[The fatty acids in experimental encephalopathy caused by cyanides].
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Biomedical subjects
Publications and source records attributed to G Arnetoli.
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Six epileptic patients are described to whom the addition of Valproic Acid (VPA) to a previously unsatisfactory antiepileptic treatment caused a toxic encephalopathy. This was characterized by alterations of the state of consciousness in all patients a few days after the beninning of the treatment with VPA. These ranged from a marked drowsiness to coma and were often associated with gastrointestinal and neurological (ataxia, asterixis) symptoms. In all cases very high blood ammonia values were found and the EEGs showed a diffuse slowing down of the activity. After the discontinuation of the drug the toxic symptoms quickly ceased and ammonia values returned to the normal values. It is hypothesized that the interference of VPA on the metabolism of ammonia could play an important role in the pathogenesis of the VPA-induced toxic encephalopathy.
The authors report the clinical, instrumental and histopathological data observed in a 27 year old man, who died 20 months after the onset of a rapidly progressive neurological syndrome referable to a multitopic brain damage. The clinical picture, which at its fully developed stage was represented by supranuclear ophthalmoplegia, cerebellar ataxia, akinetic-rigid parkinsonism with axial dystonia and dementia, appeared consistent with the diagnosis of P.S.P. The histological examination showed degenerative changes of varied degree in the cerebellum, the brain-stem and the basal ganglia, but the neurofibrillary tangles characteristic of P.S.P. were not found, either with electron-microscopy. The case presented considerable difficulties regarding its nosological classification. Nevertheless, the lack of neuropathological findings of storage disease as well as the particular location of the degenerative lesions have induced the authors to consider it as an atypical form of sporadic O.P.C.A. in the field of M.S.A.
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A case of permanent global amnesia resulting from a bilateral hippocampal lesion is reported, remarkable for its typical clinical picture and prolonged period of observation with repeated neuropsychological testing. No tendency toward spontaneous recovery was noted, even though extra-mnesic cognitive functions remained undamaged. No differences were observed between this amnesic syndrome and the neuropsychological picture during an acute episode of transient global amnesia.
Genetic, elettromyographic, enzymological and histological aspects of two cases of proximal pseudomyopathic spinal amyotrophy are described. The former is related to a typical Wohlfart-Kugelberg-Welander syndrom with characteristics of recessive autosomal heredity. It is related to a fifteen-years old patient who, since he was ten has been affected by progressively increasing muscular hypotrophy. It was localized mostly on the scapular girdle and less on the pelvic girdle, with tendency to extend to the distal muscles of the limbs, without fasciculations or sensory symptoms. Serum enzyme levels were normal. Both muscular biopsy and electromyographic tests yield reports testifying a spinal amyotrophy. The later is related to a spinal pelvic girdle amyotrophy arisen in a thirty-three-year old subject who since twenty-nine has started to present weakness and hypotrophy of pelvic girdle muscles and of the roots of the thighs particulary involving the adducent muscles. There were neither amyotrophies in other districts nor fasciculations. Serum enzyme levels were normal. Muscular biopsy clearly proved a neurogenic amyotrophy. E.M.G. showed signs of myogenic and neurogenic sufference. Problems concerning the nosographic delimitation of different spinal muscular atrophies are discussed on the ground of literature, too.
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