Acquired Cewical Spine Impairment in Young Adults with Cerebral

CASE REPORTS

Palsy Mary E. Reese Michael E. Msall Scott (3wen Sarah P . Pictor Margaret W . Paroski

Both Down syndrome (Pueschel et al. 1984, Elliott et al. 1988) and choreoathetoiid cerebral palsy (Anderson et al. 1962, Fuji et al. 1987, Kidron et al. 1987) have been associated with neurological deterioration secondary to acquired cervical spine pathology. In 1981 Pueschel and colleagues described laxity of the transverse ligament of the atlas in individuals with Down syndrome, allowing subluxation of the atlantoaxial joint and the possibilitgr of cord compression. Individuals with athetosis have been shown to develop severe cervical spondylosis and radiculomyelopathy, presumably from excessive rotation, flexion and extension of the head and neck, resulting in early degenerative changes of the spine (Hirose and Kadoya 1984, Nishihara et al. 1984, Kidron et al. 1987), or aggravation of myelopathy once spond ylosis has developed (Anderson et al. 1942). Knowledge of these two conditions has led to increased suspicion in the evaluation of patients with Down syndrome or athetosis who present with a loss of functional skills. Routine screening of Down patients has been recommended, with restrictions for those with atlantoaxial instability (Pueschel et al. 1984; Alvarez and Rubin 1986, 1987;

Msall et al. 1990). Careful documentation of the neurological examination for both groups has been advocated, with thorough evaluation of the cervical spine for those with new onset of or increase in spasticity, neurogenic bowel or bladder, or complaints of neck pain. A review of the literature failed to find reports of cervical spine pathology in individuals with spastic cerebral palsy who did not have associated athetosis or other movement disorders. We have recently encountered three individuals with spastic forms of cerebral palsy, who also have profound mental retardation and are nonverbal. Each came to medical attention because of concerns by their caretakers about the loss of previously acquired skills. In each case a classic neurological examination lacked the sensitivity to document changes in spasticity or to evoke sufficient concern to initiate evaluation of the cervical spine. We present these cases to increase awareness of the occurrence of acquired cervical spine disease in this population, and we suggest that the combination of a neurodevelopmental examination and an objective review of level of function provides a more precise and comprehensive tool for the evaluation of multiply handicapped young adults.

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Fig. I . CT demonstrates CSC6 disc herniation with cord compression.

Case reports

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CASE 1 A 29-year-old male with profound mental retardation, a well-controlled seizure disorder and spastic diplegia developed sudden loss of ambulation and left-sided weakness. A phenobarbital level of 30mg/dl was believed to be the cause of the loss of ambulation, and the dose was discontinued. A chart review revealed the patient had had an unrecognized, gradual increase in night-time enuresis over the previous two to three months, with deterioration of balance and sporadic episodes of disuse of the right arm. Over the next two months his incontinence progressed to daytime enuresis, previously never a problem, and to the new onset of fecal incontinence. These were attributed to ‘mental retardation’. Complete loss of ambulation and the ability to stand or bear weight ensued, with associated lethargy, anorexia and weight-loss. Neurological investigations focused on possible brain pathology, but CT and EEG evaluations were both normal. Because of continued deterioration, further neurological consultation was sought, this time with the recommendation for immediate admission to hospital for evaluation of the cervical spine. He was found to have narrowing of the C5-C6 interspace, reversal of the normal cervical lordosis, and C5-C6 disc herniation, with cord compression (Fig. 1). Discectomy and posterior spinal fusion were performed. Postoperatively the patient developed inflammation of the cord, with complete neurogenic bowel and bladder, respiratory difficulty, and paralysis at C6 and below. After discharge to a developmental disabilities residential center, he has recovered the use of his arms for self-feeding and wheelchair propulsion, but has a persistent neurogenic bladder, and spasticity of the lower extremities prevents ambulation.

