Recurrent HyperCKemia with Immunological Involvement of the Endomysial Capillaries in Neuromyelitis Optica.

A 55-year-old woman with neuromyelitis optica (NMO) had recurrent myalgias with hyperCKemia. A muscle biopsy suggested nonspecific myopathic changes. Regarding immunohistochemistry, the expression of both major histocompatibility complex class I and myxovirus resistance protein A was observed in the endomysial capillaries, suggesting immunological involvement of these capillaries, whereas both C5b9 (membrane attack complex) and aquaporin 4 immunofluorescence stainings were normal. The present findings led us to conclude that one possible mechanism for hyperCKemia in NMO underlying the immunological involvement of the endomysial capillaries was an as-yet-unidentified factor that triggered damage to the integrity of the sarcolemma and thereby cause CK leakage into the serum.

Prognostic evaluation of branch atheromatous disease in the pons using carotid artery ultrasonography.

BACKGROUND: Branch atheromatous disease is an ischemic stroke, involving occlusion or severe stenosis of the perforating artery, causing neurologic symptoms and serious sequelae. We aimed to investigate initial morphometric and hemodynamic characteristics of the vertebral artery immediately post-onset to predict lesion expanding. METHODS: This case-control study collected demographic, historical, and physical examination data from 44 patients with branch atheromatous disease in the pons at admission. The maximum ischemic pons area and stenosis rate in the basilar artery were calculated using magnetic resonance images. Diameter, velocity, and flow volume of the vertebral arteries were measured using carotid artery ultrasonography. Correlations between ischemic lesion extent and these parameters were investigated. RESULTS: Patients were assigned to groups of less (Group 1) or more (Group 2) than the median maximum ischemic area in the pons, calculated from magnetic resonance images (121.6 mm2). Modified Rankin scale scores were significantly worse in Group 2. Blood pressure and blood findings were similar between groups. Group 2 showed significantly higher basilar artery stenosis rates. Flow volume, velocity, peak systolic velocity, and end-diastolic velocity in the vertebral artery on both sides were significantly decreased in Group 2. CONCLUSIONS: Deteriorated vertebral artery hemodynamics caused a more extensive ischemic lesion in branch atheromatous disease in the pons. Evaluation of the vertebral using carotid artery ultrasonography in the acute phase may be useful for predicting disease progression.

Nonsystemic vasculitic neuropathy in a patient with IgG-monoclonal gammopathy of undetermined significance: A case report.

RATIONALE: Monoclonal gammopathy of undetermined significance (MGUS) is a plasma cell proliferative disorder that consistently precedes multiple myeloma. Peripheral neuropathy in patients with IgG-MGUS tends to vary in clinical phenotype. We report a rare case of a patient with IgG-MGUS who had nonsystemic vasculitic neuropathy (NSVN). PATIENT CONCERNS: A 56-year-old Japanese woman presented with progressive sensory ataxia with episodic paresthesia. Her clinical and laboratory values were compatible with IgG-MGUS. A nerve conduction study suggested possible chronic inflammatory demyelinating polyneuropathy. However, intravenous immunoglobulin therapy was not effective. A sural nerve biopsy specimen revealed mildly reduced myelinated fiber density and myelin ovoid formation, with epineural arterioles infiltrated by inflammatory cells. DIAGNOSES: We accordingly diagnosed her condition as NSVN. INTERVENTIONS: She was accordingly started on oral prednisolone (40 mg/d) at 3 months after the onset of her neurological symptoms. OUTCOMES: At 1 year after the oral prednisolone treatment was begun, the patient's neurological symptoms showed no worsening. LESSONS: These findings indicate NSVN as a possible cause of peripheral neuropathy in patients with IgG-MGUS. Cumulatively, our findings highlight the need for a nerve biopsy for peripheral neuropathy in patients with IgG-MGUS as a possible cause of NSVN. The early diagnosis of NSVN is expected to be beneficial for such patients.

