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T Uchihara

Publications and source records attributed to T Uchihara.

At least 73 records · Page 4Linked to original sources
PubMed1994

[Autosomal dominant spinocerebellar degeneration with pigmentary retinopathy in a Japanese family].

We report a pedigree of autosomal dominant spinocerebellar degeneration associated with pigmental retinopathy. The proband is a 75-year-old man. He noticed night blindness at the age of 10 years and a diagnosis of bilateral pigmentary retinopathy was made at age 63. At the age of 65 years, he developed dysarthria and difficulty in walking. At age 69, neurological examination revealed cerebellar signs, and brain CT scans showed mild atrophy of the brain stem and cerebellum. Repeated brain CT scans revealed slight progression of the brain stem and cerebellar atrophy. Molecular genetic studies showed the absence of any mitochondrial DNA mutation at 8993. The father of the proband exhibited cerebellar signs and pigmentary retinopathy. One older brother had cerebellar signs and another had pigmentary retinopathy. To our knowledge, hereditary spinocerebellar degeneration with retinal degeneration is rare in Japan. This study is the first full report on hereditary spinocerebellar degeneration with pigmentary retinopathy in Japan, although an abstract was published by Konishi et al. We also discuss the neuropathological discordance on hereditary olivoponto-cerebellar atrophy with retinal degeneration.

Adult↗
PubMed1993

Action myoclonus induced by visually guided movement.

In order to determine what kind of voluntary movement induces action myoclonus, we gave two siblings with sialidosis two kinds of tasks. When the patients were asked to move their index fingers following a smoothly moving target, action myoclonus became prominent. In contrast, when they were asked to perform the same movements with their eyes closed, they could move their index fingers very smoothly. This shows that action myoclonus was induced by visually guided movement, but not by self-paced movement. Our observations might reflect a disorder of the cerebellum, which controls visually guided movement.

Adult↗
PubMed1993

Circulating autoantibody to mature neurons and astrocytes of humans and some mammals present in a demented patient with autoimmune disorder.

Circulating autoantibody to a 48-kD nuclear protein in neurons and astrocytes of the human and bovine cerebrum were present in the serum of a demented patient with an autoimmune disorder. Other human visceral organs, dorsal root ganglion cells, neuroblastoma and glioblastoma cell lines, and rat cerebrum did not react with the patient's serum. No sera from age-matched controls, including those with Alzheimer's disease, reacted with the 48-kD protein. Only the mature neurons and astrocytes of humans and some mammals express the 48-kD protein. This antibody may be responsible for the patient's demented condition.

Aged↗
PubMed1993

[Corticobasal degeneration: clinico-pathological studies on two cases].

We reported two patients with clinical features of corticobasal degeneration (CBD), one with autopsy observations. Their illness began in their sixties, ran progressive course and ended in death in about five years. The initial symptom was difficulty in manipulating with the left hands. Gradually the left lower limbs and the right limbs were also involved and the left became useless. They developed Parkinsonism, myoclonus, dystonia, hyperreflexia and vertical gaze palsy. They also showed the dysfunction of the fronto-parietal cortices, including grasping reflex, motor neglect, hemispatial neglect, constructional disturbance and cortical sensory loss. Dementia did not manifest until the terminal stage. Neuro-radiological studies demonstrated mild dilatation of the right central sulcus and marked reduction in cerebral blood flow in the fronto-parietal cortices, predominant in the right. Pathological examination in one of the case confirmed the the clinical diagnosis of CBD. There was a neuronal loss affecting second and third layers of the fronto-parietal cortices, especially the right parasylvian region, associated with spongiosis in these laminae and marked gliosis in deeper layers. Swollen, poorly staining (achromatic) neurons were observed in fifth layer. The hippocampus was unaffected. In the substantia nigra, there was extensive loss of pigmented cells. No Pick bodies, senile plaques, neurofibrillary tangles were observed in any region of the brain. The initial manifestations of their illness, the motor disturbances in the left upper extremities, were distinctive features in our patients. We could find no detailed description of these symptoms in the literature. We analysed these manifestations and described the results.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1992

Selective loss of nigral neurons in Alzheimer's disease: a morphometric study.

