Serum immunoglobulin G level reduction is a predictor of short-term improvement in patients with myasthenia gravis undergoing plasmapheresis.

Objectives: The aim of the study was to investigate the serological factors in predicting symptom improvement in myasthenia gravis (MG) patients who underwent plasmapheresis (PP).

Methods: The relationship between symptom improvement and change in immunoglobin G (IgG) and acetylcholine receptor antibody (AchR-Ab) levels in 21 MG patients after PP was analyzed.

Results: Patients were divided into two groups: the favorable (n = 11) or unfavorable (n = 10) response groups around the median MG composite improvement rate (50 [29, 56]%) 2 weeks after a PP course.

Anti-leucine-rich Glioma-inactivated 1 Protein-antibody Positive Encephalitis with Extensive Unilateral Cerebral Cortex and White Matter Lesions.

Encephalitis caused by antibodies targeting the leucine-rich glioma-inactivated 1 protein receptor, which belongs to the anti-voltage-gated potassium channel receptor complex, is characterized by hyponatremia, progressive cognitive impairment, seizures, and psychiatric disorders. The patient initially presented with faciobrachial dystonic seizures and subsequently developed encephalopathy. Brain magnetic resonance imaging revealed atypical unilateral hyperintense signals in the cerebral cortex and white matter.

Siponimod ameliorates experimental autoimmune neuritis.

Background: Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are human autoimmune peripheral neuropathy. Besides humoral immunity, cellular immunity is also believed to contribute to these pathologies, especially CIDP. Sphingosine-1-phosphate receptor 1 (S1PR1) regulates the maturation, migration, and trafficking of lymphocytes.

Myasthenia Gravis Complicated by Eosinophilic Granulomatosis with Polyangiitis: A Case Report.

A 55-year-old woman with a history of allergic sinusitis was being administered cyclosporine for ptosis and diplopia due to myasthenia gravis since age 46 years. She developed painful dysesthesia that began in her feet and later spread to her palms, leading to difficulty in walking. Eosinophils were markedly increased in the peripheral blood.

Outcome of pitavastatin versus atorvastatin therapy in patients with hypercholesterolemia at high risk for atherosclerotic cardiovascular disease.

Background: There has been no report about outcome of pitavastatin versus atorvastatin therapy in high-risk patients with hypercholesterolemia.

Methods: Hypercholesterolemic patients with one or more risk factors for atherosclerotic diseases (n = 664, age = 65, male = 54%, diabetes = 76%, primary prevention = 74%) were randomized to receive pitavastatin 2 mg/day (n = 332) or atorvastatin 10 mg/day (n = 332). Follow-up period was 240 weeks.

Severe ileus and urinary retention in a patient with tuberculous meningitis.

An acute ileus and/or urinary retention are recognized as emergent complications requiring appropriate depressurizing treatments. Meningitis should be suspected as a cause of these autonomic disturbances.

Convulsion and cerebellar ataxia associated with maternally inherited diabetes and deafness: a case report.

Convulsion in diabetics is often considered as a result from fluctuation of blood glucose level. However, if a diabetic patient also presents abnormal neurological signs, mitochondrial diseases need to be considered in the differential diagnosis.

Association between Glucocorticoid-Induced Osteoporosis and Myasthenia Gravis: A Cross-Sectional Study.

Purpose: To investigate the association between glucocorticoid-induced osteoporosis and myasthenia gravis (MG) using a cross-sectional survey in Japan.

Methods: We studied 363 patients with MG (female 68%; mean age, 57 ± 16 years) who were followed at six Japanese centers between April and July 2012. We evaluated the clinical information of MG and fractures, bone markers, and radiological assessment.

The long-term effects of pitavastatin on blood lipids and platelet activation markers in stroke patients: impact of the homocysteine level.

To examine the impact of the plasma homocysteine level on the anti-atherosclerotic effects of pitavastatin treatment, we retrospectively examined 59 patients who had a history of stroke and had been prescribed pitavastatin for the treatment of dyslipidemia at the Neurology department of Toho University Ohashi Medical Center Hospital. The patients were classified into two groups according to their homocysteine levels. Carotid artery plaque progression was determined before and after pitavastatin treatment.

[Myasthenia gravis and acetylcholine].

A century has passed since the discovery of the first neurotransmitter, acetylcholine, in 1914. Extensive research on acetylcholine and its function has provided us with important options to treat the formerly intractable neuromuscular disease myasthenia gravis (MG). In the first days of treatment for MG, acetylcholine esterase inhibitors are administered, which is only a symptomatic therapy.

The course of incidental intraorbital cavernous hemangioma in a patient with myasthenia gravis treated with cyclosporine: a case report.

A 69-year-old woman presented with generalized myasthenia gravis and a left orbital cavernous hemangioma (OCH), which elevated the optic nerve and displaced the external eye muscles. Cyclosporine treatment induced a gradual size reduction in the OCH, and consequently contributed to improved external ocular symptoms in combination with immunosuppressant effects.

