[An autopsy case of ataxic form of Creutzfeldt-Jakob disease].

An autopsy case of ataxic form of Creutzfeldt-Jakob disease (Brownell and Oppenheimer, 1965) was reported. The patient, a 71-year-old male, noticed ataxic gait at the beginning of June in 1988, and was admitted to the Hiroshima City Hospital for the neurological examination at the end of June. He showed ataxia of the left arm and legs and diplopia. Gradually he became delirious at night. On July 16, tremor-like involuntary movement of the left hand was noticed. On July 20, he became somnolent and doubly incontinent. Myoclonus and paratonic rigidity were also observed. The EEG showed periodic synchronous discharge on July 25. The brain CT and MRI were normal. He became apallic gradually and died on October 28. The duration of illness was 5 months. At autopsy, brain weighed 1000gr. Cerebral atrophy and slight enlargement of the ventricles were observed. The cerebellum was also slightly atrophic. Histologically, the destruction of the cerebral cortical layer, slight sieve-like spongy state of the neuropil, slight neuronal loss of the thalamus and sieve-like spongy state of the striatum were observed. The cerebellar lesion was the most severe, where granular cell loss and gliosis of the cortex were observed.

[A case of Binswanger disease with numerous diffuse plaques in the neocortex].

A case of Binswanger disease with numerous diffuse plaques in the neocortex was reported. This male patient had a previous history of hypertension and myocardial infarction. From the age of 60, he developed dysarthria, bradykinesia, marche à petit pas and falling down. Neurological examination at his first admission disclosed muscular rigidity and increased jaw and deep tendon reflexes, but dementia was not found. Brain CT showed moderate brain atrophy and EEG consisted of slow wave dysrhythmia. He was diagnosed of Parkinsonism and treatment started without effects. During his second admission for the treatment of myocardial infarction, at the age of 64, delirium developed. Progressive dementia began and finally he was confined to bed. From the age of 69, spontaneous speech became almost lost. Contracture of the extremities, increased deep tendon reflexes and force grasping were noted. Brain CT showed symmetrical low attenuation in the frontal and parietal white matter with moderate dilatation of the lateral ventricles. At the age of 70, he died of general prostration about ten years after the initial symptoms. Neuropathological findings: Macroscopic findings: The brain weighed 1300 g. Atherosclerotic changes of the large arteries at the base of the brain were moderate. Coronal sections of the brain showed moderate enlargement of the lateral ventricles with multiple small lacunes in the basal ganglia. Microscopic findings: Bilateral diffuse demyelination of the white matter with sparing of the U-fibers was noted. Holzer stain revealed fibrillary gliosis in the left parietal and occipital white matter. Marked adventitial fibrosis of the deep white matter arteries and terminal stages of hyalinosis of the perforating arteries were found. Basal ganglia showed status lacunaris. Bilateral pyramidal tracts were atrophic secondly.(ABSTRACT TRUNCATED AT 250 WORDS)

[A manifesting carrier of Duchenne muscular dystrophy presenting mosaic distribution of dystrophin negative and positive muscle fibers].

A 25-year-old female patient with an approximate 10-year-history of slowly progressive muscle weakness was diagnosed as a manifesting carrier of Duchenne muscular dystrophy (DMD) because her muscle biopsy showed scattered fibers with no dystrophin on immunohistochemical staining. She had no family history of neuromuscular disorders. She was in good health until about 14 years of age, when she developed muscle weakness and atrophy of the extremities with slow aggravation. On admission at the age of 25 years, she had asymmetrical muscle atrophy in the lower extremities; the left femur, right femur, left crus, and right crus measured 36.0, 40.5, 31.5, and 35.5 cm in circumference, respectively. However, the muscle weakness of the extremities was symmetrical with no laterality, and the proximal muscles in the lower extremities were predominantly affected to 3+/5 MMT test. She walked with a mild wadding manner and stood up with Gower' maneuver. Deep tendon reflexes of the extremities were almost normoactive with no pathologic reflexes. As to laboratory findings, serum enzymes of muscular origin were elevated; GOT was 44 IU/l, GPT 60 IU/l, LDH 829 IU/l, CK 4238 IU/l, and aldolase 31 SL units. The electromyogram showed myopathic changes mixed with some neurogenic components. Peripheral nerve conduction velocity was normal. A computed tomography of the skeletal muscles showed more marked atrophy and lower density in the left lower extremity than in the right. The biopsied left gastrocnemius muscle demonstrated a marked variation in fiber size with some necrotic and regenerating fibers. On immunohistochemical stain with anti-dystrophin antibody, the dystrophin negative fibers were scattered among positive fibers in a mosaic distribution.

