Open-capsule intraocular lens to prevent posterior capsule opacification.

Purpose: To develop a single-piece open-capsule intraocular lens (IOL) that can be inserted through a small incision and that prevents posterior capsule opacification (PCO) by expanding the capsule and circulating aqueous humor into the capsular bag.

Setting: Department of Ophthalmology, Dokkyo Medical University, Tochigi, Japan.

Design: Experimental study.

Pseudopolyneuritic form of ALS revisited: clinical and pathological heterogeneity.

Pseudopolyneuritic form of ALS is a subtype of ALS characterized by distal weakness of the unilateral lower limb and absence of Achilles tendon reflex (ATR) at disease onset. Recognition of this form of ALS is important for clinicians because the combination of distal weakness of the lower limb and absence of ATR usually suggests peripheral neuropathy. We reviewed the clinical records of 42 autopsy-proven sporadic ALS cases and found three cases that showed onset of weakness of the unilateral lower limb with distal dominance and absence of ATR.

Metastatic CNS lymphoma presenting with periventricular dissemination - MRI and neuropathological findings in an autopsy case.

Metastatic CNS lymphoma usually manifests as pachymeningeal or leptomeningeal infiltrates, and periventricular dissemination is rare. A 70-year old man first noticed a mass in the left supraclavicular fossa, and then presented with bilateral parkinsonism, followed by consciousness disturbance. Fluid attenuated inversion recovery (FLAIR) image of brain MRI demonstrated hyperintensities at the parenchyma around the lateral ventricle, third ventricle, and fourth ventricle.

Protective effects of wheat bran against diquat toxicity in male Fischer-344 rats.

After injection with 0.1 mmol diquat/kg body weight, survival time was markedly shorter in Fischer-344 rats fed a purified diet than in rats fed a regular diet, and much more severe hepatotoxicity and nephrotoxicity were observed in the former than in the latter. The longer the feeding period on the purified diet, the shorter the survival time after diquat administration.

[Slowly progressive anarthria and disturbed voluntary respiration--a case report].

A 68 year-old right-handed male initially felt an abnormal sensation in the throat and slight difficulties in phonation and articulation. The difficulties slowly progressed. Dementia and kinetic disorder of limbs has not been observed over two years after onset.

Virological outcomes in patients infected chronically with hepatitis B virus genotype A in comparison with genotypes B and C.

In a single hospital in Tokyo, the 87 patients infected persistently with hepatitis B virus (HBV) genotype A, the 413 with B, and the 3,389 with C were compared for virological outcome. Hepatitis B surface antigen (HBsAg) was cleared from the serum in 12% (3/26), 2% (2/112), and 3% (23/826) of patients with genotypes A, B, and C, respectively, at 5 years of follow-up (P = 0.0395).

A mother and a child with maternally inherited diabetes and deafness (MIDD) showing atrophy of the cerebrum, cerebellum and brainstem on magnetic resonance imaging (MRI).

A mother and a child with maternally inherited diabetes and deafness (MIDD) showing atrophy of the cerebrum, cerebellum and brainstem on magnetic resonance imaging (MRI) were reported. The proband had slowly progressive cerebellar ataxia and her son had depression. Mitochondrial DNA purified from their leucocytes had the heteroplasmic point mutation at position 3243 (A-->G).

Persistence of acute infection with hepatitis B virus genotype A and treatment in Japan.

Among the 97 adult patients with acute hepatitis B who were admitted to the Toranomon Hospital in Metropolitan Tokyo during 28 years from 1976 to 2003, 31 (32%) were infected with hepatitis B virus (HBV) genotype A, nine (9%) with genotype B, 44 (45%) with genotype C, one (1%) each with genotypes E and F. HBV in the remaining 11 (11%) patients were untypeable. All the 31 patients with acute hepatitis B caused by HBV genotype A infection were male with a median age of 31 years, and 16 (52%) contracted infection through extramarital sexual contacts.

Clinical and virological characteristics of untreated patients with chronic hepatitis C who develop serum alanine aminotransferase flare-up.

Among patients with chronic hepatitis C virus (HCV) infection, serum alanine aminotransferase (ALT) rarely increases above 500 IU/L. We examined the clinical and virological features of untreated patients with serum ALT > or = 500 IU/L. One thousand seven hundred and sixty adult patients with chronic HCV infection were followed-up.

Sporadic amyotrophic lateral sclerosis of long duration mimicking spinal progressive muscular atrophy exists: additional autopsy case with a clinical course of 19 years.

This report concerns an autopsy case of sporadic amyotrophic lateral sclerosis (ALS) clinically diagnosed as having spinal progressive muscular atrophy (SPMA). The patient was a Japanese woman without hereditary burden. She developed muscle weakness in the distal part of the right upper extremity at age 52, followed by muscle weakness in the left upper extremity and lower extremities at age 54 and 64, respectively.

Coexistence of CJD and Alzheimer's disease: an autopsy case showing typical clinical features of CJD.

