Masaya Oda

Panencephalopathic type of Creutzfeldt-Jakob disease: primary involvement of the cerebral white matter

Eight necropsy cases of a “panencephalopathic” type of Creutzfeldt-Jakob disease (CJD) in the Japanese are reported. The reasons why this type should be discussed separately from other types of CJD are that there is primary involvement of the cerebral white matter as well as the cerebral cortex, and that the white matter lesion of one Japanese human brain with CJD similar to the present group has been successfully transmitted to experimental animals.

A fulminant case of Guillain-Barré syndrome: topographic and fibre size related analysis of demyelinating changes.

In a necropsy case of early fulminant Guillain-Barré syndrome, demyelinating changes were observed throughout the peripheral nervous system, most conspicuous in the spinal nerve roots. The central/peripheral nervous system transition regions and most proximal zones of the roots escaped damage. In some root areas there were widespread early changes in myelin sheaths in the absence of significant infiltrates of inflammatory cells. In the fibre size analytical study, small myelinated fibres were preferentially involved.

Loss of large myelinated nerve fibres of the recurrent laryngeal nerve in patients with multiple system atrophy and vocal cord palsy.

OBJECTIVES: Vocal cord palsy seen in some patients with multiple system atrophy may result from neuronopathy of the recurrent laryngeal nerve. METHODS: Six controls and six patients with multiple system atrophy, four with and two without vocal cord palsy were studied. The number of myelinated nerve fibres were counted and fibre diameter histograms were established for the motor and sensory divisions of the laryngeal branch of the recurrent laryngeal nerve. RESULTS: Although both groups of patients with multiple system atrophy showed selective loss of the myelinated fibres in the motor branch, the change was greater in those with vocal cord palsy than in those without. The small myelinated nerve fibres (diameter < 7 microm) were decreased in number in both multiple system atrophy groups, whereas the large myelinated nerve fibres (diameter < 8 microm) were decreased only in those with vocal cord palsy, and preserved in those without the symptom. CONCLUSION: In multiple system atrophy, the small myelinated fibres innervating the vocal cord are affected first, without obvious clinical signs. The patient develops vocal cord palsy only after the loss of the large myelinated fibres, which mostly comprise the alpha motor axons that innervate the intrinsic laryngeal muscles.

Lewy bodies in progressive supranuclear palsy.

Abstract. Lewy bodies (LBs), whose major component is α-synuclein, are a pathological hallmark of Parkinsons disease (PD) but have rarely been reported in progressive supranuclear palsy (PSP). Whether LBs in PSP represent the aging process or the coexistence of PD remains unclear. We found LBs in 5 of 16 patients with PSP. In 4 patients LBs were distributed widely throughout the brain stem and cerebrum in a pattern similar to that in PD. In the remaining patient one LB was found in the pontine reticular formation. Semiquantitative analysis showed that neuronal loss in the locus coeruleus and the dorsal vagal nucleus was more severe in patients with LBs than in patients without LBs. Double-labeling immunohistochemical studies showed co-localization of α-synuclein and tau in some neurons. Our study suggests that patients who have PSP with LBs constitute a subset of patients with PSP in whom Lewy body disease is also present.

Inflammatory demyelinating polyradiculitis in a patient with acute disseminated encephalomyelitis (ADEM).

A patient with severe acute disseminated encephalomyelitis died 12 days after the first symptom. Necropsy showed widespread severe demyelination in the CNS and some foci of demyelination in the spinal roots. The lesions in the peripheral nervous system were characterised by myelin stripping and the presence of macrophages, being severest in the spinal nerve roots. Some axons were completely demyelinated, whereas the axons themselves were preserved. Pathologically established ongoing demyelination in both CNS and peripheral nervous systems raises the possibility of a shared pathological epitope.

