Bilateral reversible optic neuropathy as the first sign of Waldenström macroglobulinema.

Objective: To report a case of bilateral reversible optic neuropathy as the first sign of Waldenström macroglobulinemia (WM).

Methods: Observational case report.

Results: A 52-year-old man had a sudden loss of vision in the left eye.

Clinical investigation in non-liver cirrhosis portosystemic shunt encephalopathy-four case series.

We reported here four cases presenting with disturbance of consciousness over long periods of time and hyperammonemia. Two patients were on maintenance hemodialysis. Contrast-enhanced computed tomography (CT) of abdomen and balloon-occluded retrograde contrast venography revealed existence of a non-cirrhotic portosystemic shunt.

Clinical study on the cerebral infarction accompanied with septic disseminated intravascular coagulation.

Objectives: Patients with disseminated intravascular coagulation (DIC) due to sepsis often develop cerebral infarction; but the frequency, mechanism of onset and prognosis have not been fully elucidated. We reported courses and characteristics of septic DIC cases hospitalized in our hospital in the present study.

Methods: Patients with septic DIC who underwent brain imaging were selected.

Subjective assessment of effectiveness, quality of life, and psychological status of patients receiving botulinum toxin therapy for hemifacial spasm, blepharospasm, or cervical dystonia.

Objectives: Evaluations of subjective effectiveness, quality of life (QOL), and mental status of patients receiving treatment with botulinum toxin (BTX) for hemifacial spasm (FS), blepharospasm (BS), and cervical dystonia (CD) were conducted using a self-administered questionnaire.

Methods: Eighty-eight patients who received BTX treatment in the stable stage were analyzed. A numerical rating scale was used to assess treatment effectiveness, home QOL, and social QOL.

An Autopsy Confirmed Neuromyelitis Optica Spectrum Disorder with Extensive Brain White Matter Lesion and Optic Neuritis but Intact Spinal Cord, Clinically Mimicking a Secondary Progressive Multiple Sclerosis-like Course.

A 57-year-old woman presented with optic neuritis with repeated clinical symptoms of focal demyelination of the cerebral white matter and brain stem for 14 years. At the end of the patient's course, the clinical signs mimicked secondary progressive multiple sclerosis, but whether it was caused by interferon administration or neuromyelitis optica spectrum disorders (NMOSD) - or a combination of both or others - was unclear. Histopathological findings indicated the etiology to be NMOSD, with no apparent plaque in spinal cord specimens.

Dopaminergic function in spinocerebellar ataxia type 6 patients with and without parkinsonism.

Background: Although pure cerebellar ataxia is usually emphasized as the characteristic clinical feature of spinocerebellar ataxia type 6 (SCA6), parkinsonism has been repeatedly described in patients with genetically confirmed SCA6.

Methods: We conducted a positron emission tomography study using a combination of [F]fluoro-L-dopa for dopamine synthesis and [C]raclopride for dopamine D2 receptor function on six genetically confirmed SCA6 patients, both with and without parkinsonism. To the best of our knowledge, this is the first dopamine receptor imaging study of patients with SCA6.

An Adult Case of Generalized Convulsions Caused by the Ingestion of Ginkgo biloba Seeds with Alcohol.

A 64-year-old woman developed symptoms of vomiting and tonic-clonic convulsions 9.5 h after eating 50 roasted Ginkgo biloba seeds with 100 g of alcohol. The intravenous administration of pyridoxal phosphate effectively improved the symptoms.

Intracranial vascular calcification with extensive white matter changes in an autopsy case of pseudopseudohypoparathyroidism.

We herein report an autopsy case of a 69-year-old man with pseudopseudohypoparathyroidism. The patient suffered from mental retardation and spastic tetraparesis and had all the features of Albright's hereditary osteodystrophy with a normal response to parathyroid hormone in the Ellsworth-Howard test. Computed tomography demonstrated symmetrical massive brain calcification involving the bilateral basal ganglia, thalami, dentate nuclei and cerebral gray/white matter junctions, which was consistent with Fahr's syndrome.

Hypocretin-1 levels in the cerebrospinal fluid of patients with Percheron artery infarction with or without midbrain involvement: A case series.

Background: Bilateral paramedian thalamic infarctions (BPTIs) due to artery of Percheron occlusion are known to cause hypersomnia. However, the role of hypocretin-1, a wake-promoting peptide that is located at the lateral hypothalamus, in hypersomnia in these patients remains unclear.