Fig. 2. Cervical myelogram demonstrates obstructed flow of contrast material secondary to severe kyphosis and spinal stenosis at C3-C5.

CASE 2 A 31-year-old male with spastic quadriplegia and profound mental retardation previously had been in good health and ambulating independently. His ambulation gradually deteriorated over the space of a year, with dragging of the right leg. This was presumed to be secondary to increasing muscle contractures of the lower extremities, and he was given a rollator. Increasing constipation, anorexia and progressive change of hand dominance followed. Repeated neurological and orthopedic examinations did not lead to diagnosis, and discussion centered on the inability to document increasing quadriplegia. Review of physical therapy evaluations documented a decline in strength, loss of ambulation, new onset of incontinence and muscle atrophy. Because of these and the onset of muscle fasciculation, the patient was admitted with a presumptive diagnosis of amyotrophic lateral sclerosis. He was found to have severe, fixed kyphosis of C4-C6, with spinal stenosis at C3-C4 and C4-C5 and secondary myelopathy (Fig. 2). Discectomy of C3-C4, C4-C5 and C5-C6, with corpectomy of C3-C5 and posterior spinal fusion was performed. The patient gradually improved postoperatively and has recovered all previous functions, with the exception of independent ambulation. A forearm support rollator is required for community ambulation. CASE 3 A 33-year-old male with spastic quadriplegia, who was non-ambulatory, developed intermittent loss of hand function, evidenced by loss of independent wheelchair propulsion. CT of the head was normal. After multiple neurological evaluations failed to define the etiology of the decline, myelography was requested by the primary physician, because of

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TABLE I Summary of case findings

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Functional symptoms

Case 1

Case 2

Case 3

Time from onset to final diagnosis (rnths)

Presumed etiology

Night-time incontinence Loss of balance Loss of handedness

4 3 3

MR, cough

Loss of ambulation Daytime incontinence No weight-bearing Loss of all hand use Fecal incontinence

2

2 2 1 Y2 1

No independent ambulation Change of handedness No assisted ambulation Constipation Anorexia Urine incontinence Loss of weight-bearing Muscle atrophy Muscle fasciculations Change of handedness Loss of mobility (wheelchair propulsion)

18 11

6 6 5

4 3-4 2 1

6 4

m

?

No motivation (secondary to MR) Phenobarbital MR No motivation No motivation MR CP Arthritis CP CP MR MR No motivation ? ALS? No motivation No motivation MR

C P = cerebral palsy, MR = mental retardation, ALS = amyotrophic lateral sclerosis.

knowledge of the two previous cases. This demonstrated spinal stenosis, with cord compression at C3-C4, felt to be mild. Conservative management, with continued surveillance, was chosen rather than surgical management. Tablr: I summarizes the functional symptoms, diagnostic delay and primary medical etiological premises in these patients.

Discussion These cases demonstrate that mentally retarded individuals with spastic cerebral palsy, who present with loss of previously acquired skills, require a thorough and careful evaluation, with particular emphasis on the cervical spine. As shown in Table I, in all three cases the loss of skills was attributed to their cerebral palsy or cognitive impairments. McCluer (1982) encountered similar problems in the diagnosis of disabled persons as were experienced by these three individuals. Several factors contributed to the delay in diagnosis. The multiply handicapped adult may have numerous medical providers, with poor continuity of care. Physicians’ records of acute illness do not provide a comparative neurological examination that would be sufficiently sensitive t o

document the mild changes in tone, strength or deep tendon reflexes that may be objective evidence of myelopathy. Even if recognized, in this population such signs often do not evoke concern until they become extreme. Physicians who care for co-operative adults of normal intelligence may not be prepared to evaluate the multiply handicapped adult, who is often labelled ‘uncooperative’. A classical neurological examination requires a mental age of seven years for optimal performance, and rarely provides a comprehensive tool for evaluation of the developmentally disabled. Direct verbal communication with the patient may be difficult or impossible. Many multiply disabled people live in institutions or in custodial groups, but the caretaker, who is the best historian of the individual’s functional skills, may not be available for questioning. Even if present, this person may not be able to convey areas of concern accurately. Cervical spine spondylosis in the nondisabled population usually occurs in late 40s to early 50s (Clarke and Robinson 1956, Odom et a/. 1958), and the younger

