Isolated oculomotor nerve palsy.
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Biomedical subjects
Publications and source records attributed to I Dehaene.
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We report a case of left superior oblique palsy combined with a right internuclear ophthalmoplegia. A right mesencephalic lesion involving the trochlear nerve nucleus (or its fibers prior to decussation) and the medial longitudinal fascicle was hypothesized. Magnetic resonance imaging showed a lesion at the suspected level.
We report a 73-year-old patient with an eye movement disorder characterized by paralysis of saccades and pursuit. At autopsy, there were small cortical lesions in the middle frontal gyrus immediately anterior to the precentral gyrus and in the inferior parietal lobule on both sides.
We report an autopsy proven case of acute hemorrhagic leukoencephalitis. There was a very rapid evolution leading to death in less than 24 hours. CT of the brain revealed widespread hypodensities in both frontal lobes. In the cerebrospinal fluid as well as in the peripheral blood, there was a moderate leucocytosis. The literature is reviewed.
Two patients developed acute sensorimotor polyneuropathy after intoxication with lithium carbonate. Nerve conduction studies, electromyography, and sural nerve biopsy proved it to be an axonal neuropathy. Recovery of muscle strength, reflexes, and sensory function started weeks after discontinuation of lithium therapy. One patient fully recovered within a year. In the literature we found nine other cases of lithium polyneuropathy.
Many lesions along the visual pathway from the retina to the occipital cortex can cause a horizontal hemianopia. The anatomy of the visual pathway is reviewed in an attempt to clarify how and where this visual field defect develops. A case is presented in which two ischemic occipital infarcts caused a bilateral inferior horizontal hemianopia and complex visual hallucinations. Previous cases of occipital lesions resulting in an altitudinal hemianopia are reviewed.
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The neuropathologic findings are described in a fatal case of Guillain-Barré syndrome with ophthalmoplegia. Demyelination was found in the oculomotor nerves, which were dissected to the terminal branches in the ocular muscles, and in other peripheral nerves. The CNS was intact except for occasional chromatolytic lesions in some motor nerves.
A patient with a classical locked-in syndrome is described, in whom bilateral ptosis was observed on the 2nd day. On the 4th day, ophthalmoplegia was complete with the exception of a voluntary downward movement of the right eye. The syndrome can be explained by the association of bilateral horizontal pontine gaze palsy and a unilateral nuclear oculomotor nerve palsy, and approximates the "total locked-in syndrome".
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Absence of AMP-deaminase was demonstrated by histochemical and biochemical methods in a muscle biopsy of a 25-year-old woman with facial and limb girdle myopathy. Venous ammonia failed to rise after ischaemic exercise. This patient further contributes to the variety of clinical pictures associated with AMP-deaminase deficiency. Whereas AMP-deaminase has been shown to play an essential role in the regulation of adenine nucleotide metabolism in the liver, its physiological function in muscle remains uncertain.
Clinical and neurophysiological studies were carried out in hereditary pressure-sensitive neuropathy. In 4 generations, 19 out of 87 family members were affected. The transmission of the disorder is autosomal dominant with total penetrance but variable expression. The motor conduction velocities of peripheral nerves were slowed even in clinically unaffected nerves. Light- and electron-microscopic studies were performed in the fibular nerve of one patient. A characteristic tomaculous neuropathy was discovered.
The authors describe a series of 26 patients (28 operations) with Arnold Chiari malformations who were admitted during a 4 year period. In 20 the malformation was treated by direct surgery on the posterior fossa. Seven were treated by ventriculoatrial shunting. An analysis of the surgical risks of each procedure is given. In addition a helpful guide in the choice of the proper surgical procedure is outlined.
Two new examples of "locked-in" syndrome are reported. The patients are mute and quadriplegic; they present a bilateral horizontal gaze palsy. Contact can be established through preserved vertical eye movements demonstrating therefore that the patients are not comatose. Postmortem examination reveals in both cases a thrombosis of the basilar artery with an infarction of the ventral rostral pons extending dorsally into the paramedian tegmental area. The main clinical features of this syndrome and their anatomical correlates are discussed.