Loss of neurons from the substantia nigra (SN), which is sometimes observed in Alzheimer's disease (AD), was quantitatively analyzed in 10 cases of presenile AD and 19 age-matched controls. On sections from the upper and lower portions of the SN, the pigmented zone (zona compacta) and the non-pigmented zone (zona reticulata) were delineated, and these zones were partitioned into quarters: medial, mid-medial, mid-lateral and lateral. This approach clarified topographical preference of neuronal depletion in the SN of AD; namely (1) pigmented neurons were more severely affected than non-pigmented neurons, (2) neuronal depletion was more marked in the lower SN (-38%, P less than 0.001), where the pigmented neurons in the medial quarter were most severely affected (-51%, P less than 0.001), (3) in the upper SN (neuronal loss: -21%, P less than 0.01), the pigmented neurons in the mid-medial quarter were most severely affected (-43%, P less than 0.01). These findings suggest that some groups of nigral neurons are primarily involved in presenile AD. Gallyas staining after bleaching of melanin pigments uncovered a large number of neurofibrillary tangles (NFTs) mainly in the pigmented zone, especially in the medial quarter. A large number of NFTs, scarse senile plaques, and substantial depletion of neurons form an unique combination of Alzheimer pathology in the SN not well recognized so far.

Aged↗
PubMed1992

[A case of angiotropic lymphoma diagnosed by adrenal biopsy].

We reported the first case of angiotropic lymphoma diagnosed by adrenal biopsy in Japan. Immunohistochemical study and southern blot hybridization analysis proved it to be B-lymphocyte origin. A 61-year-old man with history of mild hypertension and diabetes mellitus was admitted to our department because of recurrent minor stroke. On admission, general physical findings were normal. Laboratory investigations showed an elevated erythrocyte sedimentation rate and increased serum lactic dehydrogenase (LDH) level. The serial computed tomographic (CT) scan of the brain showed multifocal abnormal density lesions in bilateral hemispheres. Magnetic resonance imaging (MRI) of the brain demonstrated multiple lesions of increased signal intensity in the brainstem and bilateral hemispheres. A subsequent CT scan of the abdomen revealed swelling of bilateral adrenal glands. Adrenal biopsy was performed. Biopsy samples showed the intravascular proliferation of malignant lymphoma cells, non-Hodgkin, diffuse large cell type. These cells had the immunophenotype of a B cell lymphoma (reactive with the antileukocyte common antigen, anti-MB-1, anti-MB-2 and anti-MX-pan B, and unreactive with the anti-MT-1, anti-UCHL, anti-Ki, anti-kappa, anti-lambda and antifactor-VIII). Southern blot hybridization analysis showed monoclonal rearrangements of the immunoglobulin heavy-chain gene, which strongly suggested a B-lymphocyte origin. Thus, a diagnosis of angiotropic lymphoma was made. As soon as chemotherapy was begun, the patient fell into deep coma. A repeat CT scan of the brain was normal. His clinical status gradually deteriorated, and he died 18 months after his initial symptom. Autopsy, which was limited to the body, revealed characteristic systemic intravascular stagnation of lymphoma cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Glands↗
PubMed1991

Radiation myelopathy: significance of gadolinium-DTPA enhancement in the diagnosis.

We described two cases of chronic progressive radiation myelopathy (CPRM), in which magnetic resonance imaging (MRI) was of great value for the diagnosis. Gadolinium-DTPA (Gd-DTPA) enhancement delineated precise lesions responsible for Brown-Séquard syndrome caused by CPRM in both cases. This safe, sensitive procedure will be a requisite study in patients who are suspected of CPRM.

Aged↗
PubMed1991

Jolt accentuation of headache: the most sensitive sign of CSF pleocytosis.

We prospectively examined the clinical signs of 54 febrile patients associated with recent-onset headache. They underwent lumbar puncture (LP) on suspicion of meningitis. The relation of each sign to cerebrospinal fluid (CSF) pleocytosis was estimated. Among 34 patients with pleocytosis, 33 had jolt accentuation (sensitivity: 97.1%), while only 5 of them had neck stiffness or Kernig's sign. Among 20 patients without pleocytosis, 12 had no jolt accentuation (specificity: 60%). We found jolt accentuation to be the most sensitive sign of CSF pleocytosis. If jolt accentuation is noted in a febrile patient associated with recent onset headache, the CSF should be examined even in the absence of neck stiffness or Kernig's sign.

Adult↗
PubMed1991

Recurrent Fisher's syndrome with immunological abnormalities and replicated laterality.

A case of Fisher's syndrome which recurred 10 years apart was reported. Laterality of cranial nerve findings in the initial episode was replicated in the recurrence. Some kind of localized immune sensitization is supposed to play a role in replicating such a laterality. Increased CD4/CD8 ratio and elevated IgE were noted, which suggests immunological behavior in Fisher's syndrome similar to that in Guillain-Barré syndrome.

