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H Kurisaki

Publications and source records attributed to H Kurisaki.

At least 19 recordsLinked to original sources
PubMed1998

Loss of serotonin-containing neurons in the raphe of patients with myotonic dystrophy: a quantitative immunohistochemical study and relation to hypersomnia.

Hypersomnia occurs frequently in patients with myotonic dystrophy (MyD). We performed a quantitative immunohistochemical study of serotonin (5-HT)-containing neurons linked to hypersomnia in the dorsal raphe nucleus (DRN) and the superior central nucleus (SCN) in 8 patients with MyD, 5 of whom showed hypersomnia, and in 12 age-matched controls. The densities of 5-HT neurons in the DRN and the SCN were significantly lower in MyD patients with hypersomnia than in MyD patients without hypersomnia and controls. These data suggest that the loss of 5-HT neurons of the DRN and the SCN is associated with the presence of hypersomnia in MyD.

Aged↗
PubMed1997

Intracytoplasmic inclusion bodies of the substantia nigra in myotonic dystrophy. Immunohistochemical observations.

We recently reported a significantly higher incidence of intracytoplasmic inclusion bodies (IIBs) of the substantia nigra in patients with myotonic dystrophy (MyD) than in age-matched controls. The changes are, per se, not specific, since a small percentage of disease and normal controls also showed similar inclusions. To elucidate the pathological significance of the inclusion in MyD, we studied immunohistochemical characteristics of IIBs of the substantia nigra in eight patients with MyD. Many IIBs showed moderately intense immunoreactivity for ubiquitin, microtubule-associated protein (MAP) 1 and MAP 2. However, the IIBs did not react with any of the following: anti-neurofilament protein antibodies (Abs) (68, 160 and 200 kDa), anti-neuron-specific enolase antibody (Ab), anti-tau Ab, anti-tubulin Abs (alpha and beta), anti-paired helical filament Ab, anti-actin Ab, anti-phosphorylated epitope of neurofilaments Ab, anti-synaptophysin Ab, anti-myelin basic protein Ab, anti-actin Ab and anti-glial fibrillary acidic protein Ab. Our results suggest that IIBs of the substantia nigra in MyD are related to an alteration of neuronal cytoskeleton metabolism affecting microtubular proteins in conjunction with activation of ubiquitin proteolytic systems.

Aged↗
PubMed1997

Expression of dentatorubral-pallidoluysian atrophy (DRPLA) proteins in patients.

The genetic defect dentatorubral-pallidoluysian atrophy (DRPLA) is caused by expansion of a CAG trinucleotide repeat. The mutant gene is translated into protein whose electrophoretic mobility correlates to the number of expanded CAG trinucleotide repeats, indicating that the protein carries an expanded glutamine repeat. Using two polyclonal antibodies raised against the DRPLA gene product in immunoblotting, we determined the untruncated DRPLA proteins, and showed that the amounts of mutant and wild-type DRPLA proteins were similar in DRPLA brain tissues and lymphoblastoid cells, suggesting that regulation of the level of translation of the DRPLA gene is not central to the development of the disease.

Adult↗
PubMed1997

Brain regional differences in the expansion of a CAG repeat in the spinocerebellar ataxias: dentatorubral-pallidoluysian atrophy, Machado-Joseph disease, and spinocerebellar ataxia type 1.

Three autosomal dominant spinocerebellar ataxias, dentatorubral-pallidoluysian atrophy (DRPLA), Machado-Joseph disease (MJD), and spinocerebellar ataxia type 1 (SCA1), are associated with the expansion of a CAG repeat in the respective genes. To investigate the association between CAG repeat expansion and neuropathological findings, we analyzed several brain regions from 9 cases of DRPLA, 3 cases of MJD, and 1 case of SCA1. We found that the expanded alleles were smaller in the cerebellar cortex than in other brain regions, such as the frontal cortex, in these three diseases. The discrepancy in the expanded CAG repeat length between cerebellar cortex and other tissues was most prominent in DRPLA, and especially in cases of adult-onset DRPLA. A significant correlation was found between the age at onset of DRPLA and the size of the CAG repeat expansion. Cerebella of DRPLA patients were microscopically dissected into three layers, the molecular and granular layers and the white matter, which were analyzed separately. The lower level of CAG repeat expansion in DRPLA cerebella was representative of CAG repeat expansion in the granule cells. The microdissected samples of the granular layer of the hippocampal formation, which is densely packed with neuronal cells, revealed that the degree of CAG repeat expansion in this layer was similar to that in the cerebellum. These observations suggest that granule cells in the cerebellum and hippocampus have low levels of CAG repeat expansion, and that other types of cells exhibit a higher level of CAG repeat expansion, in spinocerebellar ataxias.