The present report concerns an autopsy case of CJD showing typical clinical features of CJD. The patient was a Japanese woman without hereditary burden or dementing disorder anamnesis who was 70-years-old at the time of death. She developed gait disturbance at age 68, followed by memory impairment, visual disturbance, and myoclonus.

Sporadic amyotrophic lateral sclerosis with circumscribed temporal atrophy: a report of an autopsy case without dementia and with ubiquitinated intraneuronal inclusions.

This report concerns an autopsy case of amyotrophic lateral sclerosis (ALS) with circumscribed temporal atrophy. The patient was a Japanese woman without hereditary burden who was 71-year-old at the time of death. She developed dysarthria and gait disturbance at age 69, followed by dysphagia.

Parkinson's disease mimicking senile dementia of the Alzheimer type: a clinicopathological study of four autopsy cases.

This report concerns four Japanese autopsy cases of Parkinson's disease (PD) mimicking senile dementia of the Alzheimer type. Three patients with a clinical diagnosis of senile dementia of the Alzheimer type developed memory disturbance as the initial sign, and a patient with a clinical diagnosis of atypical senile dementia presented with hallucination and delusion as the initial sign. Dementia was evident in all four patients, and slight parkinsonism appeared in the middle to late stages of the disease in two patients.

Motor weakness and cerebellar ataxia in Sjögren syndrome--identification of antineuronal antibody: a case report.

We report here a combination of rare neurological manifestations of primary Sjögren syndrome (SS), such as motor-dominant motor weakness of peripheral origin, cerebellar ataxia and depression, in a Japanese female patient. An autoantibody in her serum and cerebrospinal fluid immunolabelled spinal motor neurons and cerebellar Purkinje cells. On Western blot, this antibody reacted with a protein of 34 kDa from the extract of spinal cord, dorsal root ganglion, or cerebellar cortex, which might correspond to motor weakness and cerebellar ataxia, respectively.

Unilateral spatial neglect in AD: significance of line bisection performance.

Background: Unilateral spatial neglect has been rarely reported in patients with AD, although they often have right and left asymmetry of temporoparietal dysfunction.

Objective: To investigate if patients with AD would show unilateral spatial neglect in the line bisection test, and to reveal the relationship between their neglect and the area of cerebral dysfunction.

Method: Thirty-two patients with mild to moderate AD and 32 age-matched healthy control subjects underwent an extensive line bisection test.

Constant involvement of the Betz cells and pyramidal tract in multiple system atrophy: a clinicopathological study of seven autopsy cases.

We investigated clinicopathologically the pyramidal signs, including spasticity, hyperreflexia, and Babinski's sign, and the involvement of the pyramidal tract and primary motor cortex, in seven Japanese autopsy cases of multiple system atrophy (MSA). Pyramidal signs were observed in six (86%) of the seven autopsy cases. Hyperreflexia and Babinski's sign were each evident in five patients, but spasticity was observed in only one patient.

Successful plasmapheresis in the not-so-benign Bickerstaff's brain stem encephalitis associated with anti-GQ1b antibody.

A patient with Bickerstaff's brain stem encephalitis (BBE) associated with anti-GQ1b antibody developed coma, severe weakness, and respiratory distress. The patient required ventilatory support. After having failed to improve on steroids, she was treated with plasmapheresis.

A clinical, genetic, neuropathological study in a Japanese family with SCA 6 and a review of Japanese autopsy cases of autosomal dominant cortical cerebellar atrophy.

This report concerns a Japanese family with genetically confirmed SCA 6, including an autopsy case, and a review of Japanese autopsy cases of autosomal dominant cortical cerebellar atrophy (ADCCA). The proband (Case 1) was a Japanese woman. She developed gait disturbance at age 62.

Distribution of cerebellar cortical lesions in multiple system atrophy: a topographic neuropathological study of three autopsy cases in Japan.

We investigated neuropathologically the distribution of the cerebellar cortical lesions in three Japanese autopsy cases of multiple system atrophy (MSA) using hemisphere specimens. The lesions were classified as mild, moderate or severe. The distribution of cerebellar cortical lesions in all three cases were uniform: the cerebellar cortical lesions were more conspicuous in the vermis than in the hemisphere.

Serial brain CT in corticobasal degeneration: radiological and pathological correlation of two autopsy cases.

This report concerns serial brain computed tomography (CT) images in two patients with corticobasal degeneration (CBD). The diagnosis of CBD was confirmed by neuropathological examination. In one case, we obtained serial brain CT images for one year and five months, and in the other, for three years and four months.

[An unusual autopsy case of corticobasal degeneration--with special reference to clinicopathological differentiation from progressive supranuclear palsy and slowly progressive aphasia].

We report an unusual autopsy case of corticobasal degeneration. The patient was male who was 67 years old at the time of his death. He developed clumsiness of his right hand at age 65.

[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.

Preserved leftward movement in left unilateral spatial neglect due to frontal lesions.

Three patients with left unilateral spatial neglect after predominantly frontal lobe lesions were asked to extend a horizontal line leftwards to double its original length. In this line extension task, they readily executed movements in or towards the contralesional left space. They performed the task in the left and right hemispaces as well as in the midline.