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TABLE I1

Functional assessment Communication Prints name Speaks in full sentences Uses single words only Consistently obeys simple commands Has consistent yes/no responses

Yes :No

Hand skills Dresses self without assistance Uses fork and spoon independently Can reach for and pick up objects spontaneously Handedness

Yes :No

Right :Left

Mobility Walks independently Walks with assistive device, independently weight-bearing Independently stands and pivots for wheelchair transfer Able to propel wheelchair Sits without support

Yes :No

Continence Continent of urine and stool, day and night Daytime continence, spontaneously Daytime continence with timed void Incontinent of bowel and bladder

Yes :No

Comments on elimination pattern (severity and frequency of constipation):

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ages of our three patients (29 to 33 years) may have contributed to the delay in diagnosis. In the non-disabled population, the c5-c6 interspace is the most common site, i.e. the site of maximum flexion and extension (Clarke and Robinson 1956, Odom et al. 1958). Our three patients have difficulty with head control, often sitting with the head dropped forward, then throwing the head backward to attain an upright head position. This produces exaggerated flexion and extension, such as is seen in a whiplash injury. Spine involvement in the first two cases included The c s c 6 segment, although in the third case it was more superior (c3-c4). Cervical restraint was considered for case 3, but it was felt that only a rigid cervical collar would provide appropriate protection, which the patient could not tolerate. Providing both anterior and posterior head support without rigidly confining the person remains a dilemma for individuals with poor head control. There was an obvious need for a

documented level of function for these three patients. A functional neurological examination should include the classical neurological examination, together with an assessment of mental age and best level of function. An example of a functional assessment is shown in Table 11, which can easily be tailored to individual patients and practice situations. It is designed for individuals with a mental age of less than seven years, and it focuses on the best consistent and reproducible performance. The caretaker who is most familiar with the patient should be available for questioning. The onset of loss of functional skills should be noted, and the best skills of the patient before onset of loss should be ascertained. Questions must be asked to determine the previous level of independence; for example, after determining the individual's previous hand skills, was this ability present 90 per cent of the time, so the caretaker never had to intervene? It is crucial to note the difference that someone who once

fed independently with a spoon now requires hand-over-hand assistance. A wheelchair-bound individual who was able to stand and pivot for chair-to-chair transfers, but now cannot, has lost a significant skill. Questions need to address specific visible skills, but the assessment need not take more than a few minutes. It requires little co-operation from the patient, since information is obtained by asking caretakers the appropriate questions. Skills such as following requests, use of the hands in reaching for an interesting object, gait or sitting balance, and selffeeding can also be observed as they occur naturally, then recorded as the best level of proficiency. Gait and hand function can be documented with a video-recorder during therapy sessions, which will serve as a comparison for future evaluations. When past documented skills and present observations show a significant discrepancy, a prompt, thorough evaluation is indicated. Despite the degree of involvement of these patients, the development of complete paraplegia from acquired myelopathy would seriously affect their quality of life and increase the need for custodial intervention. Cervical myelopathy :should be considered when patients

with spastic cerebral palsy present with a loss of previously acquired functional skills. Appropriate evaluation and intervention is essential to prevent the development of further disability.