Adolescent↗
PubMed1996

Immunohistochemical study of intracytoplasmic inclusion bodies of the thalamus in myotonic dystrophy.

Intracytoplasmic inclusion bodies of the thalamus in eight patients with myotonic dystrophy (MyD) were studied immunohistochemically. The intracytoplasmic inclusion bodies of the thalamus (thalamic inclusions, TIs) were strongly immunostained with anti-ubiquitin antibody (Ab) and some of them were mildly stained with anti-microtubule associated protein 1 (MAP 1) and anti-MAP 2 antibodies. However, TIs did not react with any of the following: anti-neurofilament protein Ab, anti-tau Ab, anti-paired helical filament Ab, anti-tubulin Abs (alpha and beta), anti-neuron-specific enolase Ab, anti-glial fibrillary acidic protein Ab, anti-synaptophysin Ab, anti-myelin basic protein Ab, anti-actin Ab and anti-phosphorylated epitope of neurofilaments Ab. Thus, our study demonstrates the unique immunohistochemistry of TIs in MyD which differentiates them from other intracytoplasmic inclusions in various neurodegenerative disorders.

Aged↗
PubMed1996

Neuronal loss in the medullary reticular formation in myotonic dystrophy: a clinicopathological study.

Respiratory insufficiency occurs frequently in patients with myotonic dystrophy (MyD). We have performed a quantitative study of neurons linked to respiratory function in the dorsal central medullary nucleus (DCMN), the ventral central medullary nucleus (VCMN), and the subtrigeminal medullary nucleus (SMN) in seven patients with MyD and eight age-matched controls. Alveolar hypoventilation of the central type occurred in three of the MyD patients but not in the remaining MyD patients or controls. The densities of neurons of the DCMN, the VCMN, and the SMN in MyD patients with hypoventilation were significantly lower than in MyD without hypoventilation and controls. These data suggest the neuronal loss of the DCMN, VCMN, and SMN is associated with the presence of hypoventilation in MyD and may be an important feature of MyD.

Aged↗
PubMed1995

Neuronal cell loss in the dorsal raphe nucleus and the superior central nucleus in myotonic dystrophy: a clinicopathological correlation.

A quantitative study of neurons in the dorsal raphe nucleus (DRN) and the superior central nucleus (SCN) was performed in seven patients with myotonic dystrophy (MyD), five of whom showed hypersomnia, and in eight age-matched controls. The densities of neurons in the DRN and the SCN were significantly lower in MyD patients with hypersomnia than in MyD patients without hypersomnia and control subjects. There was an appreciable positive correlation in the density of neurons between the DRN and the SCN in all MyD patients. These data suggest that the neuronal loss of the DRN and the SCN is associated with the presence of hypersomnia in MyD.

Aged↗
PubMed1995

Myotonic dystrophy with alveolar hypoventilation and hypersomnia: a clinicopathological study.

We present a case of myotonic dystrophy accompanied by alveolar hypoventilation and hypersomnia. Case history, pulmonary function tests, polygraphic recording, and multiple sleep latency test, concomitant with a restrictive ventilatory abnormality, suggested a central origin of alveolar hypoventilation and hypersomnia in our case. The most significant neuropathological findings were in the tegmentum of the brain stem. Severe neuronal loss and gliosis were observed in the midbrain and pontine raphe, particularly in dorsal raphe nucleus and superior central nucleus. Pontine and medullary reticular formation also showed a marked cell loss and fibrillary gliosis. The alveolar hypoventilation and the hypersomnia in our case may be attributed to these morphological abnormalities, and would appear to be central in nature.

