Journal of neurology, neurosurgery, and psychiatry
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J. Neurol. Neurosurg. Psychiatr. · Jan 1974
Mixed or immune complex cryoglobulinaemia and neuropathy.
Three patients with peripheral neuropathy and mixed or immune complex cryoglobulinaemia are reported. The significance of mixed cryoglobulinaemia and the pathogenesis of the peripheral neuropathy are discussed.
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J. Neurol. Neurosurg. Psychiatr. · Aug 1973
Creutzfeldt-Jakob disease treated with amantidine. A report of two cases.
The treatment of two cases of Creutzfeldt-Jakob disease with amantidine is described. The first case made a remarkable initial improvement which was sustained for two months, but then deteriorated and died. ⋯ The second case which was clinically one of Creutzfeldt-Jakob disease has now been followed for 30 months since the start of treatment and appears to be cured. It is considered that amantidine has a definite effect in this disease and it is suggested that its mode of action, though unknown, is more likely to be metabolic than antiviral.
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This is the second reported case, known to the authors, of complete, but selective, limbic lobe destruction in previously normally functioning central nervous systems. Both cases had an amnestic syndrome, whose characteristics were essentially similar to amnestic syndromes previously documented with less complete limbic destruction, with one difference-confabulation remained a prominent feature in the chronic stages of the memory disorder. ⋯ Our case illustrates that the syndrome can be produced by necrotizing encephalitic lesions. We suggest that the combination of the above two syndromes is essentially a `limbic dementia'.
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The diagnosis of porencephaly by isotope cisternography is described. In the three cases presented, porencephaly was associated with non-resorptive hydrocephalus. The communicating hydrocephalus caused the isotope to enter the ventricular system and visualize the cyst, and the diagnosis of both disorders was established by RIHSA cisternography. The method is simple and non-traumatic and provides information about abnormalities which air may fail to demonstrate.
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Myasthenia gravis in one member each of two sets of twins is described. A 17 year old girl developed generalized myasthenia gravis at the age of 14 years, while her monozygotic twin sister has remained in good health during a three year period of observation. ⋯ Both members of twins were affected in five sets of monozygotic twins and one set of twins with undetermined zygosity, and only one twin was affected in the remaining sets of twins. Occurrence of myasthenia gravis in both members of monozygotic twins and in none of dizygotic twins, and frequent familial occurrence of the disease suggest the role of a genetic factor in the pathogenesis of this disease.