Development of an I-metaiodobenzylguanidine Myocardial Three-Dimensional Quantification Method for the Diagnosis of Lewy Body Disease.

Objectives: We recently developed a new uptake index method for I-metaiodobenzylguanidine (I-MIBG) heart uptake measurements by using planar images (radioisotope angiography and planar image) for the diagnosis of Lewy body disease (LBD), including Parkinson's disease (PD) and dementia with Lewy bodies (DLB). However, the diagnostic accuracy of the uptake index was approximately equal to that of the heart-to-mediastinum ratio (H/M) for the discrimination of the LBD and non-LBD patients. A simple and pain-free uptake index method using I-MIBG SPECT images by modifying the uptake index method may show better diagnostic accuracy than the planar uptake index method.

Uptake Index of I-metaiodobenzylguanidine Myocardial Scintigraphy for Diagnosing Lewy Body Disease.

Objectives: Iodine-123 metaiodobenzylguanidine (I-MIBG) myocardial scintigraphy has been used to evaluate cardiac sympathetic denervation in Lewy body disease (LBD), including Parkinson's disease (PD) and dementia with Lewy bodies (DLB). The heart-to-mediastinum ratio (H/M) in PD and DLB is significantly lower than that in Parkinson's plus syndromes and Alzheimer's disease. Although this ratio is useful for distinguishing LBD from non-LBD, it fluctuates depending on the system performance of the gamma cameras.

Distribution of cerebello-olivary degeneration in idiopathic late cortical cerebellar atrophy: clinicopathological study of four autopsy cases.

Late cortical cerebellar atrophy (LCCA) is a neurodegenerative disease which presents with slowly progressive cerebellar ataxia as a prominent symptom and is characterized neuropathologically by a limited main lesion to the cerebellar cortex and inferior olivary nucleus. To elucidate the features of lesions in the cerebellar cortex and inferior olivary nucleus, four autopsy cases suffering from idiopathic LCCA without other cortical cerebellar atrophies, such as alcoholic cerebellar degeneration, phenytoin intoxication, or hereditary cerebellar atrophy including spinocerebellar ataxia type 6, were examined. All affected patients had identical distinct features of cerebellar cortical lesions.

[An autopsy case of miliary brain metastases].

A 57-year-old man was admitted to our hospital with a diagnosis of psychiatric emergency. His symptoms were similar to encephalitis, metabolic encephalopathy or acute depressive psychosis because of poor focal neurological signs. Laboratory examinations, including routine hematological and biochemical investigations, serum vitamin B1 B12 levels, and cerebrospinal fluid obtained by lumbar puncture, were normal.

"Forme fruste" of amyotrophic lateral sclerosis with dementia: a report of five autopsy cases without dementia and with ubiquitinated intraneuronal inclusions.

We clinicopathologically investigated five autopsy cases of ALS without dementia and with ubiquitinated intraneuronal inclusions. The age at onset of symptoms ranged from 52 to 81 years and the duration of the disease was from 10 months to 3 years, 3 months. All five patients initially developed lower motor neuron signs, including bulbar signs, and upper motor neuron signs were found in the middle to late clinical stages, but dementia was not observed in all five cases.

[Cerebral small infarcts of the precentral and postcentral gyri presenting with unilateral monoataxia: a report of two cases].

Two cases of unilateral monoataxia, due to small infarcts in the precentral and postcentral gyri, were reported. A 76-year-old man (case 1), and a 90-year-old woman (case 2) suddenly developed clumsiness of the left upper extremity. Neurological examination revealed cerebellar ataxia in the left upper extremity in both cases.

[Pure akinesia presenting with antecollis].

We reported a 77-year-old woman having pure akinesia who presented with antecollis induced by L-threo-3, 4-dihydroxyphenylserine (L-DOPS). At the age of 70, she noticed increasing difficulty in standing up from a seat and moving. Afterward, she developed gait disturbance with difficulty in initiating walking, frozen gait, and postural instability.

[A trial of biweekly paclitaxel administration in consideration of QOL for advanced or recurrent gynecologic cancer].

At present there is no oral medicine available which is effective for advanced or recurrent case of elderly patients with gynecologic cancer. We report that a low-dose biweekly paclitaxel administration preserves quality of life (QOL) and seems to be "tumor dormancy like" therapy of good compliance with few side effects. A total of 11 cases were in ovarian cancer (5), uterine cancer (3), cervical cancer (2), and uterine sarcoma (1).

[An autopsy case of dementia lacking distinctive histology showing semantic dementia].

We report an autopsy case of dementia lacking distinctive histology (DLDH) showing semantic dementia. At age 47, a Japanese man developed aspontaneity, followed by semantic dementia a few months after the onset. Thereafter he developed disinhibition and the language disturbance, which progressed transcortical sensory aphasia and terminally mixed transcortical aphasia.

A novel L266V mutation of the tau gene causes frontotemporal dementia with a unique tau pathology.

We report a novel mutation of tau (L266V missense mutation in exon 9) which may cause a type of familial frontotemporal dementia. The brain of a patient showed Pick body-like inclusions and unique tau-positive, argyrophilic astrocytes with stout filaments and naked, round, or irregular argyrophilic inclusions with deposits of both three-repeat and four-repeat tau. Recombinant tau with a L266V mutation showed a reduced ability to promote microtubule assembly, which may be the primary effect of the mutation.