Parkinsonism Relating to Intoxication with Glyphosate.

We herein report the case of a 38-year-old man who developed parkinsonism 4 years after ingesting glyphosate. The patient presented with right-sided bradykinesia and cogwheel rigidity without autonomic symptoms. Magnetic resonance imaging of the brain and [I]-metaiodobenzylguanidine myocardial scintigraphy were normal.

Heterogeneity and continuum of multiple sclerosis phenotypes in Japanese according to the results of the fourth nationwide survey.

There are two distinct phenotypes of multiple sclerosis (MS) in Asians, optic-spinal MS (OSMS) and conventional MS (CMS). In 2004, we performed the fourth nationwide epidemiological survey of MS. The epidemiological features were reported elsewhere; here we report the characteristic features of patients with each MS phenotype, classified according to the clinically estimated sites of involvement and MRI findings.

Temporal changes and geographical differences in multiple sclerosis phenotypes in Japanese: nationwide survey results over 30 years.

Background: There are two distinct phenotypes of multiple sclerosis (MS) in Asians, manifesting as optic-spinal (OSMS) and conventional (CMS) forms. In Japan, four nationwide surveys of MS have been conducted. The first three were in 1972, 1982, and 1989, and we performed the fourth in 2004.

Association of the HLA-DRB1 alleles with characteristic MRI features of Asian multiple sclerosis.

Background: In Asian patients with multiple sclerosis (MS), a paucity of brain lesions and longitudinally extensive spinal cord lesions (LESCLs) extending three or more vertebral segments are characteristic findings on magnetic resonance imaging (MRI). We aimed to disclose possible factors contributing to the development of such MRI features.

Method: Genotyping of HLA-DRB1 and -DPB1 alleles was performed in 121 consecutive Japanese patients with clinically definite MS based on the Poser criteria and 125 healthy controls.

Reduction of angiotensin II in the cerebrospinal fluid of patients with multiple sclerosis.

We previously demonstrated that angiotensin II acts as a crucial neuroprotective factor after neural injury through angiotensin II type-2 (AT2) receptor signaling. Although the pathway is known to play an important role in the development of experimental autoimmune encephalomyelitis, cerebrospinal fluid (CSF) angiotensin II levels in patients with multiple sclerosis (MS) have never been studied. To clarify the significance of angiotensin II in MS, we assayed angiotensin II concentrations using an established enzyme-linked immunoabsorbent assay in CSF samples from patients with MS (n = 21), patients with inflammatory neuropathies (IN) (n = 23) and control individuals who did not have either of the neurological diseases or any other disease that might affect the angiotensin II levels in the CSF (control) (n = 24).

[Case of posterior reversible encephalopathy syndrome with cerebral vasoconstriction].

A 56-year-old woman attended our hospital because of acute severe (thunderclap) headache. Neurological examination was normal and no abnormality was found on head CT or by cerebrospinal fluid examination. A few days later, she experienced a recurrence and suffered a seizure in her left upper and lower extremities.

Upper motor neuron syndrome associated with subclinical Sjögren's syndrome.

We present two patients with primary lateral sclerosis-like upper motor neuron disease accompanying subclinical Sjögren's syndrome. Both patients showed progressive spastic quadriparesis, but neither sensory involvement nor detrusor dysfunction was noted. Lower motor neuron signs were detected only in their late follow-up period.

Atopic myelitis with focal amyotrophy: a possible link to Hopkins syndrome.

Among 22 consecutive patients with myelitis, of unknown etiology, and atopic diathesis (atopic myelitis) who from April 2002 to March 2006 had been studied in our clinic, 5 (23%) showed focal amyotrophy in one or two limbs. These 5 patients were subjected to combined clinical, electrophysiological, neuroimaging and immunological studies. Ages were 18 to 58-years-old (average 39).

Anterior periventricular linear lesions in optic-spinal multiple sclerosis: a combined neuroimaging and neuropathological study.

There are two distinct subtypes of multiple sclerosis (MS) in Asians, optic-spinal (OSMS) and conventional (CMS). In OSMS, severe spinal cord lesions are characteristic while brain lesions are scant. We sought to clarify atypical brain lesions in OSMS by neuroimaging and pathological studies.

