Quadriparesis

and Nuclear Oculomotor Palsy With Total Bilateral Ptosis Mimicking Coma A

Mesencephalic 'Locked-in Syndrome'?

Otmar

Meienberg, MD; Marco Mumenthaler, MD;

\s=b\ Upper lid and vertical eye movements

usually preserved in patients with the "locked-in" syndrome, a condition manifested by anarthria, spastic quadriparesis, and intact mental function, and caused by lesions in the ventral portion of the pons. We describe a presumed mesencephalic "locked-in" syndrome in a patient who had total bilateral ptosis at the onset of his disease and later on experienced complete bilateral oculomotor nerve paralyare

sis.

(Arch

Neurol

36:708-710, 1979)

"Datients with profound quadriparesis and anarthria sometimes ap¬ pear to be comatose when in fact they are awake with normal mental func¬ tion. Plum and Posner' introduced the expression "the locked-in syndrome" to describe this pseudocomatose state. Vertical eye movements and lid move¬ ments are often the which such patients

only can

means

by

communi¬

A majority of necropsyconfirmed cases have demonstrated bilateral corticobulbar and corticospinal lesions in the basis pontis.'-'" Karp and Hurtig7 recorded ventral mesen¬ cephalic lesions in a patient with the locked-in syndrome. Eye and lid move¬ ments were unaffected in their case. We report the clinical findings in another case of a mesencephalic locked-in syndrome in which bilateral cate.'"

Accepted

From the

publication Nov 18, 1978. Neurological Department, Universi-

for

ty of Berne, Switzerland. Reprint requests to Neurological University Clinic, Inselspital, CH 3010, Berne, Switzerland (Dr Meienberg).

Kazimierz Karbowski, MD

paralytic ptosis and, eventually, bilat¬ eral oculomotor paralysis were the unique features. REPORT OF A CASE A

65-year-old previously healthy

man

found unconscious with left-sided hemiplegia and Cheyne-Stokes respiration on the morning of Sept 9,1977. During the days that followed in the hospital, his neurologic state remained unchanged. He did not react to painful stimuli. Cardiovas¬ cular findings and blood pressure were normal. Four weeks later, the first signs that he was regaining consciousness were noted. He could execute simple commands with his right arm and leg. He was unable to open his eyes or to speak. He remained in this state for several weeks. A radioisotope brain scan performed in December 1977 was interpreted as normal. On Jan 1, 1978, the patient was clearly conscious. He could follow commands to protrude his tongue and point to various parts of his head. When his upper lids were held open, he could mimic with his hand the number of fingers presented to him by the examiner. His bilateral ptosis was com¬ plete and could not be overcome by volun¬ tary effort. Spontaneous contractions of the orbicularis oculi occurred from time to time. Corneal reflexes were hyperactive bilaterally. He could move his eyes in all directions, horizontally and vertically. Pu¬ pillary reactions were reduced but defi¬ nitely present in response to light and near stimuli. The pupils were round and equal in diameter (approximately 4 mm). When attempting to speak, he could make only stammering sounds. Voluntary innerva¬ tion of the buccal branch of the facial nerve was reduced bilaterally, while contraction of orbicularis oculi and frontalis muscles was normal. The gag reflex was increased and he could not swallow. The tongue could be protruded in the midline. The left arm was

and leg were paretic with increased tendon reflexes and an extensor plantar response. The plantar response was also extensor on the right. On May 16, 1978, further neurologic signs were recorded. In addition to his bilateral ptosis, marked exotropia had developed (Fig 1), with paralysis of adduc¬ tion elevation and depression of both eyes. Further voluntary abduction to the ex¬ treme position of gaze was possible to both sides. Pupils were mid-dilated and fixed. Bell's phenomenon was absent. Muscle reflexes in the face were hyperactive, while the palmomental reflex was absent bilater¬ ally. The right arm and leg were now also paralyzed and spastic, and their tendon reflexes were hyperactive. He was con¬ scious and could obey commands and communicate by moving his partially paretic right arm or by protruding his tongue. He had preserved sleep-awakeness cycles and normal spontaneous respira¬ tion. Computerized axial tomography (CAT) scans showed marked "dilation" of the aqueduct and mild but diffuse cortical atro¬ phy of both hemispheres appropriate for the patient's age. No other abnormal midbrain findings could be seen. This could have been due to poor image resolution and the relatively thick scan sections obtained on the scanner. The EEG (Fig 2) revealed slow back¬ ground activity of about 6 Hz. Reactivity to passive lid opening was partially pre¬ served. Now and then, 2- to 4-Hz 8- trains appeared in the record. Also, 1- to 2-Hz intermittent high, mostly rhythmic waves with bifrontal maxima occasionally were linked with steep potentials. From the history, the clinical findings, and the course of the disease, we concluded that our patient had sequential infarctions in his midbrain. He died on June 20, 1978. Permission for necropsy examination was denied.