Spinal cord lesions of myelitis with hyperIgEemia and mite antigen specific IgE (atopic myelitis) manifest eosinophilic inflammation

We report the neuropathological findings of spinal cord specimens obtained from two patients who had localized myelitis with hyperIgEemia and mite antigen specific IgE (atopic myelitis). Both cases showed mild spinal cord dysfunction, and the gadolinium-enhanced area of the isolated spinal cord lesion observed on MRI was biopsied, respectively. Neuropathologically, both cases showed many perivascular lymphocyte cuffings associated with disrupted vessels, and the infiltration of eosinophils in the spinal cord lesions. Both myelin and axons were lost in the lesions, which were associated with astrogliosis. These findings suggest that an allergic mechanism may play a role in this condition.

Cerebellar degeneration in hereditary dentatorubral-pallidoluysian atrophy and Machado-Joseph disease

Abstract We examined the mechanism of cerebellar degeneration in brains obtained at autopsy from six cases of hereditary dentatorubral-pallidoluysian atrophy (DRPLA) and six cases of Machado-Joseph disease (MJD), using terminal deoxynucleotidyltransferase-mediated in situ nick end labeling (TUNEL) and immunohistochemistry for apoptosis-related proteins, neurotrophin receptors and glutamate transporters. In three subjects with DRPLA, who developed dementia and cerebellar ataxia at over 50 years of age, the number of Purkinje cells was mildly reduced, TUNEL-positive cells were observed in the molecular layer of the cerebellar cortex, and immunoreactivities for calbindin D28K and excitatory amino acid transporter-1 (EAAT1) were altered in the molecular layer. In addition, all cases of DRPLA showed a reduction of immunoreactivity for EAAT1 in the dentate nucleus. In MJD, augmentation of Bcl-x expression by the Purkinje cells, and increases in Trk B- and GFAP-immunopositive glial cells in the granular layer were observed in half of the cases, whereas immunoreactivity for EAAT-1 was preserved both in the cerebellar cortex and dentate nucleus. One case of MJD showed TUNEL-positive granular cells in the cerebellar cortex. Age-matched control subjects did not show TUNEL-positive cells or immunohistochemical changes in the cerebellum. There were neither TUNEL-positive cells nor alteration of the in situ expression of apoptosis-related proteins in the dentate nucleus in either variant of hereditary spinocerebellar degeneration, although both exhibited grumose degeneration in the dentate nucleus. These findings indicate that latent degeneration in the cerebellar cortex may occur in DRPLA and MJD, in addition to the dentate change, which is the cardinal feature in the neuropathology of these two diseases. The lesion of Purkinje cells and their processes in the molecular layer associated with altered glutamate transport may be important in DRPLA, while the significance of the abnormalities observed in some MJD cases, which might be related to apoptotic mechanism, remains unclear.

Creutzfeldt-Jakob disease with congophilic kuru plaques: CT and pathological findings of the cerebral white matter.

In a patient whose Creutzfeldt-Jakob disease with congophilic kuru plaques that was proved at necropsy, the early brain CT showed low-density areas in the cerebral white matter before cortical atrophy and ventricular enlargement became apparent. Subsequently, there occurred diffuse white matter lucency and severe brain atrophy. At necropsy, there was severe white matter destruction which was more prominent than cortical neuronal loss. Serial CT scans were of great value for demonstrating the early and predominant changes in the cerebral white matter.

Ein Autopsie-Fall von hypoglykämischem Koma bei einer jungen Diabetikerin mit verzögerter Geistes-und Körperentwicklung

SummaryThe autopsy findings of a 27-year-old diabetic woman who had a mental and somatic retardation since childhood (Prader-Labhart-Willi syndrome) and expired of hypoglycemic coma due to the insulin administration 13 days later are reported. The most remarkable changes in the CNS were prominent vascular reaction in the involved areas and fresh necrotic foci in the corpus callosum, optic chiasm, midbrain, pons and other areas, indicating recent disturbances of blood-brain-barrier. Some metabolic-humoral dysfunctions which cosisted in the background of Prader-Labhart-Willi syndrome could play a significant role for the development of the above mentioned fresh changes. Generalized hypoplasia of the endocrine organs, fibrosis of the pituitary posterior lobe, phenomena of the precocious senility in the CNS and an advanced focus of the optic tract, on the other hand, were possibly related to this longstanding syndrome of the present example.