[Acetylcholinesterase activity in CSF in senile dementia of Alzheimer type, vascular dementia, and Parkinson's disease].

Acetylcholinesterase (AChE) activity was measured in cerebrospinal fluid (CSF) from 25 patients with senile dementia of Alzheimer type (SDAT), 11 patients with vascular dementia (VD), 26 patients with Parkinson's disease (PD), and 30 normal controls. AChE activity also was measured in 46 normal subjects whose ages ranged from 15 to 85 to evaluate the effect of age on AChE activity. CSF AChE activity for the SDAT, VD and PD groups showed no significant difference compared with the value for the control group. However, there were significant decreases in CSF AChE activity in the VD and PD groups with the development of ventricular enlargement. There was no significant correlation between CSF AChE activity decrease and ventricular enlargement in the SDAT group. AChE activity increased significantly over the age range of 15 to 85. These results suggest that, although CSF AChE activity is not a useful parameter in the diagnosis of dementia, it may be a marker indicating abnormalities of the intracerebral cholinergic system during the process of cerebral atrophy.

A case of progressive supranuclear palsy with fibrillary gliosis of the midbrain and pontine reticular formation.

An autopsy case of progressive supranuclear palsy with onset at age 53 and a clinical course of 7 years is reported. The clinico-pathological characteristics of the case were as follows: Fibrillary gliosis of the midbrain and pontine reticular formation corresponded clinically to personality changes: The patient had formerly been irascible and became extremely mild-mannered. He showed lack of spontaneity and initiative and slowing of thought processes, taking him longer than normal to find words and ideas. Gliosis of the nucleus ventralis lateralis of the thalamus was secondary to the degeneration of the cerebellar dentate nucleus and the globus pallidus from which they receive fibers. Neurofibrillary tangles were found in the cerebral cortex.

[A study on mental symptoms developing during the treatment of Parkinson's disease (author's transl)].

UNLABELLED: We studied on mental symptoms which developed during the treatment of Parkinson's disease. Hallucination, delusion, and/or delirious state were observed in 23 (22.8%) of 101 cases. The cases with mental symptoms were studied for the incidence, detail of the symptoms, and the factors involved in their development. RESULT: 1) Mental symptoms were observed in 23 patients and the incidence tended to be highest in the patients in their sixties. 2) In classification of the cases with mental symptoms, 12 cases showed the change of visual experience as main symptom and 3 cases had delusion. In these two cases, disturbance of consciousness was minimum, if any. Eight cases showed delirious state. 3) As for mental symptoms, visual hallucination was most frequently observed. 4) Complication of hypertension and history of depressive state were more frequently observed in the cases with mental symptoms than in those without. 5) Mean daily dose of L-dopa upon the onset of mental symptoms was less than mean daily maximum dose in the cases without mental symptoms. 6) The cases with cerebral atrophy in CT had a tendency to develop mental symptoms. 7) The cases with abnormal EEG were liable to develop mental symptoms. 8) The cases with mental symptoms were more often complicated by dyskinesia than those without. CONCLUSION: The incidence of mental symptoms during the treatment of Parkinson's disease tended to be high in the aged patients who had all or some of these findings as previous history of psychiatric illness, hypertension, cerebral atrophy and abnormal EEG.