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F Udaka

Publications and source records attributed to F Udaka.

At least 37 records · Page 2Linked to original sources
PubMed1999

[A sporadic case of spinocerebellar ataxia 6 (SCA 6) with large CAG expansion of the CACNL1A4 gene].

We reported a 73-year-old woman of spinocerebellar ataxia 6 (SCA 6). There was no family history of neurological diseases. She demonstrated cerebellar ataxia and scanning speech at the age of 48. These symptoms gradually developed. Brain MRI showed severe cerebellar atrophy and no abnormality in the brain stem. Her neurological symptoms and MRI findings were compatible with cerebellocortical atrophy (CCA). Analysis of the CACNL1A4 gene on chromosome 19p 13 demonstrated she had an expanded allele with 27 CAG repeats. Therefore, she was diagnosed with SCA 6. In spite of her large CAG expansion, there was no family history of SCA 6 in this case. SCA 6 needs to be ruled out in cases of clinical CCA.

Aged↗
PubMed1999

[A case of giant meningioma that induced visual changes similar to those observed in amaurosis fugax in an early stage of disease].

The patient was a 47-year-old female. A giant meningioma was detected in the right middle cranial fossa by exploration for the cause of right-sided transient monoocular blindness of a 1-2 minute duration accompanied by headache. The headache was localized on the right side, occurred more frequently early in the morning, and continued for several hours. No neurological abnormality was noted except for right-side dominant bilateral choked disc and reduced vision. Both headache and transient monoocular blindness disappeared after total resection of the meningioma. A decrease in the cerebral blood flow associated with an increase in the intracranial pressure and right-sided occurrence of paroxysmal visual impairment due to choked disc were possible mechanisms of the transient monoocular blindness. Amaurosis fugax must be initially first in patients complaining of transient monoocular blindness, and exploration for carotid artery lesions is important. In the present case, however, this symptom led to detection of a giant meningioma.

Blindness↗
PubMed1999

[An autopsy case of intracranial T cell type malignant lymphoma with fluctuating neuropsychological symptoms].

A 59-year-old man was admitted to the hospital due to leg edema. He had multiple sclerosis-like episodes of transient diplopia at the age of 36 years and spastic paraplegia at the age of 38. After admission he showed various fluctuating neuropsychological symptoms (disorientation, hallucination, apraxia, aphasia) and cranial nerve palsy. Magnetic resonance imaging revealed abnormal signal intensity in the right thalamus and deep white matter. The patient was diagnosed as having malignant lymphoma and treated with steroid therapy and chemotherapy. Although partial improvement of clinical findings was observed, recurrent cerebral hemorrhage followed. Autopsy findings revealed perivascular infiltration of T cells in the brain, spinal cord and other general organs.

Aphasia↗
PubMed1999

[A juvenile case of chronic inflammatory demyelinating polyradiculoneuropathy with severe onion bulb-like change mimicking hereditary neuropathy].

A 15-year-old male developed symmetrical weakness of the limb muscles. He had not had any previous developmental disorders except delayed initiation of walking. Flexion contraction of ankle joint and pes cavus deformity were seen. The cerebrospinal fluid protein concentration was elevated. Nerve conduction study showed severe conduction block and temporal dispersion. A sural nerve biopsy revealed remarkable onion bulb-like changes and perivascular infiltration of inflammatory cells. After high-dose corticosteroid treatment, he showed improvement in muscle strength. Although there were no abnormalities of genes related to hereditary neuropathy, the atypical findings of severe demyelinating changes of peripheral nerves mimicked hereditary neuropathy.

Adolescent↗
PubMed1998

The changes of lacunar state during a 5-year period in NIDDM.