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Accepted f o r publication 26th July 1990. Acknowledgements A grant from the New York State Office of Mental Retardation and Developmental Disabilities/CMEDD Program and the Children’s Guild of the Robert Warner Rehabilitation Center helped in the preparation of this paper. These cases were presented at the 43rd Annual Meeting of the American Academy for Cerebral Palsy and Developmental Medicine, San Francisco, 25th-28th October 1989. Authors’ Appointments Mary E. Reese, M.D., Fellow in Developmental Pediatrics; *Michael E. Msall, M.D., Assistant Professor, of Pediatrics, Genetics and Developmental Medicine; State University of New York at Buffalo, Children’s Hospital of Buffalo and Robert Warner Rehabilitation Center, Buffalo. Scott Owen, M.D., Clinical Assistant Professor, Division of Developmental Medicine, Department of Family Medicine, State University of New York, Erie County Medical Center, Buffalo. Sarah P. Pictor, P.T., Physical Therapist, East Aroura, New York. Margaret W. Paroski, M.D., Assistant Professor of Neurology and Physiology, State University of New York at Buffalo, Buffalo Psychiatric Center, Buffalo. *Correspondence to second author at Robert Warner Rehabilitation Center, 936 Delaware Avenue, Buffalo, New York 14222.

SUMMARY Three patients with spastic cerebral palsy and n o associated movement disorder-each of whom presented with loss of functional skills and delay in the definitive diagnosis of cervical myelopathy-are reported, in order to increase awareness of the possibility of cervical spine pathology in these adults. The possibility of myelopathy should be investigated when considering the etiology of functional deterioration. A functional neurological examination for all multiply disabled individuals is proposed as a reference for future comparison, RESUME Atteinte acauise de la moelle PDiniere cervicale chez les ieunes adultes I.M.C. L’article rapporte le cas de tr& I.M.C. de type spastiGe, sans trouble de rnouvements associes, chacun presentant une perte des capacites fonctionn,elles et un retard dans le diagnostic d’une mydopathie cervicale, pour souligner la possibilite cl’une pathologie medullaire cervicale chez ces adultes. La possibilite d’une myelopathie doit &re evoquee lorsque I’origine d’une deterioration fonctionnelle est recherchb. Un examen neurologiqiie fonctionnel de tous les patients a incapacites multiples est propose comme reference pour une comparaison future. ZUSAMMENFASSUNG Erworbene Schadigung der Halswirbelsaule bei Jugendlichen mil Cerebralparese Es wird uber drei Patienten mit spastischer Cerebralparese ohne assoziierte Bewegungsstorung berichtet, bei denen ein Funktionsverlust auffiel, aber die endgiiltige Diagnose einer zervikalen Myelopathie erst verspatet gestellt wurde, um auf die Moglichkeit einer zervikalen Wirbelsaulenschadigung bei diesen Heranwachsenden aufmerksam zu rnachen. Die Myelopathie sollte bei der Differentialdiagnose einer funktionellen Verxhlechterung erortert werden. Fur zukunftige Vergleiche wird eine funktionelle neurologische Unti:rsuchung fur alle mehrfach behinderten Patienten angeboten.

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RESUMEN Alteracion adquirida de la columna cervical en adultos jovenes con paralisis cerebral Se aportan tres pacientes con p a r a h i s cerebral espastica sin alteracion del movimiento, que presentaban pkrdida de habilidades funcionales y retraso en el diagnostic0 definitivo de mielopatia cervical. La finalidad era el insistir en pensar en la posibilidad de una patologia de la columna cervical en estos adultos. Debe investigarse la posibilidad de una mielopatia a1 considerar la etiologia de una alteracidn funcional. Se propone un examen neurologica funcional en 10s casos de individuos con multiples minusvalencias como una referencia para futuras comparaciones. References Alvarez, N., Rubin, L. (1986) ‘Atlantoaxial instability in adults with Down syndrome: a clinical and radiological survey.’ Applied Research in Mental Retardation, 7, 67-78. Anderson, W. W., Wise, B. L., Itabashi, H. H., Jones, M. (1962) ‘Cervical spondylosis in patients with athetosis.’ Neurology, 12, 410-412. Clarke, E., Robinson, P. K. (1956) ‘Cervical myelopathy: a complication of cervical spondylosis.’ Brain, 79, 483-509. Elliott, S., Morton, R. E., Whitelaw, R. A. J. (1988) ‘Atlantoaxial instability and abnormalities of the odontoid in Down’s syndrome.’ Archives of Disease in Childhood, 63, 1484-1489. Fuji, T., Yonenobu, K., Fujiwara, K., Yamashita, K., Ebara, S., Ono, K., Okada, K. (1987) ‘Cervical radiculopathy or myelopathy secondary to athetoid cerebral palsy.’ Journal of Bone and Joint Surgery, 69A, 815-821. Hirose, G., Kadoya, S. (1984) ‘Cervical spondylotic radiculo-myelopathy in patients with athetoiddystonic cerebral palsy: clinical evaluation and surgical treatment.’ Journal of Neurology, Neurosurgery and Psychiatry, 47, 175-180. Kidron, D., Steiner, I., Melamed, E. (1987) ‘Late onset progressive radiculomyelopathy in patients with cervical athetoid-dystonic cerebral palsy.’ European Neurology, 27, 164-166. McCluer, S. (1982) ‘Cervical spondylosis with

