Pure Motor Hemiplegia, Medullary Pyramid Lesion, and Olivary Hypertrophy
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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.39.9.877 on 1 September 1976. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry, 1976, 39, 877-884 Pure motor hemiplegia, medullary pyramid lesion, and olivary hypertrophy J. E. LEESTMA' AND A. NORONHA From the Departments ofPathology and Neurology, Northwestern University School ofMedicine, Chicago, Illinois, USA SYNOPSIS The case is presented of a 60 years old man who developed sudden right hemiplegia without other accompanying neurological signs and later a spastic hemiparesis. Neuropathological studies indicated an ischaemic lesion ofthe left medullary pyramid which was accompanied by hyper- trophy of the left inferior olivary nucleus. An additional lesion, demyelination of the right gracile tract, is poorly explained. This case represents the second reported instance of pure motor hemiplegia due to a circumscribed lesion in the medullary pyramid and possibly an unique instance of olivary hypertrophy without obvious damage to the central tegmental tract, ipsilateral superior cerebellar guest. Protected by copyright. peduncle, or contralateral dentate nucleus. The olivary hypertrophy is thought to have arisen from local damage to the termination ofthe central tegmental fibres at the left inferior olivary nucleus. The question of the development of spasticity in a pure pyramidal tract lesion is discussed. Fisher and Curry (1965) defined pure motor consciousness, headache, convulsion, paraesthesiae, hemiplegia as a paralysis, complete or incomplete, vertigo, diplopia, dysphagia, or visual difficulty. One of the face, arm, and leg on one side unaccom- month before this episode hypertension was noted for panied by sensory signs, visual field defect, the first time. He was in congestive heart failure and was treated with methyldopa, digoxin and frusemide. dysphasia, or apractagnosia. In their cases that There was no previous history of transient ischaemic came to necropsy the syndrome resulted from episodes. At the time of examination he was alert, infarction in the internal capsule or the basis oriented, and there was no mental impairment. The pontis. They stated that it was doubtful that a visual fields were intact; the pupils were equal and pyramidal infarction would result in pure motor reacted to light and accommodation. The extraocular hemiplegia without other medullary signs. movements were normal and there was no nystagmus. Chokroverty et al. (1975a) reported the first case Facial sensation was normal. There was a question- of an infarction of the medullary pyramid in able mild right central facial paresis; there was which hemiplegia was the only sign. We report flattening of the right nasolabial fold but no deviation http://jnnp.bmj.com/ another case of motor due to an of the angle of the mouth. No dysphagia, palatal or pure hemiplegia lingual paresis, or palatal myoclonus was noted. The ischaemic vascular lesion of the medullary patient had a right hemiplegia which was dense in the pyramid. arm, less in the right leg with the muscle strength estimated at 4/5. Initially there was flaccidity in the CASE REPORT hemiplegic limb but in two weeks this changed to spasticity on the affected side. The reflexes were A 60 years old man developed weakness of the right brisker on the affected side and right ankle clonus was on September 26, 2021 by side of his body on 2 November 1973. The onset of also noted. The plantar responses were extensor on weakness was sudden. There was no impairment of the right and flexor on the left. There were no cerebel- lar signs, no sensory deficit to touch, pin-prick, vibration, orjoint position. Cortical sensory functions I Address for correspondence and reprimt requests: Dr Leestma, were intact. There were bilateral carotid bruits. A Department of Pathology, Northwestern University School of Medicine, 303 East Chicago Avenue, Chicago, Illinois 60611, USA. brain scan performed two weeks after admission was (Accepted 26 April 1976.) interpreted as within normal limits. 