With the increment of the aged, primary and secondary prevention of cerebrovascular disease in the Japanese diabetics are crucial problems. Early detection of asymptomatic infarction, not rare in diabetics, is important in the view of cost benefits. Little is known about the development and progression of asymptomatic infarction. We investigated the number of lacuna during a period of 5 years in 51 non-insulin-dependent diabetes mellitus (NIDDM). At the initiation of this study, 22 patients were free from lacuna and another 29 patients had asymptomatic multiple lacunae. In the former patients, no risk factors were different between the 13 patients with still no lacuna and the nine patients with lacuna-developed after 5 years. In the latter patients, the 17 patients with worsening lacunar state had significantly higher systolic blood pressure, and a higher prevalence of macroalbuminuria than the 12 patients with no increment of lacuna. Multiple logistic regression analysis showed that macroalbuminuria after 5 years was the only contributing factor to the increased lacuna. Therefore, in the NIDDM, macroalbuminuria induced an increase of lacuna in magnetic resonance imaging, although numbers of cases were relatively small. Prevention of the development of macroalbuminuria are essential to avoid the progression of asymptomatic lacuna.

Aged↗
PubMed1997

Molecular features of the CAG repeats of spinocerebellar ataxia 6 (SCA6).

Spinocerebellar ataxia 6 (SCA6) is an autosomal dominant spinocerebellar degeneration caused by the expansion of the polymorphic CAG repeat in the human alpha1A voltage-dependent calcium channel subunit gene (CACNL1A4 gene). We have analyzed 60 SCA6 individuals from 39 independent SCA6 Japanese families and found that the CAG repeat length is inversely correlated with the age of onset (n = 58, r = -0.51, P < 0.0001). SCA6 chromosomes contained 21-30 repeat units, whereas normal chromosomes displayed 6-17 repeats. There was no overlap between the normal and affected CAG repeat number. The anticipation of the disease was observed clinically in all eight parent-child pairs that we examined; the mean age of onset was significantly lower (P = 0.0042) in children than in parents. However, a parent-child analysis showed the increase in the expansion of CAG repeats only in one pair and no diminution in any affected cases. This result suggests that factors other than CAG repeats may produce the clinical anticipation. A homozygotic case could not demonstrate an unequivocal gene dosage effect on the age of onset.

Adult↗
PubMed1997

[Creutzfeldt-Jakob disease(CJD) and Gerstmann-Sträussler-Scheinker syndrome(GSS)].

Prion diseases are now a focus of attention since the diseases are known to disseminate beyond expectation. They are various types of Gerstmann-Sträussler-Scheinker syndrome(GSS), fatal familial insomnia, iatrogenic Creutzfeldt-Jakob disease(CJD), and variant CJD which suspected to have disseminated from bovine spongiform encephalopathy. Abnormal prion protein deposits as amyloid structure in the brain of the patients with these diseases. Mutation of the prion protein gene exists in a part of these diseases. Depending on their kind, patients represent a variety of clinical and neuropathological manifestations. They can be clinically divided into two general groups of the CJD type and the GSS type. For clinical diagnosis of the GSS type prion diseases, analysis of prion protein gene is useful.

Animals↗
PubMed1997

[Secondary parkinsonism following midbrain hemorrhage].

We report a patient who developed right sided cogwheel rigidity and resting tremor after left midbrain hemorrhage. Brain magnetic resonance imaging (MRI) showed left midbrain old hemorrhage including substantia nigra. I-123 iodoamphetamine single photon emission computed tomography (IMP-SPECT) images showed reduced radioisotope (RI)-uptake in the left striatum, thalamus and frontal lobe. Our report shows that focal midbrain lesion can produce parkinsonism.

Cerebral Hemorrhage↗
PubMed1997

[A case of adult-onset neurenteric cyst presenting as chronic progressive muscular atrophy in lower legs].

A 50-year-old man was admitted, because of motor weakness of the lower limbs, dysesthesia of the left lower extremity, and anuresis. He had an episode of pain in his gluteal region 17 years ago, and then, no abnormalities were detected including myelography in a hospital, followed by slowly progressive muscular atrophy of his lower legs. At 50 years of age, dysuria appeared. He was diagnosed as having neurogenic bladder by urologists, and was admitted to our hospital. On admission, abnormal neurologic findings included: severe muscular atrophy in his lower legs, pes cavus, dysesthesia at the left S1 level, and autonomic bladder. Magnetic resonance imaging (MRI) showed mass lesion involving lower conus and cauda equina. After resection, pathological study revealed the mass was a neurenteric cyst. It is said that the neurenteric cyst causes an asymmetrical and sequential loss of specific neurological functions, with a subsequent return of these functions in the reverse order. That mechanism is not clear. However, in our case, the course of the illness was slowly progressive. We speculate that, because of the cyst's adhesion to cauda equina and perforation through the cyst by a nerve root, the cyst was fixed and caused slowly progressive neurological deficits in proportion to increase of the cyst's size. Our report suggests that a neurenteric cyst, involving the lower conus and cauda equina, can produce severe muscular atrophy in the lower legs.

