Efficacy of Intrathecal Isoniazid and Steroid Therapy in Refractory Tuberculous Meningitis.

Tuberculous meningitis is an infectious disease with high mortality. Literature describing intrathecal therapy for tuberculous meningitis is scarce. We herein report a case of refractory tuberculous meningitis in a 52-year-old woman with underlying neuropsychiatric systemic lupus erythematosus. Despite systemic treatment with anti-tuberculosis drugs and dexamethasone, her meningeal irritation deteriorated. Intrathecal isoniazid and prednisolone administration was therefore initiated, and the symptoms of severe meningeal irritation improved along with head magnetic resonance imaging and cerebrospinal fluid findings. This case report highlights the efficacy of intrathecal isoniazid and steroid injections for refractory tuberculous meningitis, particularly in patients with severe meningeal irritation.

Impact of width of susceptibility vessel sign on recanalization following endovascular therapy.

BACKGROUND AND PURPOSE: We aimed to investigate the relationship between arterial recanalization following endovascular therapy and the susceptibility vessel sign (SVS) length and width on susceptibility-weighted imaging. METHODS: We retrospectively evaluated consecutive patients with anterior circulation ischemic stroke who underwent magnetic resonance imaging preceded endovascular therapy, and measured the SVS length and width. Successful recanalization was defined as expanded thrombolysis in cerebral infarction grade of 2b to 3. Logistic regression analysis was executed to determine the independent predictors of successful recanalization and first-pass reperfusion (FPR) after endovascular therapy. RESULTS: Among 100 patients, successful recanalization and FPR were observed in 77 and 34 patients, respectively. The median SVS length and width were 10.3 mm (interquartile range, 6.8-14.1 mm) and 4.2 mm (interquartile range, 3.1-5.2 mm), respectively. In multivariate logistic regression analysis, SVS width was associated with successful recanalization (odds ratio, 1.88; 95% confidence interval, 1.14-3.07; p = 0.005) and FPR (odds ratio, 1.38; 95% confidence interval, 1.01-1.89; p = 0.039). The optimal cutoff value for the SVS width to predict successful recanalization and FPR were 4.2 mm and 4.0 mm, respectively. CONCLUSIONS: Larger SVS width may predict successful recanalization and FPR following endovascular therapy.

Perfusion abnormality in neuronal intranuclear inclusion disease with stroke-like episode: A case report.

Neuronal intranuclear inclusion disease (NIID) is a slowly progressive neurodegenerative disease. Some patients with NIID occasionally present with acute symptoms. However, its mechanism remains unclear. We report a patient with NIID who presented with a stroke-like episode. Arterial spin labeling magnetic resonance imaging revealed hypoperfusion in the focal cerebral region at the onset while no apparent arterial occlusion was observed. The abnormal perfusion area was normalized 6 days after admission. Therefore, the perfusion abnormality was likely the main cause of acute neurologic deficits in NIID. NIID should be considered in the differential diagnosis of stroke mimics.

Motor end-plate analysis to diagnose immune-mediated myasthenia gravis in seronegative patients.

This study aimed to evaluate the diagnostic usefulness of motor end-plate (MEP) analysis along with clustered acetylcholine receptor (AChR) antibody (Ab) assays in patients with myasthenia-like symptoms but negative routine AChR and muscle-specific kinase (MuSK) Ab tests. MEP analysis of muscle biopsies of the biceps brachii was performed in 20 patients to try to differentiate between those with or without immune-mediated myasthenia gravis (MG). Using a quantitative method, complement C3 deposition and AChR densities in MEPs were examined. Independently, cell-based assays were used to detect serum clustered-AChR Abs. Only five of 20 patients had complement deposition at MEPs; four of these patients had reduced AChR densities similar to those in patients with typical AChR Ab positive MG, and distinct from those in the remaining 15 patients. Two of the four serum samples from these patients had clustered-AChR Abs. All complement-positive patients were considered as having immune-mediated MG and improved with appropriate treatments; although one patient presented with MG 3 years later, the remaining patients had other diagnoses during over 10 years of follow-up. These results suggest the usefulness of MEP analysis of muscle biopsies in diagnosing immune-mediated MG in seronegative patients with myasthenia-like symptoms but, due to the invasiveness of the muscle biopsy procedure, clustered AChR Abs should, if possible, be tested first.

Influenza A (H3N2) infection followed by anti-signal recognition particle antibody-positive necrotizing myopathy: A case report.

