the least likely to recover completely.‘4 Improvement is not always complete or permanent. Some patients who are recovering stabilize with persisting delicits.i6 Some patients worsen hours to days after their initial injury.’ Some make a good recovery but then deteriorate again months or years later.3.36 CONCLUSIONS A number of factors contribute to the difficulty in recognizing IC-SCI. Many patients, regardless of age, have no spinal fracture or dislocation,‘*7225 and many report only trivial injuries: a “whiplash”, a bump on the forehead, a leap onto a bed,“*” or a simple fa11.8V”The symptoms may be changeable, clearing quickly after injury,25 or coming on hours or days later. 17,2J The complaints may seem bizarre: weakness in the arms with none in the legs,12 sensory loss without weakness,‘0s’3 or painful dysesthesia.6.‘3 Patients with head injury,2 the very old,” or the very youngZS may be unable to give a history or cooperate for neurologic examination. Systemic problems such as long bone fractures, shock, or thoracoabdominal injury can overshadow the neurologic deficits.2 Patients with mental illness6~‘2~20*37 may be hard to interview or examine, and their symptoms can easily be blamed on their psychiatric disorders. Finally, alcohol intoxication both increases the risk of SC1 and impedes the neurologic evaluation.37 Bohlman2 found that the diagnosis of SC1 was delayed in one third of all cases, and Geisler et al38 reported neurologic deterioration as a result of faulty management in 3% of SC1 patients. To avoid these errors, the neck should be protected and SC1 suspected in every patient with face, head, or neck injury, every injured patient with neurologic signs or symptoms, and every patient who cannot cooperate for neurologic evaluation. Lateral films of the cervical spine should be obtained with special projections as needed to see all the vertebrae.39 Patients should be re-examined regularly for changes that would necessitate further imaging or surWhen these patients have neurologic deficits or gery. 6*15*16 complaints, they should be treated for SC1 even when the spine films are normal or the complaints seem bizarre. Understanding the anatomy, pathology, mechanisms, and neurologic deficits of IC-SCI, and observing these precautions, should improve the recognition and management of this potentially reversible neurologic disorder. The authors express their gratitude to Kirsten Shelstad for support in computerized literature research and Verna Lee for assistance in manuscript preparation.
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4. Shrosbee RD: Acute central cervical spinal cord syndrome: Aetiology, age incidence and relationship to the orthopaedic injury. Paraplegia 1977;14:251-258 5. Braakman R, Penning L: Injuries of the cervical spine. In Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology, vol 25. New York, NY, American Elsevier, 1976, pp 227-380 6. Hopkins A, Rudge P: Hyperpathia in the central cervical cord syndrome. J Neurol Neurosurg Psychiatry 1973;36:637-642 7. Hardy AG: Cervical spinal cord injury without bony injury. Paraplegia 1977;14:296-305 8. Maxted NJ, Dowd GSE: Acute central cord syndrome without bony injury. Injury 1982;14:103-106 9. Scher AT: Hyperextension trauma in the elderly: An easily overlooked spinal injury. J Trauma 1983;23:1068-1068 10. Good DC, Couch JR, Wacaser L: “Numb, clumsy hands” and high cervical spondylosis. Surg Neurol 1984;22:285-291 11, Crawford PM, Shepherd DI: Hyperextension injuries to the cervical cord in the elderly. Br Med J 1989;299:669-670 12. Schneider RC, Cherry G, Pantek H: The syndrome of acute central cervical spinal cord injury. J Neurosurg 1954;11;547-577 13. Biemond A: Contusio cervicalis posterior. Ned Tijdschr Geneeskd 1964;108:1333-1335 14. Bailey P: Traumatic hemorrhages into the spinal cord. Med Ret 1900;57:573-582 15. Bose B, Northrup BE, Osterholm JI, et al: Reanalysis of central cervical cord injury management. Neurosurgery 1984;15: 367-372 16. Brodkey JS, Miller CF, Harmody RM: The syndrome of acute central cervical spinal cord injury revisited. Surg Neurol 1989;14:251-257 17. Chen LS, Blaw ME: Acute central cervical cord syndrome caused by minor trauma. J Pediatr 1986;108:96-97 18. Merriam WF. Tavlor TKF. Ruff SJ. et al: A reaooraisal of acute traumatic cential cord.syndrome. J Bone Joint Surg 1986;68-B:708-713 19. Morse SD: Acute central cervical spinal cord syndrome. Ann Emerg Med 1982;11:436-439 20. Peterson DI, Altman K: Central cervical spinal cord syndrome due to minor hyperextension injury. West J Med 1989; 150:691-694 21. Rand RW, Crandall PH: Central spinal cord syndrome in hyperextension injuries of the cervical spine. J Bone Joint Surg 1962;44-A:1415-1422 22. Szymanski D, Collier R, Orr S: Central cord syndrome. Ann Emerg Med 1983;12:45-47 23. Ducker T: Experimental injury of the spinal cord. In Vinken PJ, Bruyn GW feds): Handbook of Clinical Neurology, vol 25. New York, NY, American Elsevier, 1976, pp 9-26 24. Turnbull IM, Briege A, Hassler 0: Blood supply of cervical spinal cord in man, a microangiographic cadaver study. J Neurosurg 1966;24:951-965 25. Pang D, Wilberger JN: Spinal cord injury without radiographic abnormalities in children. J Neurosurg 1982;57:114-129 26. Guttmann L: Initial treatment of traumatic paraplegia and tetraplegia. In Harris R (ed): Proceedings Symposium on Spinal Injuries. Edinburgh, Scotland, Royal College of Surgery, 1966, pp 80-92 27. Dimitrijevic MR: Clinical neurophysiological evaluation of motor and sensory functions in chronic spinal cord injury patients. In lllis LS (ed): Spinal Cord Dysfunction: Assessment. New York, NY, Oxford, 1988, pp 274-286 28. Taylor AR, Blackwood W: Paraplegia in hyperextension cervical injuries with normal radiographic appearances. J Bone Joint Surg 1948;30-B:245-248 29. Crandall PH, Batzdorf V: Cervical spondylotic myetopathy. J Neurosurg 1966;25:57-66 30. O’Connell JEA: Involvement of the spinal cord by intervertebral disc protrusions. Br J Surg 1955;43:225-247 31. O’Laoire SA, Thomas DGT: Spinal cord compression due to prolapse of cervical intervertebral disc (herniation of nucleus pulposus): Treatment in 26 cases by diskectomy without interbody bone graft. J Neurosurg 1983;59:847-853 32. Lees F, Turner JWA: Natural history and prognosis of cervical spondylosis. Br Mad J 1963;2:1607-1610
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33. Bracken MB, Shepard MJ, Collins WF, et al: A randomized controlled trial of methylprednisolone or naloxone in the treatment of acute spinal cord injury. Results of the Second National Acute Spinal Cord Injury Study. N Engl J Med 1990;322:14051411 34. Young S, Tamas L, O’Laoire SA: Prolapse of a cervical disc in elderly patients with cervical spondylosis. Br Med J 1988; 293:749-750 35. Suwanwela C, Alexander E Jr, Davis CH: Prognosis in spinal cord injury with special reference to patients with motor paralysis and sensory preservation. J Neurosurg 1982;19:220227 38. Guttman L. In discussion following Shrosbee RD: Acute
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