myelopathy as a complication of cerebral palsy.’ Paraplegia, 20, 308-312. Msall, M. E., Reese, M. E., DiGaudio, K., Griswold, K., Granger, C. V., Cooke, R. E. (1990) ‘Symptomatic atlantoaxial instability associated with medical and rehabilitativ: procedures in children with Down’s syndrome. Pediatrics, 85, 447-449. Nishihara, N., Tanabe, G., Nakahara, S., Imai, T., Murakawa, H. (1984) ‘Surgical treatment of cervical spondylotic myelopathy complicating athetoid cerebral palsy.’ Journal of Bone and Joint Surgery, 66B, 504-508. Odom, G. L., Finney, W., Woodhall, B. (1958) ‘Cervical disk lesions.’ Journal of the American Medical Association, 166, 23-28. Pueschel, S. M., Scola, F. H., Perry, C. D., Pezzullo, J. C. (1981) ‘Atlantoaxial i,nstability in children with Down syndrome. Pediatric Radiology, 10, 129-132. - Herndon, J. H., Gelch, M. M., Senft, K. E., Scola, F. H. (1984) ‘Symptomatic atlantoaxial subluxation in persons with Down syndrome.’ Journal of Pediatric Orthopedics, 4, 682-688. - Findley, T. W., Furia, J., Gallagher, P. L., Scola, F. H., Pezzullo, J. C. (1987) ‘Atlantoaxial instability in Down syndrome: roentogenographic, neurologic and somatosensory evoked potential studies.’ Journal of Pediatrics, 110,

EncephaI iti s Lethargica-like Illness in a Five-year-old

1987). The prognosis of encephalitis lethargica is poor, and survivors are frequently left with permanent neurological deficits. Acute and para-infectious encephalomyelitis may also produce somnolence and, rarely, Parkinsonism, but without such a poor outcome (Tidstrram 1968). We describe a five-year-old boy with a para-infectious encephalomyelitis presenting as encephalitis lethargica, who made a complete recovery.

A . F. Mellon R . E. Appleton D. Gardner-Medwin A . Aynsley-Green

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Encephalitis lethargica (EL), a frequent cause of childhood Parkinsonism in the 1920s, is now rarely reported (Greenough and Davis 1983, Howard and Lees

515-521.

Case report This previously well five-year-old boy developed a febrile illness, with a diffuse erythematous, maculopapular rash, conjunctivitis and frontal headache. The illness resolved over three days. He was then well for four days but over the next week he became increasingly drowsy. Two days before admission he developed urinary and faecal incontinence, and stopped talking; he had slept for 42 of these 48 hours.

Acquired cervical spine impairment in young adults with cerebral palsy.

Three patients with spastic cerebral palsy and no associated movement disorder--each of whom presented with loss of functional skills and delay in the...
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