A 60-year-old Japanese woman presented with subacute progressive muscle pain and weakness in her proximal extremities. She was diagnosed with influenza A (H3N2) infection a week before the onset of muscle pain. At the time of admission, she exhibited weakness in the proximal muscles of the upper and lower limbs, elevated serum liver enzymes and creatinine kinase, and myoglobinuria. She did not manifest renal failure and cardiac abnormalities, indicating myocarditis. Electromyography revealed myogenic changes, and magnetic resonance imaging of the upper limb showed abnormal signal intensities in the muscles, suggestive of myopathy. Muscle biopsy of the biceps revealed numerous necrotic regeneration fibers and mild inflammatory cell infiltration, suggesting immune-mediated necrotizing myopathy (IMNM). Necrotized muscle cells were positive for human influenza A (H3N2). Autoantibody analysis showed the presence of antibodies against the signal recognition particle (SRP), and the patient was diagnosed with anti-SRP-associated IMNM. She was resistant to intravenous methylprednisolone pulse therapy but recovered after administration of oral systemic corticosteroids and immunoglobulins. We speculate that the influenza A (H3N2) infection might have triggered her IMNM. Thus, IMNM should be considered as a differential diagnosis in patients with proximal muscle weakness that persists after viral infections.

An adult case of parainfectious optic neuritis associated with genital herpes simplex virus type 2 infection.

Although acute retinal necrosis (ARN) and optic neuritis following herpes encephalitis are known causes of acute visual impairment associated with herpes simplex virus (HSV) infection, there have been no reports of parainfectious optic neuritis associated with genital HSV type 2 (HSV-2) infection. A young Japanese woman developed unilateral optic neuritis 7 days after the onset of genital HSV-2 infection. Ophthalmologic examination revealed no findings suggestive of ARN and both multiple sclerosis and neuromyelitis optica were ruled out by the brain images and serum antibody testing. An oral steroid therapy improved her symptoms. Here, we describe the first case of parainfectious optic neuritis associated with genital HSV-2 infection.

Neuromyelitis Optica Spectrum Disorder Complicated by Posterior Reversible Encephalopathy Syndrome as an Initial Manifestation.

A 25-year-old woman was admitted to our hospital due to tonic convulsion with severe headache after having experienced symptoms of nausea and vomiting for a month. Brain magnetic resonance imaging showed extensive symmetrical lesions in the cortical and subcortical areas of parieto-occipital lobes and basal ganglia, consistent with typical characteristics of posterior reversible encephalopathy syndrome (PRES). Furthermore, some residual lesions in the left side of dorsal medulla oblongata and central area of the cervical spinal cord along with the presence of serum anti-aquaporin-4 antibody yielded the diagnosis of neuromyelitis optica spectrum disorder (NMOSD). We herein discuss the mechanism by which PRES may occur together with NMOSD.

[A Case of Flexion Contracture Associated with Hypopituitarism].

A 72-year-old Japanese man developed progressive disturbance in ambulation with flexion contractures 5years before this admission. At 49 years of age, he was diagnosed with hypopituitarism after an operation for a Rathke's cleft. On admission, he could not fully extend his knees and hips because of painful muscle stiffness of the lower extremities. Initially, we suspected Stiff-person syndrome and initiated diazepam, which had no effect. Serum anti-glutamic acid decarboxylase antibody was negative. Next, we suspected flexion contractures associated with hypopituitarism. Endocrine evaluation revealed that ACTH, cortisol, and other hormone levels were lower than those reported in the previous evaluation. We treated the patient with hydrocortisone and his symptoms dramatically improved. It is rare for patients with hypopituitarism to have flexion contracture. This case suggests that we should consider hypopituitararism in the setting of flexion contractures. (Received October 18, 2018; Accepted March 26, 2019; Published June 1, 2019).

Two novel VCP missense variants identified in Japanese patients with multisystem proteinopathy.

VCP mutations were first associated with inclusion body myopathy with Paget's disease of bone and frontotemporal dementia (IBMPFD) but was later associated with amyotrophic lateral sclerosis and Charcot-Marie-Tooth disease. Now, a new name, "multisystem proteinopathy (MSP)", is proposed for this condition. VCP encodes valosin-containing protein, which is involved in protein degradation in the ubiquitin proteasome system. We report here two MSP patients with two novel heterozygous missense variants in VCP: c.259G>T (p.Val87Phe) and c.376A>G (p.Ile126Val).

Hiding an image with a light-scattering medium and use of a contrast-discrimination method for readout.

Hiding image data with a material such as a light-scattering medium is useful as an initial stage of data protection, because the hidden image can be detected only by observation with a specific technique. A light-scattering medium is used to hide the image data, and a low-temporal-coherence interferometer performs the readout processing. A new readout method for detecting pixel values of the image is proposed to overcome spatial variation of the light intensity and distortion of the interference fringes. The introduction of spatial coding further improves the performance by overcoming spatial variations of the light-scattering medium and variations in the reflectance of given pixels.