Guillain-Barré, Fisher, Bickerstaff syndromes: nature versus well established ideas.
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Biomedical subjects
Publications and source records attributed to I Derakhshan.
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Lymphocyte subpopulations ("active' T cells, total T cells, and B cells), leukocyte migration, lymphocyte transformation, complements (CH50, C3, and C4), and immune complexes in serum and cerebrospinal fluid were studied in 23 patients with subacute sclerosing panencephalitis. The percentage of active T cells was normal. The number of patients with positive migration inhibitory response to phytoyhemagglutinin was lower than controls, corresponding to the diminished percentage of total T cells in patients. C1q binding (immune complex formation) was found in the sera of 10 patients.
This report describes the computerized tomographic, pneumoencephalographic, and anatomic findings in a case of holoprosencephaly. Computerized tomography is suggested as the most accurate diagnostic method in defining the nature and extent of those anomalies related to the failure of cleavage of the prosencephalon. The value of this method in detecting less severely affected family members is evident.
Two patients are reported with a chronic progressive illness characterized by dementia, ataxia and spasticity. There were no myoclonic jerks and both had normal electroencephalograms (EEG). Pathological findings in three brain biopsies were those of viral meningoencephalitis with perivenous demyelination. Serological data in both patients indicated the presence of measles virus infection. Intracytoplasmic structures resembling measles virus nucleocapsids were found in the brain biopsy of one patient. Immunofluorescent staining showed antibody in the temporal lobe biopsy of both patients. It is suggested that these patients are examples of a chronic form of measles meningoencephalitis hitherto undescribed.
Clinical and pathological findings in two cases of degenerative progressive myoclonic epilepsy (PME) are described. The clinically difficult task of differentiating a "cerebellar" tremor from an action myoclonus is emphasized. Simultaneous electroencephalography and electrokymography was done, using capacity to ground transients for recording hand movements. This method was found useful in corroborating the cerebellar nature of the remaining disorder, after successful treatment of the myoclonic element with anticonvulsants.
Antimeasles antibody was detected in the saliva of 14 patients with subacute sclerosing panencephalitis. Immunofluorescent staining showed antibody in salvary gland biopsy of 7 patients.
This report concerns a patient with ophthalmoplegia, ataxia and hyporeflexia (Fisher's syndrome) with a lesion in the midbrain tegmentum demonstrated by computerized-tomography (CT) scanning. Spontaneous recovery was almost complete 1 month after the onset. Based upon its strategic location, it is suggested that the lesion can explain the findings in the patient. The CT finding, if confirmed, will necessitate a reconsideration of our current pathogenetic views about Landry-Guillain-Barré syndrome in general and Fisher's syndrome in particular.
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The validity of reports suggesting that Lyssa and Negri bodies are non-specific in the light-microscopical diagnosis of rabies was investigated. With Seller's impression technique, a substantial proportion of speciments found to be non-rabid with the fluorescent-antibody technique showed structures indistinguishable from Lyssa or Negri bodies. Neither histological examination nor inoculation of animals with non-rabid Seller-positive material explained the nature of these rabies-like structures. The simplicity and reliability of the fluorescent-antibody technique and the occasional serious complications of prophylactic anti-rabies measures make the diagnostic use of Seller's method at best undesirable and at worst dangerous.
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Thirty-five patients (19 male and 16 female) with clinically definite multiple sclerosis and 100 healthy control subjects were studied for the A and B locus of the HLA system. A significant increase of HLA A3 and A11 of the A locus and B7 of the B locus was observed in patients with multiple sclerosis compared with controls. An increase of antigen A3 was also observed in eight cases of probable multiple sclerosis. A significant increase of both A3 and B7 was observed in patients with multiple sclerosis.
Three cytopathic strains of subacute sclerosing panencephalitis (SSPE) virus were isolated from brain biopsies of three patients. These strains were isolated and maintained by cocultivation of infected brain cells with fresh Vero cells. The biological characteristics of two strains were studied. It was found that these strains remain cell-associated after repeated cocultivations with Vero cells and produce plaques under fluid medium or tragacanth overlay. The correlation with measles virus was demonstrated by the plaque reduction test as well as by the immunofluorescence test. Large numbers of nucleocapsids were observed in the cytoplasm of infected cells but none in nuclei. Intracerebral inoculation of monkeys, adult guinea pigs, newborn and adult hamsters or mice was followed by acute encephalitis and death.
Since first described by Negri in 1903, the Negri body has been regarded as a pathognomonic finding in signifying the presence of rabies encephalitis. Negri bodies (light microscope) were found in the brain of a patient with conclusive evidence against the presence of rabies encephalitis. This case provided the opportunity for a pertinent review of the literature in bringing the subject into a reasoned perspective. A definitive etiologic diagnosis of rabies requires the use of electron microscopical or immunofluorescent methods or both.
A patient with Reye syndrome had intracytoplasmic inclusion bodies in Purkinje cells. Electron microscopical examination demonstrated the inclusions to contain internal particles, but these were not identifiable as known virions. Results of viral studies were normal.
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The clinical features of a patient with bilateral oculomotor palsy, ataxia, disturbance of memory, and hypokinesia are described. Pneumography and CT scanning showed dilation of the posterior portion of the third ventricle, indicating involvement of the postero-medial thalamic structures. The relation of this finding to the patient's amnesia and hypokinesia is briefly discussed. It is concluded that the patient suffered an infarction within the region served by penetrating branches which arise from the cephalad end of the basilar artery, probably including the mesencephalic artery.