An autopsy case of progressive supranuclear palsy with central pontine myelinolysis]

An autopsy case of progressive supranuclear palsy (PSP) associated with central pontine myelinolysis (CPM) is reported. A 73-year-old male patient suffered from gait disturbance for about 5 years. The clinical features were characterized by gradual development of supranuclear ophthalmoplegia, tremor, bradykinesia, rigidity, neck dystonia, dementia and pseudobulbar palsy at the advanced stage of his illness. Treatment with levodopa did not improve his neurological signs and symptoms. PSP or multiple system atrophy was considered as a clinical diagnosis of the patient. He died of pneumonia, acute pancreatitis and liver dysfunction in November 1985. The main neuropathological findings were neuronal loss and gliosis with neurofibrillary tangles of globose type in the globus pallidus, subthalamic nucleus, substantia nigra and dentate nucleus, and at the base of the pons, bilateral and symmetrical demyelination was found. In addition, myelin staining revealed circumscribed pallor in the cerebral white matter. The histologic diagnosis was PSP associated with CPM. An association of PSP with CPM is rare in the elderly and possible etiologic factors of both diseases were discussed.     

Clinical study of spinal dural arteriovenous fistula-with special reference to neurological symptoms and magnetic resonance imaging]

We have evaluated the backgrounds, neurological symptoms, signs, and magnetic resonance images (MRI) in six cases of spinal dural arteriovenous fistula. The subjects were 2 males and 4 females, and the mean age was 67.2 years old. The cardinal subjective symptoms were progressive motor weakness of lower extremities (2 cases), paroxyomal weakness of lower extremities (3 cases), sensory disturbance of polyneuritic type (2 cases), and intermittent claudication (1 case). Administration of beverages and drugs, which might include vasodilators and taking baths, worsened the symptoms and signs in 3 cases and 2 cases respectively. Concerning to MRI findings, high signal intensities or swellings were observed at mid-thoracic area to upper conus of the spinal cord in all cases. These changes, which would be induced by edema caused by high venous pressure, were especially prominent in the central area of the cords. By these mechanisms, sensory disturbance like polyneuritic type caused by the disturbance in deep areas of posterior columns might more prominent than the disorder of spinothalamic tracts placed in superficial areas of cord in the early stage of the disease. These abnormal intensities of MRI were higher than the level of clinical symptoms and signs. The difference was about 6.5 segments. For this reason, the upper border of the spinal cord lesions could not be identified by the neurological symptoms. Although the clinical features and MRI findings are relatively characteristic, flow void signs by high resonance MRI and abnormal blood vessels by spinal angiography will be necessary to diagnose this disease confidently.     

Posterior canal-type ocular tilt reaction caused by unilateral rostral midbrain hemorrhage

A 56-year-old man receiving treatment for hypertension experienced sudden-onset dizziness and was admitted to our hospital. Computed tomography of the brain revealed a small high-density lesion in the right rostral midbrain. Subsequent magnetic resonance imaging revealed a small hemorrhagic lesion in the same position. The patient complained of double vision affecting the right side when viewing a horizontal linear target. Vergence was intact, but vertical gaze was severely restricted downward and moderately restricted upward. Vestibulo-ocular reflex maneuvers did not alleviate the patient's vertical gaze palsy. By 40 days after the incident, his motility had recovered and he could begin to tilt his head to the left. Consecutive Hess tests demonstrated a skew deviation of right hypertropia, which continued for over two years. Funduscopic examination revealed left extorsion but no right intorsion. A diagnosis of posterior canal-type ocular tilt reaction (OTR) was made from these signs. The patient's course indicated that periaqueductal syndrome dominated in the acute stage whilst unilateral symptoms became more apparent in the chronic stage. The rostral interstitial nucleus of the medial longitudinal fasciculus and the posterior commissure were estimated to contain the main causative lesion for the acute symptoms, whereas the interstitial nucleus of Cajal (INC) was implicated in the chronic stage. Posterior canal-type OTR caused by unilateral INC lesion has been reported only rarely.     

Pure agraphia with topographical disorientation caused by right hemisphere lesion

A 69-year-old, right-handed, Japanese male patient presented with pure agraphia with topographical disorientation after hemorrhage in the right parietal lobe. Upon developing cerebral hemorrhage, he was referred to our hospital for close examination of agraphia. There was no paresis or clumsiness in his extremities. His speech was fluent and well-articulated. Neither aphasia nor reading impairments was found, although there was a clear writing impairment with effort and hesitation. His writing of both kanji and kana letters contained additional, absent or deformed strokes or parts. No hemianopia, prosopagnosia, constructional disturbances and dressing apraxia were found. He could recognize familiar buildings or landscapes, but often lost his way around well-known areas. MR images revealed subcortical lesions of precuneus, superior and inferior parietal lobules in the right hemisphere, around the posterior horn of the lateral ventricle. He revealed pure agraphia and topographical disorientation after the right brain haemorrhage, without dementia or personality change. These findings indicate that the right parietal lobe participates in the kinesthetic movements of writing. Some authers have been documented cases of aphasic agraphia or alexia with agraphia caused by right hemisphere damage in dextrals, but pure agraphia caused by the lesion in the right hemisphere is very rare.     

Brainstem in Machado–Joseph disease: atrophy or small size?

Machado–Joseph disease (MJD), one of the most common types of hereditary spinocerebellar degeneration caused by abnormal expansion of the CAG repeat in the MJD1 gene, presents atrophy of the infratentorial structures neuropathologically and neuroradiologically. Although a significant positive correlation has been reported between infratentorial atrophy and the number of expanded CAG repeat units, the exact changing course of brainstem size in the individual case remains to be resolved. We investigated seven cases of genetically confirmed MJD longitudinally by magnetic resonance imaging with observation periods of 4.5–10.6 years. Measurement of the midsagittal areas of infratentorial structures disclosed progressive atrophy of the pontine base and cerebellum, which correlated significantly with age, whilst midbrain and pontine tegmentum showed atrophy with no significant progression, suggesting it was better identified as 'small size' and might have mostly been completed before the initial symptoms. Such differences between regions in atrophy progression must be caused by a difference in the neuropathological course.     

Intellectual ability and activity of daily living of centenarians in institutions for the elderly]

The purpose of this study was to assess the intellectual ability and activity of daily living (ADL) of 12 centenarians in institutions for the elderly and to compare them with individuals in the 62-99 age group. At the time of our study, 66.7% of the centenarians were severely demented, three quarters of them suffering from Alzheimer's type dementia and the other one quarter the mixed type. There were qualitative differences between non-demented centenarians and the demented elderly in general, particularly in regard to understanding of surrounding objects and the presence or absence of mental symptoms indicating intellectual deterioration. A total of 50% of the centenarians were bedridden, but 41.7% of them could eat without assistance. Intellectual ability and ADL directly decreased with aging. We think centenarians do not present a special case and our clinical observations suggest a continuous process of aging. Five of the centenarians recently died and were autopsied. The agreement rate between clinical diagnoses and pathological findings with respect to dementia was 80%.