Heterogeneity and continuum of multiple sclerosis in Japanese according to magnetic resonance imaging findings.

There are two distinct subtypes of multiple sclerosis (MS) in Asians: optic-spinal (OSMS) and conventional (CMS). Longitudinally extensive spinal cord lesions (LESCLs) extending over three or more vertebral segments are characteristic of patients with OSMS, yet in Asians, one-fourth of CMS patients also have LESCLs. To clarify the distinction between LESCLs in OSMS and CMS, and to characterize the relationship between the presence of LESCLs and brain magnetic resonance imaging (MRI) findings, we studied 142 patients with clinically definite MS of relapsing-remitting onset and 12 patients with primary progressive MS (PPMS) by MRI of the whole spinal cord and brain.

Heterogeneity of aquaporin-4 autoimmunity and spinal cord lesions in multiple sclerosis in Japanese.

Opticospinal multiple sclerosis (OSMS) in Asians has similar features to the relapsing-remitting form of neuromyelitis optica (NMO) seen in Westerners. OSMS is suggested to be NMO based on the frequent detection of specific IgG targeting aquaporin-4 (AQP4), designated NMO-IgG. The present study sought to clarify the significance of anti-AQP4 autoimmunity in the whole spectrum of MS.

A distinct subgroup of chronic inflammatory demyelinating polyneuropathy with CNS demyelination and a favorable response to immunotherapy.

To explore subclinical central nervous system (CNS) involvement in chronic inflammatory demyelinating polyneuropathy (CIDP), we recorded somatosensory evoked potentials (SEPs) and motor evoked potentials (MEPs) using transcranial magnetic stimulation, to measure central sensory conduction time (CSCT) and central motor conduction time (CMCT) and examined brain and spinal cord MRI in patients with probable CIDP based on the American Academy of Neurology AIDS Task Force criteria. Eighteen patients with probable CIDP (12 males and 6 females; mean age at examination+/-SD, 45.8+/-17.

Helicobacter pylori infection is a potential protective factor against conventional multiple sclerosis in the Japanese population.

Persistent Helicobacter pylori (H. pylori) infection is a chronic inflammatory stimulus to hosts with an inverse correlation to atopic disorders. In this study, a total of 105 consecutive multiple sclerosis (MS) patients were divided into 52 opticospinal MS (OSMS) and 53 conventional MS (CMS), and their sera, along with those from 85 healthy controls (HC), were examined by an enzyme-linked immunosorbent assay using antibodies against H.

Increased IL-13-producing T cells in ALS: positive correlations with disease severity and progression rate.

We measured the intracellular productions of IFNgamma, IL-2, IL-4, IL-13 and TNFalpha in peripheral blood CD4(+) and CD8(+) T cells from 21 amyotrophic lateral sclerosis (ALS) patients, 14 disease controls (DC) with spinocerebellar degeneration and 16 healthy controls (HC). Only the percentages of CD4(+)IL-13(+) and CD8(+)IL-13(+) T cells were significantly higher in ALS patients than in DC and HC. The CD4(+)IL-13(+) T cell percentages showed a significant negative correlation with the revised ALS functional rating scale scores and significant positive correlation with the disease progression rate, suggesting that IL-13 contributes to ALS.

The contactin 4 gene locus at 3p26 is a candidate gene of SCA16.

Objective: To identify of the gene responsible for the onset of spinocerebellar ataxia type 16 (SCA16).

Methods: We reanalyzed the linkage of the original Japanese pedigree using updated information, including three additional subjects. We then screened all exons located in the critical region.

The selective Rho-kinase inhibitor Fasudil is protective and therapeutic in experimental autoimmune encephalomyelitis.

We studied the role of fasudil, a selective Rho-kinase inhibitor, in experimental autoimmune encephalomyelitis (EAE). Both parenteral and oral administration of fasudil prevented the development of EAE induced by proteolipid protein (PLP) p139-151 in SJL/J mice. Specific proliferation of lymphocytes to PLP was significantly reduced, together with a downregulation of interleukin (IL)-17 and a marked decrease of the IFN-gamma/IL-4 ratio.