An exploratory study of the efficacy and safety of yokukansan for neuropsychiatric symptoms in patients with Parkinson's disease.

The present study examined the efficacy and safety of yokukansan (YKS) in neuropsychiatric symptoms in patients with Parkinson's disease (PD) using the neuropsychiatric inventory (NPI). Twenty-five patients with PD (M:F 14:11; age 72 years) were enrolled and treated with YKS (7.5 g/day) for 12 weeks.

DAB389IL-2 suppresses autoimmune inflammation in the CNS and inhibits T cell-mediated lysis of glial target cells.

In multiple sclerosis (MS) and its rodent model, experimental autoimmune encephalomyelitis (EAE), activated CD4(+) T cells with upregulated IL-2R mediate inflammation and demyelination in the central nervous system (CNS). DAB(389)IL-2, a chimeric fusion protein of IL-2 and diphtheria toxin, inhibits human and rodent IL-2 activated T cells that express the high affinity interleukin-2 receptor. In the present study, DAB(389)IL-2 was used to treat rats with EAE.

Legionellosis presenting as singultus and external ophthalmoplegia.

We report a 71-year-old man with legionellosis, who presented with abducens nerve palsy, singultus, confusion, memory impairment, ataxia, and hyporeflexia. Legionella pneumonia was diagnosed on the basis of detection of Legionella pneumophila antigen in the urine. The cerebrospinal fluid was negative for the antigen and antibody, but an oligoclonal band was detected, and the IgG index was elevated.

Anti-TNF therapy using etanercept suppresses degenerative and inflammatory changes in skeletal muscle of older SJL/J mice.

Limb-girdle muscular dystrophy 2B and Miyoshi myopathy are characterized by muscle fiber necrosis caused by a defect in dysferlin and inflammatory changes. SJL/J mice are deficient in dysferlin and display severe inflammatory changes, most notably the presence of cytokines, which may be related to destruction of the sarcolemma. We tested the hypothesis that tumor necrosis factor (TNF) contributes to myofibril necrosis.

[Case of neuralgic amyotrophy with anti GT1a antibody].

A 48-year-old-man had intense pain in the neck and muscle weakness in the left upper limb after he presented low grade fever and appetite loss for a week. Several days later, he developed intense pain and severe muscle weakness in bilateral upper limbs. Laboratory examination showed elevated liver enzyme levels.

Triphasic waves in a patient with tuberculous meningitis.

We report on the case of a 32-year-old woman with tuberculous meningitis (TBM) with electroencephalogram (EEG) output displaying triphasic waves (TWs). The EEG on day 8 revealed generalized slowing, frontal bilateral TWs, a background of 2Hz delta waves, and no epileptiform activity. The patient's condition improved slowly with antituberculosis chemotherapy treatment.

Autoimmune polyglandular syndrome type 2 with myasthenia gravis crisis.

We describe a rare case of autoimmune polyglandular syndrome type 2 initially presenting as Addison disease and autoimmune thyroid disease, with subsequent development of autoimmune hepatitis and myasthenia gravis (MG) crisis in a Japanese woman. MG improved with oral prednisolone followed by plasmapheresis for immunoadsorption; thymectomy was not performed. Conventional treatment for MG was effective and safe in this case, in which there was positivity for human leukocyte antigen A23, B52, B62, DR11, and DR15.

Neurosyphilis with unilateral optic tract lesion causing homonymous hemianopia.

Introduction: Homonymous hemianopia is not a rare symptom. Many causative lesions and pathologies are known, although a unilateral optic tract lesion caused by syphilis is rare.

Case Report: A 56-year-old woman developed a congruous right homonymous hemianopia and bilateral tonic pupils with irregular shape.

Beneficial effect of pramipexole for motor function and depression in Parkinson's disease.

We examined whether pramipexole (PPX) can influence depressive scale in normal and mild depressive parkinsonian patients. In an open study of PPX as an add-on to L-dopa therapy or single administration, 36 nondemented outpatients with Parkinson's disease (PD) were entered first. All were in the stage II or III of Hoehn and Yahr scale (H&Y).

A case of Vernet syndrome with varicella zoster virus infection.

A 40-year-old man was admitted to our department, because of sudden onset of dysphagia, hoarseness, left neck pain and headache. There were no skin lesions. On neurological examination, there were paralysis of the left soft palate and constrictor muscles of the pharynx, weakness of the left sternocleidomastoid and left upper trapezius.

Familial Creutzfeldt-Jakob Disease with a codon 200 mutation presenting as thalamic syndrome: diagnosis by single photon emission computed tomography using (99m)Tc-ethyl cysteinate dimer.

The clinical features of familial Creutzfeldt-Jakob disease with a codon 200 point mutation [fCJD (E200K)] are similar to those of sporadic CJD (sCJD). MRI diffusion-weighted imaging (MRI-DWI) has been reported to be useful for the early diagnosis of CJD. We describe a Japanese fCJD (E200K) case in which thalamic symptoms were the initial manifestations.