Disorders of Excessive Somnolence↗
PubMed1995

A new familial disorder presenting with amyotrophic lateral sclerosis-like manifestation: a clinicopathological study.

We studied a family in which three siblings had an identical clinical feature indistinguishable from familial amyotrophic lateral sclerosis (ALS), consisting of progressive generalized neurogenic muscular atrophy with hyporeflexia and normal sensations beginning in the fourth decade. The duration of illness was about 4 years in all affected members. Autopsy of one patient revealed multiple foci of spongy degeneration in the white matter of the spinal cord, brain stem, cerebellum, and the thalamus, characterized by vacuoles of various size, foamy macrophages and degenerating swollen axons. These changes were most marked in the spinal cord, where there was neither pyramidal tract involvement nor neuronal loss in the anterior horn. The pathological findings were different from those of ALS. A similar disease affected the siblings' mother, suggesting an autosomal dominant inheritance. The disease in the kindred, therefore, appears to be a unique hereditary disorder.

Amyotrophic Lateral Sclerosis↗
PubMed1993

Carcinomatous neuropathy associated with hepatic cell carcinoma: an autopsy case report.

A 76-yr-old male patient with carcinomatous neuropathy associated with hepatic cell carcinoma, whose initial symptom was deep sensory disturbance followed by muscle weakness is described. The onset was subacute, followed by slow progression. Sural nerve biopsy, as well as electrophysiological examinations, revealed severe axonal degeneration without any evidence of demyelination. The autopsy findings were similar to findings described in the literature on carcinomatous neuropathy. Although carcinomatous neuropathy is usually associated with lung cancer, this report describes an association with hepatic cell carcinoma. The patient also had motor nerve involvement with positive serum anti-GM1 ganglioside antibody which decreased after immunosuppressant therapy in parallel with recovery of muscle weakness. The anti-GM1 ganglioside antibody may be involved in the pathogenesis of motor disturbance in the present case.

Aged↗
PubMed1993

[A case of rabbit syndrome--its unique pharmacological feature].

A 61-year-old woman developed cerebellar ataxia as an initial symptom when she was 52 years old. Her neurological symptom was gradually followed by autonomic nervous system disturbance, pyramidal sign, and rigidity. She was diagnosed as suffering from multiple system atrophy (MSA), when she was 53 years old. Magnetic resonance imaging revealed atrophy of the cerebellum and brainstem. She was accompanied by rabbit syndrome (RS), when she was 61 years old. She had not been given any neuroleptics, which might produce RS, before she developed RS. Her regular involuntary movement was localized in lips, and its frequency was about 3 Hz. While sleeping, she did not have the involuntary movement. We had a chance to conduct pharmacological examination on RS. The administration of atropine or trihexyphenidyl did not change her symptom of RS, and the intravenous injection of levodopa deteriorated the movement. Haloperidol, sulpiride, or chlorpromazine was significantly effective on her involuntary movement of RS. These results indicated that our patient had the supersensitivity in her dopamine receptor. Such supersensitivity might result from the denervation in MSA, because she had not been administered any neuroleptics. RS is generally considered to be a kind of extrapyramidal sign, and we have not been aware of any report about RS, which levodopa deteriorated and neuroleptics improved. The mechanism of her symptom is discussed.

Antipsychotic Agents↗
PubMed1993

[A case of multiple system atrophy presenting a regular involuntary movement of the neck muscles synchronous with respiration].

A patient with multiple system atrophy developed a regular, rhythmic involuntary movement of the neck muscles which appeared synchronous with respiration and could be described as "rocking-of-the-head". He had been treated with antiparkinsonian drugs such as L-dopa, L-dopa/carbidopa, amantadine hydrochloride and trihexiphenidyl hydrochloride for approximately one year. A fluoroscopic study assured that the involuntary "rocking-of-the-head" movement synchronized with the diaphragmatic up-and-down movement. A polygraphic study showed that the accelerometer curve which reflected the rocking movement of the head oscillated at approximately 0.7 Hz and synchronized with the surface EMG discharges of the right sternocleidomastoid muscle and the nasal flow curve. This involuntary movement was seen almost all day long as well as asleep but severest usually in the afternoon. Discontinuation of trihexiphenidyl hydrochloride alone made the involuntary movement less severe but never suppressed it completely. Resumption of the drug made the involuntary movement as severe as it had been. Discontinuation of L-dopa, L-dopa/carbidopa and amantadine hydrochloride was followed by disappearance of the involuntary movement in a day or so, in spite of continued intake of trihexiphenidyl hydrochloride. Rigidity and bradykinesia induced by discontinuation of these drugs, however, made it necessary to resume them. As a result the involuntary movement again exacerbated.

