Jun-ichi Shiota

Prosopagnosia: A Clinical and Anatomical Study of Four Patients

We tested in great detail face perception and face memory in four cases of prosopagnosia. Three of them showed deficits of face perception and/or of familiar faces memory. There was one prosopagnosic patient, however, who showed no abnormality in face perception and discrimination and could recall familiar faces. This case suggests that a form of prosopagnosia may exist, which is due to the disconnection between face perception and face memory. X-ray CT, MRI and PET studies revealed that two patients did not have damage of the left hemisphere, supporting the view that prosopagnosia can arise from unilateral right-sided lesion.

Argyrophilic grain disease presenting with frontotemporal dementia: a neuropsychological and pathological study of an autopsied case with presenile onset.

A right‐handed Japanese man with no consanguinity exhibited personality changes, speech disorder and abnormal behaviors, such as stereotypical, running‐away, environment‐dependent, and going‐my‐way behaviors, since the age of 49 years. At age 52 years, neuropsychological examination revealed frontal lobe dysfunctions, mild memory impairment, and transcortical sensory aphasia. MRI showed symmetrical severe atrophy of the anterior part of the temporal and frontal lobes. The clinical diagnosis was FTD. He died at age 54 years after a clinical illness of approximately 5 years. Numerous argyrophilic grains were observed throughout the limbic system, temporal lobe, frontal lobe and brainstem. In addition, there were many tau‐positive neurons and glial cells. These findings are all compatible with argyrophilic grain disease (AGD). Our case, however, is atypical AGD because of the young age of onset of the disease and sharply circumscribed cortical atrophy exhibiting severe neuronal loss and gliosis. Our case, together with some other similar cases of atypical AGD, gives rise to the possibility that this type of AGD would constitute a part of pathological background of FTD.

An autopsy case of frontotemporal dementia with severe dysarthria and motor neuron disease showing numerous basophilic inclusions.

We report a clinicopathological study of a patient suffering from frontotemporal dementia (FTD) with severe dysarthria and concomitant motor neuron disease (MND). The patient was a 52‐year‐old woman with almost simultaneous emergence of severe dysarthria and FTD. The severe dysarthria subsequently evolved into anterior opercular syndrome. Motor neuron signs then emerged, and the patient developed akinetic mutism approximately 2 years after the onset of the disease. The patient died of pneumonia after a 7‐year clinical illness. Pathologically, severe and widespread degeneration in the frontal and temporal lobes, including the anterior opercular area, limbic system, basal ganglia, spinal cord and cerebellum, and frequent ubiquitin‐ and tau‐negative basophilic inclusions were observed. The pyramidal tracts and anterior horns of the cervical cord also showed marked degeneration. Cases showing basophilic inclusions reported so far have been divided into two groups: early onset FTD and MND with basophilic inclusions. Our case presented clinicopathological features of both FTD and MND, which suggests that cases showing basophilic inclusions may constitute a clinicopathological entity of FTD/MND.

An autopsy case of Marchiafava-Bignami disease with peculiar chronological CT changes in the corpus callosum: neuroradiopathological correlations

We conducted a comparative analysis of the autopsy findings in a case of Marchiafava-Bignami disease with the X-ray computed tomography (CT) and magnetic resonance imaging (MRI) findings obtained while the patient was alive. Histologically, massive deposits of hemosiderin were demonstrated in the astrocytes and macrophages located in the walls of the callosal cystic lesion, suggesting that the transient increase in density during the subacute stage observed on X-ray CT is secondary to significant hemorrhage.

Severe cortical involvement in MV2 Creutzfeldt-Jakob disease: an autopsy case report.

MV2 type sporadic Creutzfeldt–Jakob disease (sCJD) is reported to have a long duration and marked involvement of the cerebral deep gray matter. We describe an autopsied long‐surviving sCJD case of MV2. In the early stages, the patient exhibited memory impairment, attention deficit and semantic memory disorder. Diffusion‐weighted MRI showed abnormal hyperintensity signals along the cerebral cortex, sparing the thalami and basal ganglia. Pathological observations included: severe spongiosis throughout the cerebral cortex, several kuru plaques and plaque‐like PrP deposits in the cerebellum, with only minimal degeneration in the thalami and basal ganglia. Our case suggests that MV2 has a wide clinicopathological spectrum, which ranges from “VV2” to “MM2” type.

Another piece in the jigsaw: a case report of prosopagnosia with symptomatological, imaging and post mortem anatomical evidence.

This study was supported by a grant from the Tamagawa University Center of Excellence under the Ministry of Education, Culture, Sports, Science and Technology (MEXT), a Grant-in-Aid for Scientific Research on Innovative Areas, “Face perception and recognition” from MEXT (No. 21119518), a Showa University Grant-in-Aid for Innovative Collaborative Research Projects and a Special Research Grant-in-Aid for the Development of Characteristic Education from MEXT.

Lewy body-free nigral degeneration—a case report

A 70-year-old Japanese woman developed progressive, dopa-responsive parkinsonism consisting of akinesia, resting tremor, rigidity, and postural instability. Neuropathological examination revealed a marked loss of nigral neurons, but no Lewy bodies (LBs) were observed. Lewy bodies were also absent from their usual site, with the exception of a small number seen in the dorsal motor nucleus of the vagus nerve (DVN) and sympathetic ganglion. We propose that our case and several similar reported cases represent Lewy body-free nigral degeneration.

Pseudobulbar Dysarthria in the Initial Stage of Motor Neuron Disease with Dementia: A Clinicopathological Report of Two Autopsied Cases

We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was dysarthria, involving low pitch, slow rate, hypernasality and hoarseness. Other than these findings, effortful speech, sound prolongation and initial difficulty were observed. Moreover, repetition of multisyllables was severely impaired compared to monosyllables. Repetition and comprehension of words and sentences were not impaired. Neither atrophy nor fasciculation of the tongue was observed. Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed. The bilateral pyramidal tracts also showed severe degeneration. However, the nucleus of the hypoglossal nerve showed only mild degeneration. These findings suggest upper motor neuron dominant motor neuron disease with dementia. We believe the results indicate a subgroup of motor neuron disease with dementia whose initial symptoms involve pseudobulbar palsy and dementia, and which shows rapid progression to mutism.

Early clinical and radiological diagnosis of sporadic Creuzfeldt-Jakob’s disease (sCJD) — a case study of pathologically-proved sCJD MV2 —

Background Early clinical and radiological features of sCJD with long clinical illness have not been clarified yet. Subject An exceptioal case of pathlogically-proved sCJD MV2 exhibiting severe status spongiosus in the entire cerebral cortex and kuru-plaque with only mild degeneration in the thalami and basal ganglia. Total clinical illness was about four years. Method We compared clinical and radiological features chronologically. Results At the initial radiological study, about half a year after the initial symptom (forgetfulness), diffusion weighted magnetic resonance images (DW-MRI) revealed abnormal high signal along the occipito-parietal lobe cortex with