An Iraqi brother and sister both developed progressive dementia of sudden onset with generally increased muscle tone and hyper-reflexia. In the sister this was accompanied by grand ma1 fits and myorlonic jerks. The disease began in both siblings in June 1967 when the brother and sister were aged 20 and 21 yr. respectively. The parents were said to be distantly related. Three other siblings were normal. Laboratory investigations were non-contributory. The brains showed conspicuous generalized cortical atrophy, less marked in the occipital regions, and dilatation of the ventricular system. Microscopically there was considerable nerve cell loss with fibrous glial reaction in the frontal, temporal and parietal cortex and less marked changes in the occipital lobe. The most conspicuous feature was neurofibrillary change, most prominent in the cerebral cortex, hippocampus, hypothalamus, substantianigra, mid-brain tegmcntal region and the pontine and medullary periventricular regions. No senile plaques were evident. Granulo-vacuolar change was demonstrated in occasional degenerate nerve cells. Conditions in which neurofibrially change has been described include Alzheimer’s disease, senile dementia, post-encephalitic Parkinsonism, Pick’s disease, sporadic motor neurone disease, infantile neuroaxonal dystrophy, vincristine neuropathy, Down’s syndrome, Steel-Richardson-Olszewski syndrome, olivary hypertrophy, various experimental conditions, and Guam-Parkinsonian dementia. The distribution of the pathological findings in these cases most closely resembled that in Guam-Parkinsonian dementia although clinically our patients did not show Parkinsonian features. In most of the above diseases neurofibrillary change is considered to be due to intraneuronal masses of closely packed twisted tubules which displace normal cell organelles. The mechanism by which thcse twisted tubules develop is not known. Electron microscopy was not performed in the authors’ cases. It has been postulated that the Guam-Parkinsonian ementia is caused by a viral infection in a person who is genetically susceptible and the authors considered a similar aetiology possible in their cases. An Iraqi brother and sister both developed progressive dementia of sudden onset with generally increased muscle tone and hyper-reflexia. In the sister this was accompanied by grand ma1 fits and myorlonic jerks. The disease began in both siblings in June 1967 when the brother and sister were aged 20 and 21 yr. respectively. The parents were said to be distantly related. Three other siblings were normal. Laboratory investigations were non-contributory. The brains showed conspicuous generalized cortical atrophy, less marked in the occipital regions, and dilatation of the ventricular system. Microscopically there was considerable nerve cell loss with fibrous glial reaction in the frontal, temporal and parietal cortex and less marked changes in the occipital lobe. The most conspicuous feature was neurofibrillary change, most prominent in the cerebral cortex, hippocampus, hypothalamus, substantianigra, mid-brain tegmcntal region and the pontine and medullary periventricular regions. No senile plaques were evident. Granulo-vacuolar change was demonstrated in occasional degenerate nerve cells. Conditions in which neurofibrially change has been described include Alzheimer’s disease, senile dementia, post-encephalitic Parkinsonism, Pick’s disease, sporadic motor neurone disease, infantile neuroaxonal dystrophy, vincristine neuropathy, Down’s syndrome, Steel-Richardson-Olszewski syndrome, olivary hypertrophy, various experimental conditions, and Guam-Parkinsonian dementia. The distribution of the pathological findings in these cases most closely resembled that in Guam-Parkinsonian dementia although clinically our patients did not show Parkinsonian features. In most of the above diseases neurofibrillary change is considered to be due to intraneuronal masses of closely packed twisted tubules which displace normal cell organelles. The mechanism by which thcse twisted tubules develop is not known. Electron microscopy was not performed in the authors’ cases. It has been postulated that the Guam-Parkinsonian ementia is caused by a viral infection in a person who is genetically susceptible and the authors considered a similar aetiology possible in their cases.
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Jan 1, 1981
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