Characteristic cerebrospinal fluid findings in immune checkpoint inhibitor-related peripheral neuropathy: A case report.

Although immune checkpoint inhibitors (ICIs) are widely used to treat unresectable malignant tumors, they can cause undesirable side effects called immune-related adverse events, including neurological toxicities. Here, we describe a case of ICI-related peripheral neuropathy (irPN) with characteristic cerebrospinal fluid (CSF) findings. In addition to pleocytosis and increased protein levels, the present case showed increased levels of CSF soluble interleukin-2 receptor (sIL-2R), IL-6, and IL-10, suggesting activated T cell-related autoimmunity. We believe that CSF cytokines and sIL-2R could be novel biomarkers of irPN.

Diagnostic Utility of Polymerase Chain Reaction for Paraffin-embedded Sinus Specimens for Rhinocerebral Mucormycosis Complicated by Internal Carotid Artery Thrombosis and Cerebral Infarction.

We herein report a 73-year-old man who experienced cerebral infarction caused by infection with a Mucromycocetes species. A delay in anti-fungal treatment might result in a lethal clinical outcome. We were unable to establish an accurate diagnosis based on histological findings and cerebrospinal fluid culture. Therefore, we performed polymerase chain reaction (PCR) using paraffin-embedded specimens, and based on the findings, successfully started administering anti-fungal treatment. We suggest that PCR using sinus specimens be applied when mucormycosis is suspected as an etiology of cerebral infarction and a confirmative diagnosis cannot be established based on the results of pathological examinations or cerebrospinal fluid culture.

Periventricular White Matter Lesions Influence Gait Functions in Parkinson's Disease.

OBJECTIVE: We quantitatively evaluated the gait of Parkinson's disease (PD) patients over a 10-m course during normal walking and during dual-task walking while performing a calculation task, and clarified which parts of white matter lesions (WML) influence gait in PD patients. METHODS: Gait parameters, including walking speed, gait cycle, stride length, and left-right instability, were measured in 64 PD patients and 20 controls who walked 10 m with normal gait and as they were performing a calculation task. WML on magnetic resonance imaging (MRI) of PD patients were scored according to Scheltens' criteria, and associations with gait parameters were investigated. RESULTS: Compared to controls, the PD group showed decreased walking speed and narrowed stride (p < 0.05), and the stride length and step time coefficient of variation changed significantly during the calculation task (p < 0.001). Frontal lobe functions correlated positively with walking speed and stride during the calculation task in patients with PD (p < 0.05). The total score for periventricular hyperintensity (PVH) on MRI correlated with walking speed and stride (p < 0.01). Multiple regression analysis revealed significant correlations between walking speed and frontal cap of PVH, and between stride and occipital cap (p < 0.05). CONCLUSION: Gait of PD patients deteriorated not only due to motor dysfunction but also due to mental burden in association with frontal lobe function and periventricular lesions of cerebral white matter.

Ischemic Stroke due to Virologically-Confirmed Varicella Zoster Virus Vasculopathy: A Case Series.

BACKGROUND: Limited data are available regarding the characteristics and prognosis of patients with stroke due to varicella zoster virus (VZV) vasculopathy. METHODS: We studied 4 patients (2 men and 2 women; age, 38-63 years) from a single center who developed acute ischemic stroke due to VZV vasculopathy. The virological diagnosis was confirmed by detecting VZV DNA and/or the IgG antibody to VZV in the cerebrospinal fluid. RESULTS: Three patients were taking immunosuppressive agents, including prednisolone and/or methotrexate, at baseline. Each patient had a characteristic skin rash prior to stroke, with the interval from rash to stroke onset ranging from 13 to 122 days. Two patients experienced antecedent cranial nerve palsies; one had the third, seventh, ninth, and 10th nerve palsies and the other had the fourth nerve palsy before stroke. Cerebral infarctions were located in the anterior circulation lesion (n = 1), in the posterior circulation lesion (n = 2), and in both lesions (n = 1). Intracranial arterial stenosis was only identified in one patient on magnetic resonance angiography. A high plasma d-dimer level was detected in 1 patient, whereas high ß-thromboglobulin and platelet factor 4 levels were detected in 2 patients. As a result of combined therapies with acyclovir, steroid, and antithrombotic agents, neurological symptoms markedly improved in 3 patients, whereas 1 patient was left with moderate hemiplegia. CONCLUSIONS: Cranial nerve palsies may be prodromal symptoms of VZV-associated stroke. Increased levels of thrombotic markers may support the use of antithrombotic agents, although the benefit of combined treatment should be determined through larger studies.

Charcot-Marie-Tooth disease type 4C in Japan: report of a case.

INTRODUCTION: The distribution of documented cases of Charcot-Marie-Tooth disease type 4C (CMT4C) is mainly limited to the Mediterranean region. We report the first documented case of CMT4C in East Asia. Furthermore, we estimate the proportion of CMT4C in Japan and compare the same with that in European countries. CASE REPORT: A 72-year-old Japanese woman presented with early-onset motor and sensory neuropathy associated with scoliosis, deformities of the hands and feet, and carpal tunnel syndrome. A genetic screen detected a homozygous p.R529Q mutation in SH3TC2, the causative gene of CMT4C. The SH3TC2 mutation identified here is unique among 426 unrelated Japanese CMT patients, excluding those with CMT1A. CONCLUSIONS: Although CMT4C also occurs in Japan, it is less common than in European countries.