Amantadine↗
PubMed1993

[Natural type interferon-alpha (Namalwa) therapy for multiple myeloma complicated by hemolytic anemia].

A 45-year-old woman with Bence-Jones type multiple myeloma was treated with natural type alpha-interferon (Namalwa interferon), 3 million IU every other day subcutaneously. After about 5 months, she developed hemolytic anemia. However, screening tests for autoantibodies, including direct and indirect antiglobulin (Coombs' tests), were negative. This report is the first case in which hemolytic anemia appeared to be caused by natural type alpha-interferon. It is likely that interferons will be used in treating increasing numbers of patients and that more patients will develop this complication.

Anemia, Hemolytic↗
PubMed1992

Japanese crossed Wernicke's aphasia.

A right-handed Japanese man with no personal or family history of left-handedness developed severe Wernicke's aphasia, a mild constructional disorder, and slight left hemiparesis. MRI revealed infarction in the territory of the righ middle cerebral artery, including areas homologous to Broca's and Wernicke's areas. The cerebral blood flow in these areas remained diminished even after language activation. The most likely explanation is that language production occurred in the left Broca's area, while language comprehension occurred in the right Wernicke's area (a dissociated aphasia).

Aphasia, Wernicke↗
PubMed1992

[Isospora belli infection in a patient with adult T-cell leukemia].

An adult T cell leukemia (ATL) accompanied with Isospora belli infection was described. A 65-year-old male was admitted to our hospital because of a two month history of watery diarrhea. On admission, physical examination showed slight pallor but no detectable superficial lymphadenopathies. Hepatosplenomegaly was not observed. Laboratory examination revealed a leukocyte count 5,500/microliters with 10% abnormal lymphoid cells. A majority of the abnormal lymphoid cells expressed both CD 4 and CD 8 antigens. The patient was diagnosed as chronic ATL, since anti-HTLV-1 antibody in his serum and monoclonal integration of HTLV-1 proviral DNA in his peripheral mononuclear cells were detected. Isospora belli was found in his feces thereafter, and trimethoprim/sulfamethoxazole was effective for diarrhea. In Japan, there have been only 9 reported cases of lymphoproliferative disorders (including five ATL patients) accompanied with Isospora belli infection. From the descriptions in those reports, these 9 cases might all be ATL patients.

Aged↗
PubMed1991

[Chronic dementia in Parkinson disease treated by long-term administration of anticholinergic drug--evaluation of neuropsychological test, PET, and SPECT].

It is known that the anticholinergic drug causes acute psychosis and memory disturbance, which may be due to cholinergic dysfunction. But it has not been well described that the anticholinergic drug causes chronic dementia after long administration. We describe six cases of chronic dementia, which is thought to be induced by anticholinergic drug. We conducted detailed neuropsychological and neuroradiological examination in these six cases. All of them were Parkinson's disease, and had used anticholinergic drug for more than six months without any side effect, and then their initial symptom of dementia appeared, followed by chronic dementia. All cases recovered reversibly from dementia within a few weeks after stoppage of taking anticholinergic drug. And any of our cases had no possibility in the cause of chronic dementia other than the side effect of anticholinergic drug. Detailed neuropsychological examinations (WAIS, WMSR, and so on) were conducted in all cases, SPECT was in two cases, and PET was in one case, before and after stoppage of the anticholinergic drug. And in all cases, brain MRI was conducted. The indices of attention, verbal memory, visual memory, and delayed recall were significantly elevated after stoppage of drug. And both verbal IQ and performance IQ were increased after stoppage of drug, too. SPECT and PET revealed significant improvement, which showed diffuse and generalized change, not localized change.

Aged↗