Expression of granulysin in polymyositis and inclusion-body myositis.

Background: Granulysin, a recently defined cytolytic molecule, is expressed in cytotoxic T cells and natural killer cells in a similar way to perforin, which is reported to have a major role in the pathogenesis of polymyositis and inclusion-body myositis (IBM).

Objective: To clarify the role of granulysin in polymyositis and IBM.

Methods: The expression of granulysin and perforin was examined by double staining with CD8, CD4 and CD56 in endomysial infiltrating cells and autoinvasive cells in muscle biopsy specimens of 17 patients with polymyositis (6 steroid resistant and 11 steroid responsive) and of 7 patients with IBM.

Upregulation of myeloperoxidase in patients with opticospinal multiple sclerosis: positive correlation with disease severity.

To clarify the role of myeloperoxidase (MPO) in multiple sclerosis (MS), we measured serum MPO levels in 86 Japanese patients with relapsing remitting MS, 47 with opticospinal MS (OSMS) and 39 with conventional MS (CMS), and 85 healthy subjects by sandwich enzyme immunoassays and analyzed relationships with clinical features. We found a significant increase in serum MPO in OSMS patients at relapse and remission, and in CMS patients at remission compared with controls. By logistic regression analysis, the clinical variable associated with high level of MPO at remission in OSMS patients (higher than the mean+/-2 S.

CSF cytokine and chemokine profiles in acute disseminated encephalomyelitis.

We simultaneously measured 16 cytokines/chemokines in cerebrospinal fluid (CSF) from 14 patients with acute disseminated encephalomyelitis (ADEM) and 20 controls using a fluorescent bead-based immunoassay. A variety of cytokines, such as IL-1beta, IL-2, IL-4, IL-5, IL-6, IL-8, IL-10, IFN-gamma, TNF-alpha, G-CSF and MIP-1beta, were significantly elevated in ADEM. In particular, G-CSF showed a marked 38-fold increase compared to the control mean.

Long-term favorable response to interferon beta-1b is linked to cytokine deviation toward the Th2 and Tc2 sides in Japanese patients with multiple sclerosis.

To address the immune mechanism of the long-term beneficial effects of interferon beta (IFN-beta), we measured the intracellular cytokine production patterns of IFN-gamma, IL-4 and IL-13 in peripheral blood CD4+ and CD8+ T cells, which previously displayed alterations during the early course of IFN-beta treatment, in 15 Japanese patients after long-term IFN-beta administration. The patients were treated with IFN-beta-1b 8 x 10(6) units given subcutaneously every other day for a mean period of 34.5 +/- 5.

Upregulation of vascular growth factors in multiple sclerosis: correlation with MRI findings.

Vascular permeability changes precede the development of demyelinating lesions in multiple sclerosis (MS), and vessel wall thickening and capillary proliferation are frequently seen in autopsied MS lesions. Although vascular growth factors are critical for inducing such vascular changes, their involvement in MS has not been extensively studied. Thus, we examined the involvement of various vascular growth factors in MS according to their clinical phase and subtype.

Comparison of the clinical courses of the opticospinal and conventional forms of multiple sclerosis in Japan.

We evaluated the clinical courses of 216 patients with multiple sclerosis (MS) diagnosed according to the recommended diagnostic criteria of McDonald et al (10). Sixty-five patients clinically displaying selective involvement of the optic nerves and spinal cord were classified as opticospinal MS (OS-MS), while the other 151 showing disseminated involvement of the central nervous system were classified as conventional MS (C-MS). The disease duration did not differ significantly between the two subtypes (11.

Platelet-activating factor receptor gene polymorphism in Japanese patients with multiple sclerosis.

We evaluated the association of the platelet-activating factor receptor (PAFR) gene polymorphism (A224D) with the susceptibility and severity of multiple sclerosis (MS) in a Japanese population. DNA was collected from 162 Japanese patients with clinically definite 'conventional' MS (MS) and 245 healthy controls. The missense mutation A224D that impairs PAF-PAFR signaling was determined by polymerase chain reaction restriction fragment length polymorphism.