Methods: To clarify the role of hypocretin-1 in hypersomnia in patients with BPTIs, hypocretin-1 levels in the cerebrospinal fluid (CSF) were measured in 6 patients with BPTIs: 2 with rostral midbrain involvement (BPT+RMI) and 4 without midbrain involvement (BPT-MI).

An autopsy case of hemiconvulsion-hemiplegia-epilepsy syndrome manifesting as cerebral hemiatrophy in an elderly man.

We report an autopsy case of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome in a 79-year-old man. HHE syndrome usually occurs in children younger than 4 years of age. Although most HHE syndrome patients live into adult life, only a few cases of the syndrome have been reported in the elderly.

Hemodialysis-related portal-systemic encephalopathy.

Hemodialysis-related portal-systemic encephalopathy (HRPSE) is characterized by the presence of portosystemic encephalopathy without liver dysfunction, usually caused by changes in the systemic venous flow related to hemodialysis. We herein describe the case of a 75-year-old woman who developed hepatic encephalopathy five years after the initiation of hemodialysis. Abdominal contrast-enhanced computed tomography (CT) and three-dimensional CT angiography revealed a portosystemic venous shunt, and the patient was diagnosed with portosystemic encephalopathy.

Gerstmann-Straeussler-Scheinker disease with P102L prion protein gene mutation presenting with rapidly progressive clinical course.

We describe an autopsied case of a Japanese woman with Gerstmann-Straeussler-Scheinker disease (GSS) presenting with a rapidly progressive clinical course. Disease onset occurred at the age of 54 with dementia and gait disturbance. Her clinical course progressively deteriorated until she reached a bedridden state with myoclonus 9 months after onset.

Longitudinal study on MRI intensity changes of Machado-Joseph disease: correlation between MRI findings and neuropathological changes.

To disclose the neuropathological progression course of Machado-Joseph disease (MJD), magnetic resonance imaging (MRI) findings of six genetically confirmed MJD cases (four males and two females, including an autopsied female, all unrelated to one another) were further investigated on neurodegeneration. Brain MRI studies were repeated in all cases at different stages of the disease. Ages at the first MRI study ranged from 47 to 65 years (55.

[Pure agraphia with topographical disorientation caused by right hemisphere lesion].

A 69-year-old, right-handed, Japanese male patient presented with pure agraphia with topographical disorientation after hemorrhage in the right parietal lobe. Upon developing cerebral hemorrhage, he was referred to our hospital for close examination of agraphia. There was no paresis or clumsiness in his extremities.

Acute motor and sensory neuronopathy associated with small-cell lung cancer: a clinicopathological study.

A 48-year-old Chinese woman developed ascending motor paralysis while visiting Japan, leading to tetraplegia and respiratory failure over 2 weeks. The patient's course was complicated by anoxic encephalopathy. Nerve conduction studies revealed a severely decreased amplitude of compound muscle action potentials and a sural nerve biopsy specimen showed findings consistent with axonal-form Guillain-Barr6 syndrome.

Neurologic complications associated with influenza vaccination: two adult cases.

We describe two adult cases of neurologic complications occurring after influenza vaccination. The first case was a 62-year-old man who experienced convulsions 5 days after vaccination, and the second case was a 70-year-old man who exhibited paraplegia 7 days after vaccination. Diagnoses of acute disseminated encephalomyelitis and transverse myelitis with acute motor axonal neuropathy were made, respectively, and steroid pulse therapy and intravenous gamma globulin therapy alleviated the patients' symptoms.

[A probable case of Creutzfeldt-Jakob disease with electroretinogram alterations].

Although visual disturbance is recognized as a clinical feature of Creutzfeldt-Jakob disease (CJD), lesions within the retina have to date, recorded little attention. In this single case study, we report a case of retinopathy observed in a 72-year-old female patient with CJD. The patient admitted to the hospital complaining of a 2-month history of visual disturbance, dysarthria, and gait disorder.

[A case of meningoencephalitis and spondylodiscitis caused by Campylobacter fetus subsp. fetus infection].

Campylobacter fetus subsp. fetus (C. fetus) is a gram-negative, curved, rod-shaped microaerophile, occasionally may cause meningitis or meningoencephalitis in humans.