[Hypertrophic pachymeningitis: three adult cases and a review of the literature].

Hypertrophic pachymeningitis (HP) is thought to have an autoimmune etiology but its precise cause and treatment remains to be elucidated. Here, we report the clinical details and therapeutic responses of 3 patients with HP and reviewed 66 previously reported cases in the literature. Among these patients, headache was the most frequent complaint.

Different characteristics of human herpesvirus 6 encephalitis between primary infection and viral reactivation.

Background: Pathogenesis of human herpesvirus 6 (HHV-6) encephalitis, in particular difference between HHV-6 encephalitis at the time of primary infection and reactivation remains unclear.

Objectives: To elucidate the mechanism of HHV-6 encephalitis at the time of primary infection and reactivation.

Study Design: Twenty-two HHV-6 encephalitis patients at the time of primary infection, 6 febrile convulsion (FC) patients caused by HHV-6 infection, and 14 FC patients without HHV-6 infection (non HHV-6 FC) were enrolled.

Clioquinol inhibits NGF-induced Trk autophosphorylation and neurite outgrowth in PC12 cells.

Clioquinol is considered to be a causative agent of subacute myelo-optico neuropathy (SMON), although the pathogenesis of SMON is yet to be elucidated. To investigate the mechanism of neurotoxicity of clioquinol, we used PC12 cell line and focused on nerve growth factor (NGF) signaling through Trk receptor, which is essential for survival and differentiation of neuronal cells. Clioquinol inhibited NGF-induced Trk autophosphorylation in a dose-dependent manner.

Acute autonomic, sensory and motor neuropathy: successful treatment with IVIg.

Acute autonomic, sensory and motor neuropathy (AASMN) is a rare peripheral nerve disorder characterized by prominent dysautonomia with somatic sensory and motor impairment. Dysautonomia in AASMN is intractable even with corticosteroid therapy or plasmapheresis. Here we report a case of AASMN with severe orthostatic hypotension.

[A case of acute disseminated encephalomyelitis (ADEM) following treatment for pneumococcal meningoencephalitis].

A 58-year-old man was admitted to our hospital with fever, vomiting and disturbance of consciousness after common cold-like symptoms for 2 days. Physical examination showed high fever, moderate hypertension and tachycardia. There were no superficial lymph nodes swelling nor skin rashes.

Role of glycosphingolipids and therapeutic perspectives on Alzheimer's disease.

Alzheimer's disease (AD) is a devastating neurodegenerative disorder dividing into two forms, early onset familial and late onset sporadic forms. Early onset genetic cases (familial AD (FAD)) constitute about 10% of all AD cases. Heretofore, highly fibrillinogenic and pathological Abeta peptide formation is regarded as the fundamental molecular basis for this disorder.

Postinfectious myeloradiculoneuropathy with cranial nerve involvements associated with human herpesvirus 7 infection.

Background: Infection with human herpesvirus 7 (HHV-7) generally results in a febrile illness with accompanying exanthema subitum.

Objectives: To ascertain and describe the role of HHV-7 in a case of acute myeloradiculoneuropathy.

Patient: A previously healthy young man with complaints of motor weakness, dysphasia, and nasal voice.

Impairment of Trk-neurotrophin receptor by the serum of a patient with subacute sensory neuropathy.

Background: Paraneoplastic peripheral neuropathy is sometimes associated with unidentified neuronal autoantibodies.

Objective: To examine the effects of serum from a patient with subacute sensory axonopathy on the function of the Trk high-affinity nerve growth factor receptor.

Patient: An 86-year-old man with sensory neuropathy exhibiting an autoantibody to Trk.

Abnormal intracellular trafficking of high affinity nerve growth factor receptor, Trk, in stable transfectants expressing presenilin 1 protein.

The pathogenesis of Alzheimer's disease (AD) is now thought to be tightly linked to Abeta deposition and oxidative stress, but it is still unknown how these factors result in neuronal dysfunction and cell death. Mutations of presenilin 1 (PS1) gene are the causative gene for early onset familial AD (FAD) due to the overproduction and deposition of pathogenic Abeta1-42 peptides. We report here the molecular influences of the overexpression of PS1 protein by stable transfection of PS1 cDNA into SH-SY5Y neuroblastoma cells on the function of high affinity nerve growth factor receptor, Trk, that is essential for neuronal survival and differentiation.