A case of Refsum’s disease treated by serial plasma exchanges together with a moderate low phytanate diet is reported. Serial plasma exchanges determined a rapid significant clinical improvement (neuropathy and cerebellar ataxia) that allowed immediate return to full-time employment. The initial improvement could be maintained by intermittent serial plasmapheresis despite partial failure of the initially introduced low phytanate diet bringing 20 mg phytanic acid daily. A new dietary regimen bringing 10 mg phytanic acid was later introduced that was well tolerated. No liquid formula was used. The clinical improvement was clearly correlated to a fall in serum phytanic acid from 45.3 to 16.2 mg/100 ml.
We studied a patient with progressive myelopathy, in whom systemic sarcoidosis was discovered. Involvement of other organs was asymptomatic. The myelopathy considerably improved after corticosteroid therapy. Spinal cord involvement in sarcoidosis is very uncommon, and its occurrence as a presenting manifestation is even rarer. Prompt recognition is emphasized, because it is a treatable condition.
627allowed demonstration of marked microcirculatory changes occuring during vasospasm which cannot be detected by macroscopic techniques. SUMMARY Twenty-three patients with occlusion of an internal carotid artery hare been followed 5 to 60 months after angiography. None had a later permanent stroke. Eight had delayed TIAs in the occluded internal carotid area, never in another area. In these TIAs the role of the homolateral external carotid artery is emphasized, because in the 8 cases this artery was the main collateral to the occluded internal carotid, and angiography had shown atheromatous stenosis of homolateral external/common carotid arteries or an irregular stump at the site of the occlusion. Hemodynamic and embolic mechanisms are discussed, especially the latter, because of the absence of serere stenosis and eridence of emboligenic plaques. Stroke, Vol 12, No 5, 1981
References
A patient with infarction of the paramedian part of the lower midbrain on the right side is described, in whom internuclear ophthalmoplegia and bilateral ptosis were associated with limitation of elevation of the contralateral eye from paresis of the superior rectus. Supranuclear paresis was suggested by partial dissociation between phasic and tonic components of upward gaze. It is suggested that the right-sided lesion involved the rostral median longitudinal fasciculus before it reached the oculomotor nucleus, the central caudal nucleus medially situated in the lower end of the oculomotor nucleus, and the prenuclear fibres from the posterior commissure to the ipsilateral nucleus of the contralateral superior rectus. A supranuclear lesion may be suspected when there is a complex association of dysconjugate palsy of oculomotor muscles.
Three cases of syphilis with cochleovestibular symptoms are reported. There was either cochlear or vestibular involvement in two cases and, in the trird, cochlear and vestibular involvement together. The disorder was unilateral in all three. In one case there was a concurrent lesion of the oculomotor nerve which was the motive for consultation. There was no other symptom suggesting the diagnosis of syphilis in any case. The important features of diagnosis are discussed.
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