Effects of immunotherapy on isolated optic neuritis associated with ozoralizumab, a novel anti-tumor necrosis factor α NANOBODY® compound: A case report.

Tumor necrosis factor (TNF)-α inhibitors rarely cause optic neuritis as a side effect although the disease associated with ozoralizumab, a novel anti-TNF-α NANOBODY® compound, has not been reported. A 76-year-old female with rheumatoid arthritis, medicated with ozoralizumab, was hospitalized with a two-day history of extending partial visual field loss in the left eye. Campimetry revealed top horizontal hemianopia in the left eye.

Anti-galactocerebroside Antibody-Associated Bickerstaff's Brainstem Encephalitis With Dysautonomia: A Case Report and a Review of Associated Central Nervous System Diseases.

A 68-year-old man developed diplopia, unsteady walking, and bladder and bowel dysfunction followed by consciousness disturbance within four weeks. On physical examination, consciousness disturbance, bilateral ptosis, ophthalmoplegia, disappearing of doll's eye phenomenon, dysarthria, and diminished deep tendon reflexes were observed. Cerebrospinal fluid (CSF) examination showed oligoclonal bands.

Association of dysphagia severity in multiple system atrophy with the specific binding ratio on dopamine transporter SPECT.

Objective: Dysphagia in multiple system atrophy (MSA) is life-threatening and is caused by parkinsonism with cerebellar ataxia as a contributing factor. The present study investigated the relationship between dysphagia severity in MSA and the specific binding ratio (SBR) on dopamine transporter (DaT) SPECT using the Hyodo score, a qualitative scale for use with fiberoptic endoscopic evaluation of swallowing (FEES).

Methods: Hyodo score's ability to predict aspiration during a FEES examination of 88 patients with MSA was first tested.

Two distinct degenerative types of nigrostriatal dopaminergic neuron in the early stage of parkinsonian disorders.

Introduction: The present study characterized the degeneration of nigrostriatal dopaminergic neurons in the early stages of parkinsonian disorders using integrative neuroimaging analysis with neuromelanin-sensitive MRI and I-FP-CIT dopamine transporter (DAT) SPECT.

Methods: Thirty-one, 30, and 29 patients with progressive supranuclear palsy (PSP), corticobasal syndrome (CBS) with abnormal specific binding ratio (SBR) in either hemisphere (mean ± 2SD), and parkinsonism-predominant multiple system atrophy (MSA-P), respectively, were enrolled. Neuromelanin-related contrast (NRC) in the substantia nigra (NRC) and locus coeruleus (NRC) and the SBR of DAT SPECT were measured.

Hashimoto Encephalopathy Presenting with Acute Psychosis and Inappropriate Secretion of Antidiuretic Hormone: A Rare Case Responding to Steroid Therapy.

BACKGROUND Hashimoto's encephalopathy (HE) is an autoimmune encephalopathy that can involve various symptoms including psychosis. Syndrome of inappropriate secretion of antidiuretic hormone (SIADH) may be a complication in some neurological diseases. However, the simultaneous occurrence of subacute psychosis and SIADH as the manifestation of HE, observed in the present case, has rarely been reported.

[MR neurography reveals fascicular constriction of the median nerve in a patient with neuralgic amyotrophy].

Diagnosing neuralgic amyotrophy can be challenging in clinical practice. Here, we report the case of a 37-years old Japanese woman who suddenly developed neuropathic pain in the right upper limb after influenza vaccination. The pain, especially at night, was severe and unrelenting, which disturbed her sleep.

Dopa-responsive dystonia in spinocerebellar ataxia 6: A case report.

Spinocerebellar ataxia 6 (SCA6) often presents with pure cerebellar ataxia. It is rarely accompanied by extrapyramidal symptoms, such as dystonia and parkinsonism. Here, we describe a case of SCA6 with dopa-responsive dystonia for the first time.

Degeneration of nigrostriatal dopaminergic neurons in the early to intermediate stage of dementia with Lewy bodies and Parkinson's disease.

Objective: To investigate differences in nigrostriatal dopaminergic neuron degeneration between dementia with Lewy bodies (DLB) and Parkinson's disease (PD) in the early to intermediate stage of these diseases.

Methods: An integrative neuroimaging analysis was developed using 3-Tesla neuromelanin-sensitive MRI and I-FP-CIT dopamine transporter SPECT, and the relationship and laterality of three variables, including neuromelanin-related contrast in the substantia nigra (NRC) and locus coeruleus (NRC) and the specific binding ratio (SBR) in the striatum, were examined in detail. Patients with DLB and PD and control subjects (n = 29, 52, and 18, respectively) were enrolled.

Association of autoantibodies to muscarinic acetylcholine receptors with gastrointestinal symptoms and disease severity in patients with postural orthostatic tachycardia syndrome.

Previous studies have reported a relationship between postural orthostatic tachycardia syndrome (POTS) and positivity for serum autoantibodies against G-protein-coupled receptors (GPCRs). However, the role of these autoantibodies in POTS is unclear. The present retrospective study analyzed the autoimmune etiology of POTS in 24 patients using a head-up tilt test to assess for any correlation between the clinical features of POTS and serum levels of autoantibodies against diverse GPCRs.

Mild form of Danon disease: two case reports.

Danon disease is typically lethal by the mid-twenties in male patients due to cardiomyopathy. This report aims to describe two unrelated male patients showing mild manifestations of the disease. A 39-year-old man presented with a 10-year history of elevated serum creatine kinase levels with slowly progressive muscle weakness.

Myoclonic Epilepsy with Ragged-red Fibers with Intranuclear Inclusions.

We herein report a case of myoclonic epilepsy with ragged-red fibers (MERRF) harboring a novel variant in mitochondrial cysteine transfer RNA (MT-TC). A 68-year-old woman presented with progressive myoclonic epilepsy with optic atrophy and peripheral neuropathy. A skin biopsy revealed p62-positive intranuclear inclusions.

[A case of chronic postural tachycardia syndrome with positive anti-ganglionic acetylcholine receptor (gAChR) antibody].

Postural orthostatic tachycardia syndrome (POTS) is a form of orthostatic intolerance characterized by symptoms such as lightheadedness, fainting, and brain fog that occur with a rapid elevation in heart rate when standing up from a reclining position. The etiology of POTS has yet to be established. However, a growing body of evidence suggests that POTS may be an autoimmune disorder such as autoimmune autonomic ganglionopathy, an acquired, immune-mediated form of diffuse autonomic failure.

Midbrain atrophy related to parkinsonism in a non-coding repeat expansion disorder: five cases of spinocerebellar ataxia type 31 with nigrostriatal dopaminergic dysfunction.

Background: Spinocerebellar ataxia type 31 (SCA31) is caused by non-coding pentanucleotide repeat expansions in the BEAN1 gene. Clinically, SCA31 is characterized by late adult-onset, pure cerebellar ataxia. To explore the association between parkinsonism and SCA31, five patients with SCA31 with concomitant nigrostriatal dopaminergic dysfunction (NSDD) development, including three cases of L-DOPA responsive parkinsonism, were analyzed.

Iliopsoas Hematomas in a Patient with Progressive Encephalomyelitis with Rigidity and Myoclonus.

Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare and severe syndrome characterized by rigidity of the limb and truncal muscles, brainstem signs, myoclonus, and hyperekplexia. Iliopsoas hematoma is a serious complication of bleeding disorders that occurs most commonly in patients with hemophilia and also in association with anti-coagulant drug treatment. We herein present a case of PERM complicated with bilateral iliopsoas hematomas.