877 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.39.9.877 on 1 September 1976. Downloaded from 878 J. E. Leestma and A. Noronha The patient was discharged three months later appeared altered and was substantially larger and with improvement in the hemiparesis. He was re- more distinct than the right. These changes in the admitted in September 1974 with gangrene of the olive were seen throughout the medulla. The speci- lower right leg. An aortogram revealed occlusive men terminated at the lower medullary level and no vascular disease with complete obstruction of the spinal cord was available for study. Sections of the right femoral artery. An above the knee amputation cerebellum revealed no abnormality, the dentate was done and the patient recovered promptly. He was nuclei appearing well preserved. admitted again in October of 1975 for an inguinal Microscopically, sections stained with haematoxy- herniorrhaphy. After this procedure he sustained a lin and eosin, and Kluver-Barrera stain (Nissl with cardiac arrest and after abortive resuscitation he died. Luxol Fast Blue) were studied. The sections correlated well with the gross findings. The cerebral cortex was NECROPSY FINDINGS The general necropsy revealed unremarkable. One or two senile plaques were found severe atherosclerosis which involved aorta, carotid only in the hippocampus. The small lacunar infarcts arteries, coronary arteries, and peripheral vessels in in the basal ganglia were all old and the sections of the extremities. There was a massive acute myocardial mid-brain and pons showed no lesions and uniform infarction affecting the posterior wall of the left myelinization. The most rostral section of the medul- ventricle with evidence of old scarring and hyper- lary region (Fig. I) showed myelin loss in the left trophy throughout the left ventricular myocardium. pyramid extending to a small extent into the medial There was an unruptured abdominal aortic aneurysm. lemniscus. There was also some pallor in the myelin Pulmonary oedema and congestion were noted as of the external arcuate fibres lateral to the left olive. were other signs of terminal myocardial failure. The The olive itself showed no alterations apart from the stump of the right leg was well healed. usual accumulation of lipofuscin within the neurones guest. Protected by copyright. at this level. There was pronounced astrocytosis in NEUROPATHOLOGICAL EXAMINATION Grossly, the the affected pyramid as well as a slight perivascular brain was unremarkable; no obvious lesions pre- lymphoid cell infiltrate. Scattered lipid laden macro- sented themselves. The leptomeninges were slightly phages as well as some swollen axons were seen in the thickened in keeping with the age of the patient and pyramid. The opposite pyramid was unremarkable. slight cortical atrophy mostly in the frontal lobes was In the mid-portion of the medulla at the level of the present. The inferior surface of the brain revealed no 12th cranial nerve nucleus (Fig. 2) several changes obvious abnormalities with the circle of Willis con- taining only scattered atheromatous plaques, signi- cantly less in amount than were seen in the extra- cranial vessels. The plaques were found mostly in the internal carotid vessels, scattered along the middle cerebral arteries, at the distal ends of the vertebral arteries and at the proximal and distal ends of the basilar artery. The circle of Willis was normal anatomically with two vertebral arteries of about equal calibre, two posterior communicating and one anterior communicating artery. No obstruction ofany vessel was noted. Outwardly the brain stem and http://jnnp.bmj.com/ cerebellum appeared unremarkable. Multiple coronal sections of the brain revealed slight cortical atrophy with widening of the sulci and widening of the insula. The ventricles were slightly enlarged. The cortical ribbon was uniform and there were no cerebral softenings. The globus pallidus and putamen contained several small perivascular la- cunes, but none was larger than two millimetres. The on September 26, 2021 by thalamus was preserved. Cross-sections of the mid- brain revealed a pigmented substantia nigra and a patent aqueduct and no lesions. No lesions were found FIG. 1 The most rostral section ofmedulla stained by in the pons. However, the most rostral section of the the Kiiver-Barrera methodshows myelin loss in the left medulla oblongata revealed that the left medullary pyramid as well as slight pallor laterally and medially. pyramid was shrunken and of a tan-brown colour No hypertrophic changes in the inferior olives at this compared with the right. The inferior olive also level are noted. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.39.9.877 on 1 September 1976. Downloaded from Plure m0otor hemiplegia, medullary pyramid lesion, and olivary htypertrophy 879 ( FIG. 2 A mid-sectioni of the mnedulla reveals the same FIG. 3 The most caudal section of medulla illustrates pyramidal mnyelin loss as in Fig. 1 but additionally the continued pyramidal tract demyelination, hyper- shows hypertrophic changes in the left inferior olivary trophy ofsuch olivary elements as are remaining on the nucleus. Myelin pallor lateral and medial to the olive left side, and myelin loss in the right gracile nucleus and guest. Protected by copyright. is also noted. tract. were apparent. In addition to the changes previously alterations in the left medullary pyramid; hyper- noted in the pyramid, there was pronounced hyper- trophy of the caudal half