Age Factors↗
PubMed1996

Asymptomatic multiple lacunae in diabetics and non-diabetics detected by brain magnetic resonance imaging.

We investigated the relationship between asymptomatic multiple lacunae (ASML) and related risk factors by using brain MRI in 209 patients including 152 NIDDM patients. Patients with ASML (97 cases) were significantly older (68 +/- 9 vs. 63 +/- 10) and hypertension was more frequent (57/97 vs. 33/112) than in patients without ASML. In addition, by multivariate analysis, ASML showed significant association with aging and hypertension, but not with NIDDM. In the NIDDM patients, diabetics with ASML were significantly older, and showed a higher association with hypertension and triopathy than those without ASML, although the results were the same for the middle-aged (< 65 years old) diabetics. From multivariate analysis, the lesions in the penetrating branch area were highly associated with hypertension (F = 8.46) and nephropathy (F = 4.75), while those in the subcortex and white matter were associated with aging (F = 6.02) and retinopathy (F = 5.15). In the middle-aged diabetics, the former was associated with hypertension (F = 10.72) and retinopathy (F = 13.32), whereas the latter was associated with retinopathy (F = 20.76). In the elderly diabetics, no significant association was found in either lesions. These results suggest that control of hypertension and prevention of microangiopathy by keeping good control of blood glucose, is essential to prevent asymptomatic lacunae in NIDDM patients.

Age Factors↗
PubMed1996

Asymptomatic cerebral small infarcts (lacunae), their risk factors and intellectual disturbances.

This study examines the relationship between cerebral small infarcts (lacunae) and the multiple risk factors of diabetes, age, hypertension, hyperlipidemia, and atherosclerosis in asymptomatic NIDDM patients and nondiabetic subjects by comparing brain magnetic resonance imaging (MRI) findings to these risk factors. Brain MRI was performed on 155 asymptomatic NIDDM and 39 asymptomatic nondiabetic patients, using a Shimazu SMT-150, 1.5-T instrument. Among the diabetic patients, 65 showed evidence of lacunae. The incidence of lacunae was significantly higher in older diabetic patients, but it did not significantly differ in those with or without the risk factors of atherosclerosis. We also correlated the results of a freehand cube-drawing test with the incidence of lacunae. Cube-drawing is a good indicator of spatial cognition ability supported by wide association areas of the brain. Drawing ability was tested in 56 diabetic and 39 nondiabetic subjects. Correlations of lacuna incidence with deformity in drawing and with age were high in the diabetic group. Correlation of lacunae with deformity in drawing was also significant in nondiabetic subjects. The incidence of lacunae was highly correlated with intellectual impairment.

Age Factors↗
PubMed1996

[A case of a thoracic extradural arachnoid cyst presenting with slowly progressive muscle weakness in the right upper and lower limbs].

A 49-year-old woman developed slowly progressive muscle weakness of the right upper and lower limbs. Physical examination revealed exaggeration of deep tendon reflexes in bilateral lower extremities and a Th5-Th6 girdle sensation. Weakness in her right upper extremity suggested cervical or intracranial lesion. Neuroradiological studies detected no abnormalities in her cervical cord and cranium. So the symptoms and signs were similar to those of motor neuron disease except for the sensory disturbance. MRI study of thoracic cord demonstrated a thoracic extradural arachnoid cyst. After removal of the cyst, the patient's muscle weakness was prominently relieved. We postulate that the cyst stretched spinal cord and dura mater, which led to affection of her cervical cord. We propose weakness of an upper limb as a pseudo-localizing sign of a thoracic extradural arachnoid cyst.

Arachnoid Cysts↗