Ataxia↗
PubMed1990

Selective loss of nigral neurons in Pick's disease: a morphometric study.

Loss of neurons from the substantia nigra (SN), which is often encountered in Pick's disease, was quantitatively analyzed in 13 cases of Pick's disease and 19 age-matched controls. On sections from the upper and lower portions of the SN, the pigmented zone (zona compacta) and the non-pigmented zone (zona reticulata) were delineated, and these zones were partitioned into quarters: medial, mid-medial, mid-lateral and lateral. Neuronal loss was fairly severe and more evident in the upper section of the SN (-40%), especially in the mid-medial and lateral quarters. In the lower section (neuronal loss: -28%), the medial quarter was most severely affected. Non-pigmented neurons were preserved. Fibrillary gliosis was denser in the zona reticulata, where neuronal loss was minimal. These findings revealed a selective vulnerability of nigral neurons according to their topography and pigmentation and suggests the primary involvement of some neuronal groups (especially the pigmented neurons) of the SN in Pick's disease.

Aged↗
PubMed1990

Myoclonus with burning sensation in legs that remits with sympathetic blockade.

Two patients with hitherto unrecognized spells of involuntary movements in legs are described. They occurred on dropping off to sleep, were preceded by a burning sensation and resembled myoclonus. Subcutaneous injection of epinephrine reproduced the spells. Neurogenic bladder of uninhibited type was noted on cystometry. Epidural or sympathetic nerve block of the lumbar region relieved all the symptoms including the neurogenic bladder. A few minutes before the spells, a rise in blood pressure and pulse rate was observed in one of the cases. Pathophysiological resemblance to painful legs and moving toes was pointed out, but more widespread involvement of the spinal cord was suspected.

Adrenocorticotropic Hormone↗
PubMed1990

CSF lymphocyte subsets in aseptic meningitis: dual-labelling analysis with flow cytometry.

In order to characterize the CSF (cerebrospinal fluid) lymphocytes in CNS (central nervous system) inflammation, we examined paired samples of CSF and PB (peripheral blood) of 19 patients with acute aseptic meningitis, performing the dual labelling method on flow cytometry. Significantly higher percentages of CD3+ (T cell), CD4+ (helper-inducer), Leu3a+ Leu15- (cytotoxic-T) and HLA-DR+ CD3+ (activated-T) cells were identified in the CSF than in the PB of these patients. We observed significantly lower percentages of CD19+ (B cell), Leu2a+ Leu18+ (suppressor-inducer) and HLA-DR+ CD3- cells in the CSF than in the PB of these patients. Relative increases in helper-inducer, cytotoxic-T and activated-T cells in the CSF of aseptic meningitis are supposed to represent an active inflammatory process. However, whether these changes are specific or pathognomonic to any disease(s) remains to be solved.

Adolescent↗
PubMed1990

[A case of Hoffmann's syndrome with peripheral neuropathy and various pathological findings in muscle biopsy].

A 58-year-old hypothyroid man developed numbness in the extremities and muscular hypertrophy without pain. Needle electromyography was normal. Nerve conduction study revealed severe entrapment and impaired conduction velocity. Muscle biopsy demonstrated small group atrophy, scattered hypertrophic fibers, interstitial fibrosis and subsarcolemmal vaculoes. These vacuoles were stained dark with NADH-TR. On electron microscopy these vacuoles were filled with aggregated mitochondria and glycogen particles. These myopathological findings have been rarely described in Hoffmann's syndrome or Kocher-Debré-Semelaigne syndrome in the literature. Sural nerve biopsy revealed a loss of myelinated fibers in large diameter and relative increase in small diameter fibers. Teased fiber examination demonstrated segmental demyelination. The nerve biopsy findings indicate slowly progressive axonopathy. In Hoffmann's syndrome, associated peripheral neuropathy may play some role in the manifestation of clinical signs and symptoms including muscle weakness and some sensory disturbance.

Humans↗
PubMed1988

Myoclonic activity associated with cefmetazole, with a review of neurotoxicity of cephalosporins.

We reported a case of a 66-year-old female with uremia who developed myoclonic activity after administration of cefmetazole (4g/2 days). Curiously, the level of the antibiotics (236 micrograms/ml) in the cerebrospinal fluid (CSF) was higher than that in the blood (103 micrograms/ml). Myoclonic activity faded away after cessation of the antibiotics. This is the first case reported developing neurotoxicity associated with intravenous administration of cefmetazole. In addition, the curious pharmacokinetic dynamics of cephalosporin in uremia is discussed.

Aged↗