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Fig 1 —Top, Total bilateral ptosis, not to be by voluntary effort. Bottom, Marked bilateral exotropia with paralysis

overcome

of adduction and vertical movements of both eyes. Further voluntary abduction of each eye to extreme position of gaze was possible. Pupils are mid-dilated and fixed.

Fig 2.—EEG at rest. Leftside, Dominant 6-Hz background activity with distinct reactivity passive lid opening (arrow). Right side, Intermittent bifrontal 8 rhythms about 1.5 Hz (monopolar recording with average reference after Goldman-Offner). to

COMMENT

nearly akinetic patient, awakeasily pass unnoticed. This oversight is particularly likely when the patient is initially comatose, as occurred in our case. Recognition of pseudocomatose states and their dif¬ ferentiation from true coma is impor¬ tant because of the psychic support required by the conscious patient. His physicians must protect him from idle In

eness

a

may

comments uttered in his presence. A patient with the locked-in syn¬ drome' " is nearly unable to move his face, arms, or legs, while sensory

systems and mental function

preserved. The

are

bilateral corticobulbar and corticospinal lesion in the brainstem, with resulting anar¬ cause

is

a

thria, dysphagia, occasionally trismus,

and spastic paralysis of all four extremities.':,K In the initial days.of a brainstem infarction, later resulting in a locked-in syndrome, the patient may be comatose.:1·-1·"·" For this reason, even in patients who remain in coma for longer periods, the physician must look repeatedly for signs of returning consciousness. Confusion has occurred because reported cases with preserved con¬ sciousness have been titled "akinetic

mutism,""" contrary propounded by Cairns

to guidelines et al."·" While

in both akinetic mutism and the locked-in syndrome crude conscious¬ ness or vigilance is retained, refined consciousness or mental function is nil in akinetic mutism. Appropriate re¬ sponses to commands fail despite absence of true paralysis.''"'·'-l:' This state has also been termed "coma vigile" '4·' ' Because of the different consequences for the management of the patients, we believe, as other

Fig 3.—Oculomotor nuclear complex (slightly modified from Warwick'8): Common subnucleus of origin for both levator palpebrae superioris muscles lies at dorsocaudal end of oculomotor nuclear complex. Subnuclei for all other extraocular muscles extend rostrocaudally over most of its length. Therefore, circumscribed lesion at level of section A can produce isolated bilateral ptosis. authors do,'"'iT that the terms "aki¬ netic mutism" and "locked-in syn¬ drome" should be clearly distin¬

guished. A diagnosis of locked-in syndrome

to be justified in our patient since there was spastic quadriparesis, anarthria, dysphagia, and preserved mental function. But he exhibited peculiarities that distinguish his synseems

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drome from those caused by ventral pontine involvement and that addi¬ tionally tended to mask his true state of consciousness. In pontine locked-in syndromes, lid and vertical eye movements are usual¬ ly preserved. Because of this, the patient has a way to communicate with those who take care of him. Our patient with total bilateral ptosis could not use this means of communi¬ cation unless his upper lids were held open. Later in the course, with paraly¬ sis of eye movements, this possibility also was lost. The abrupt onset of severe isolated bilateral ptosis occurs with ischemie involvement of the dorsocaudal cell group in the oculomotor nucleus.'"" The two levator palpebrae superioris muscles are simultaneously paralyzed by a lesion at the nuclear level because the levator subnucleus is compact and in the midline. Other oculomotor subnuclei extend rostrocaudally over most of the oculomotor nucleus.'" The

parasympathetic Edinger-Westphal nuclei lie rostrally in the nucleus, and in this location may be spared by large