Efforts by Pharmacists in Community Pharmacy in Peacetime for Headache Medical Care in Emergencies, Such as Great Earthquake and Disaster.

We herein analyzed the issues that pharmacists in a community pharmacy in peacetime need to prepare for regarding headache medical care in emergencies (the state that supply of medical supplies is difficult) using a questionnaire intended for doctors and pharmacists in a community pharmacy. Recovery rates were 48.0% (96/200) for doctors and 37.3% (112/300) for pharmacists. In order to distinguish between patients for whom pharmacists need to "recommend OTC drugs" and those who need to be encouraged "to consult a hospital or clinic", doctors indicated that pharmacists need to use an "assistance tool to diagnosis headaches, such as a migraine screener" and "guidelines for chronic headaches". However, few pharmacists used these tools. Approximately 66.7% of doctors indicated that it is "meaningful" for pharmacists to distinguish patients with headaches. Moreover, doctors indicated the need for guidance by pharmacists in peacetime regarding headache medical care in emergencies. Although 73.2% of pharmacists instructed the patients with headaches of the importance of medication notebooks in emergencies, guidance ("understanding the triggers of headaches", "understanding the importance of removing the cause of the headache", "standing OTC drugs" and "standing prescription drugs") by pharmacists to prepare for an emergency was insufficient. These results provide useful information to improve the efforts by pharmacists in community pharmacies in peacetime for headache medical care in emergencies.

False-positive results obtained for immunoglobulin M antibody tests of cerebrospinal fluid for herpes simplex virus in a patient with varicella zoster virus encephalitis.

A 66-year-old man presented with a disturbed consciousness and seizure-like movements, followed by the initial symptoms of herpes zoster. Immunoglobulin (Ig) M antibodies to varicella zoster virus (VZV) as well as herpes simplex virus (HSV) were positive in the cerebrospinal fluid (CSF), whereas polymerase chain reaction of the CSF was positive for VZV-DNA but negative for HSV-DNA. The serum/CSF IgM ratio for VZV and HSV increased in association with a clinical improvement. This is a case report of a rare case of VZV encephalitis demonstrating false-positive results for IgM to HSV in the CSF. The increase in the serum/CSF IgM ratio possibly reflects a recovery from blood-brain barrier breakdown.

[Relationship between callosal lesions and cerebral microhemorrhage in Marchiafava-Bignami disease].

Marchiafava-Bignami disease is a rare alcohol-associated disorder. Clinical features include not only disturbed consciousness, dysarthria, tetraparesis, and astasia-abasia as initial symptom but also cognitive deficits and symptoms of interhemispheric disconnection as clinical outcomes. The clinical significance of cerebral microhemorrhage has been recognized in patients with cognitive deficits. We have recently examined the clinical significance of cerebral microhemorrhage in Marchiafava-Bignami disease and demonstrated that demented patients showed higher severity of cerebral microhemorrhage than patients with normal cognitive function. However, the relationship between callosal lesions and cerebral microhemorrhage in Marchiafava-Bignami disease has not been fully examined. The aim of the present study was to clarify the relationship between callosal lesions and cerebral microhemorrhage in Marchiafava-Bignami disease. For this purpose, we report four patients with Marchiafava-Bignami disease. All cases had a history of chronic alcohol abuse and symmetrical lesions in the corpus callosum. Clinical symptoms include not only coma, dysarthria, and astasia-abasia as initial symptom but also dementia as clinical outcomes. Susceptibility-weighted imaging showed asymmetrical hypointense areas in the multiple cortico-subcortical regions, indicating the presence of cerebral microhemorrhage. There were no apparent relationships between the extension of callosal lesion and the severity of cognitive deficits or cerebral microhemorrhage. Our present report indicates that cerebral microhemorrhage, an important. factor for the severity of dementia in Marchiafava-Bignami disease as clinical outcomes, is independent of the callosal lesion.