Biomedical subjects
Publications and source records attributed to J Braham.
A 17-year-old girl suffered from episodes of flaccid paralysis during each menstrual cycle. No relevant changes were found in potassium concentrations in serum or erythrocytes, nor in the values of pituitary, ovarian, adrenal or thyroid hormones. Acetazolamide administration prevented the attacks.
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A case is reported of a 46-year old male who has been suffering from recurrent episodes of quadriplegia, characteristic of hypokalemic periodic paralysis, for 28 years. During this period he has developed typical signs and symptoms of multiple sclerosis. The association of hypokalemic periodic paralysis and multiple sclerosis is extremely unusual.
Skin wrinkling of the fingers following immersion in warm water depends on intact sympathetic innervation. It is abolished by lesions affecting both central and peripheral sympathetic pathways. It affords a simple and reliable clinical test of sympathetic function.
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Alexia without agraphia occurred in a 41-year-old man suffering from a left occipital brain tumor. The syndrome disappeared following excision of the neoplasm.
The mean activity of glutathione peroxidase (GSH-PX) in erythrocytes of 22 Israel-Jewish patients with multiple sclerosis (19.3 +/- 4.5 U/gHb) was significantly lower than in a control group of 30 Jewish patients with various neurological disorders (24.3 +/- 5.1 U/gHb). This observation confirms a similar finding of a decreased activity of GSH-Px in erythrocytes of multiple sclerosis patients in Denmark (Shukla et al. 1977). These results are discussed in relation to the possibility of selenium deficiency and to the recently described genetic polymorphism and ethnic variation of GSH-Px activity in human red cells. It is concluded that additional investigations are required in order to elucidate the cause of the decreased activity of this enzyme in red cells of patients with multiple sclerosis.
Severe dementia and ataxia in an elderly woman were related to hydrocephalus resulting from Paget disease of the skull, with basilar impression. Ventriculoatrial shunting resulted in dramatic improvement. This procedure, rather than suboccipital decompression, is probably the operation of choice, and should be carried out at an early stage.
Electromyographic examination of vertical and lateral extraocular muscles was carried out in five patients suffering from progressive supranuclear palsy, and incapable of performing voluntary vertical eye movements. No evidence of a lower motor neurone lesion or paradoxical innervation of eye muscles was noted. Reciprocal inhibiton of antagonist vertical muscles though present in oculocephalic (doll's head) stimulation, was incomplete on attempted voluntary movement. This factor is held to be the probable immediate cause of the vertical gaze palsy.
Of 150 new cases of nasopharyngeal carcinoma diagnosed in Israel during a 9-year period (nationwide study 1960--1968), 74 patients developed neurological complications. In 92% of the cases, neurologic deficits were confined exclusively to cranial nerves. The majority of these patients presented general symptoms of disease such as neck masses, head and facial pain, or nasal and aural disorders. In 23 patients (34%), the initial presentation was of neurologic disability. The most frequently affected nerves were the abducens and the trigeminal. In the fully developed clinical picture, ocular and trigeminal lesions predominated. 5-year survival in patients with cranial nerve involvement was 21% as compared to 55% in those without such lesions. Survival was not influenced by delayed diagnosis.
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Arteriovenous malformations in the vicinity of the splenium are a rare cause of subarachnoid bleeding. They are usually unaccompanied by neurological deficits. Three such cases are described and methods of identification and surgical approach discussed. Total extirpation is believed to be the treatment of choice.
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Two patients with dermatomyositis were treated with methotrexate, given intravenously. They were unresponsive to corticosteroids. The patients were bedridden and paralysed and one seemed to be in the terminal phase of the disease. The response to courses of methotrexate treatment was satisfactory and sustained over a 2-year follow-up period; muscle enzyme studies indicate that the muscle disease is no longer active. Although steroid therapy alone may be effective, its usefulness is unpredictable and may lead to dependence and undesirable side effects. Our experience with these two patients suggests that a combination of the two drugs may be justified as initial treatment in severe cases.
A case of acute flaccid paraplegia is reported, due to epidural bleeding in the dorso-lumbar region. A hitherto undiagnosed and asymptomatic hemangioma of a dorsal vertebra was considered to be the source of the haemorrhage. Recovery followed early laminectomy and evacuation of blood clots.
Arteriographic, immunological, and bone marrow studies of a young woman with Takayasu arteritis disclosed the presence of sea-blue histiocytes in her bone marrow. We know of no other similar reports.
Two cases are described presenting a clinical picture of sudden headache, fever and vomiting, accompanied by unilateral ophthalmoplegia and acute deterioration of visual acuity. In one this amounted to monocular blindness with a field defect in the other, indicative of chiasmal compression. Necrotic pituitary adenoma material was removed by the transphenoidal route in both cases. Recovery of eye movements was obtained and considerable improvement of visual acuity, which in one patient, was restored completely.