lesions in the oculomotor nucleus"' (Fig 3). It may be inferred that the initial lesion in our patient destroyed the caudal end of the oculomotor nucleus. The next ischemie event probably destroyed the more rostral portions of the oculomotor nuclei, causing total bilateral oculomotor nerve paralysis. Preserved function of the abducens nerves caused exotropia. Retention of abduction in both eyes, preservation of the corneal reflex arc and blink reflexes, and the exotropic eye position are evidence against the possibility of involvement of the pontine tegmentum in our patient. Another unusual feature in our patient's case was the EEG. The EEG in most pontine locked-in syndromes Moderate is normal, or nearly so. slowing of the background activity with intermittent mostly rhythmic high voltage 1- to 2-Hz trains with preponderant bifrontal maxima in our patient's EEG are similar to findings recorded in awake cats after partial lesions of mesencephalic ascending reticular pathways.2- Such EEG changes occur in patients with mid''-'"-'

brain lesions.'"-"-" Vigilance cannot be assessed by EEG since correlation between severity of EEG abnormali¬ ties and level of consciousness is not absolute."'" -'-'

Neuro-ophthalmologic signs permit¬

ted localization of our patient's proba¬ ble ischemie infarction in the mid¬ brain. The EEG findings added sup¬ port to this clinical localization of the lesion. To our knowledge, only one midbrain lesion has been recorded with clinical signs of a locked-in syndrome.7 Since in that case oculomo¬ tor function was normal, we conclude that the portion of the midbrain responsible for the syndrome lies ventral to the oculomotor complex in the decending corticobulbar and corticospinal pathways, and to a limited extent, probably also in the reticular activating system. Although the locked-in syndrome had not yet been defined as such, case 1 reported by Masucci et al-3 probably represented in all essential details the syndrome reported herein.

References

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6. Dehaene J, Martin JJ: "Locked-in" syndrome: A clinico-pathological study of two cases. Eur Neurol 14:81-89, 1976. 7. Karp JS, Hurtig HI: "Locked-in" state with bilateral midbrain infarcts. Arch Neurol 30:176\x=req-\ 178, 1974. 8. Kubik CS, Adams RD: Occlusion of the basilar artery: A clinical and pathological study. Brain 69:73-121, 1946. 9. Klee A: Akinetic mutism: Review of the literature and report of a case. J Nerv Ment Dis

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H, Silberman J, Feigin I: A clini-

pathologic study of akinetic mutism. Neurology 10:10-21, 1960. 11. G\l=o"\tzeP, K\l=u"\hneD: Akinetischer Mutiscal and

mus\p=m-\Einbemerkenswertes klinisches Ph\l=a"\nomen bei Verschluss der A. basilaris. Akt Neurol 4:121\x=req-\ 127, 1977. 12. Cairns H, Oldfield RC, Pennybacker JB, et al: Akinetic mutism with an epidermoid cyst of the third ventricle: With a report of the associated disturbance of brain potentials. Brain 64:273-290, 1941. 13. Cairns H: Disturbances of consciousness with lesions of the brainstem and diencephalon. Brain 75:109-146, 1952. 14. Fischgold H, Mathis P: Obnubilations, comas et stupeurs: Etudes \l=e'\lectroenc\l=e'\phalographiques. Electroenceph Clin Neurophysiol 11 (suppl), 1959. 15. Alajouanine T: Les alt\l=e'\rationsdes \l=e'\tatsde conscience caus\l=e'\espar les d\l=e'\sordresneurologiques, in Proceedings of the First International Congress of Neurological Sciences. Brussels, Editions Acta Medica Belgica, 1957, pp 19-41. 16. Stevenson GC, Hoyt WF: Metastasis to midbrain from mammary carcinoma: Cause of bilateral ptosis and opthalmoplegia. JAMA 186:514-516, 1963. 17. Growdon JH, Winkler GF, Wray SH: Midbrain ptosis: A case with clinicopathologic

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Quadriparesis and nuclear oculomotor palsy with total bilateral ptosis mimicking coma: a mesencephalic 'locked-in syndrome"?

Quadriparesis and Nuclear Oculomotor Palsy With Total Bilateral Ptosis Mimicking Coma A Mesencephalic 'Locked-in Syndrome'? Otmar Meienberg, MD; M...
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