[Seroconversion of anti-aquaporin-4 antibody in a patient with neuromyelitis optica spectrum disorder: a case report].

We report a case of a patient with neuromyelitis optica spectrum disorder (NMOSD) who was originally treated for multiple sclerosis (MS) due to a negative anti-aquaporin-4 (AQP4) antibody test, but later the antibody titer became positive. A 58-year-old woman without prior medical history developed acute left facial pain, vomiting, and hiccups. MRI showed an intraparenchymal lesion extending from the medulla oblongata to cervical cord with high T2-weighted signal intensity. The patient responded to steroid pulse therapy. However, she developed diplopia and gait disturbance after six months, and follow-up MRI revealed a new lesion in the left middle cerebellar peduncle. The patient was diagnosed with MS due to the dissemination in the time and space of her lesions and negative anti-AQP4 antibody status. There was no relapse for three and a half years on fingolimod therapy. However, a severe relapse occurred four years later that involved optic neuritis and multiple new brain lesions. During this episode, the anti-AQP4 antibody test was positive, and the patient was diagnosed with NMOSD. This case highlights the possibility that seroconversion of anti-AQP4 antibody may occur at any time in NMOSD patients. Therefore, this diagnostically paramount antibody should be measured several times during the treatment of relapsing-remitting MS in patients with repeat frequent recurrences and uncommon symptoms of MS.

Stroke Systems of Care During the COVID-19 Epidemic in Kobe City.

BACKGROUND: The novel coronavirus disease 2019 (COVID-19) outbreak raised concerns over healthcare systems' ability to provide suitable care to stroke patients. In the present study, we examined the provision of stroke care in Kobe City during the COVID-19 epidemic, where some major stroke centers ceased to provide emergency care. METHODS: This was a cross-sectional study. The Kobe Stroke Network surveyed the number of stroke patients admitted to all primary stroke centers (PSCs) in the city between March 1 and May 23, 2020, and between March 3 and May 25, 2019. In addition, online meetings between all PSC directors were held regularly to share information. The survey items included emergency response system characteristics, number of patients with stroke hospitalized within 7 days of onset, administered treatment types (IV rt-PA, mechanical thrombectomy, surgery, and endovascular therapy), and stroke patients with confirmed COVID-19. RESULTS: During the period of interest in 2020, the number of stroke patients hospitalized across 13 PSCs was 813, which was 15.5% lower than that during the same period of 2019 (p = 0.285). The number of patients admitted with cerebral infarction, intracerebral hemorrhage, and subarachnoid hemorrhage decreased by 15.4% (p = 0.245), 16.1% (p = 0.659), and 14.0% (p = 0.715), respectively. However, the rates of mechanical thrombectomy and surgery for intracerebral hemorrhage were slightly increased by 12.1% (p = 0.754) and 5.0% (p = 0.538), respectively. PSCs that ceased to provide emergency care reported a decrease in the number of stroke cases of 65.7% compared with the same period in 2019, while other PSCs reported an increase of 0.8%. No case of a patient with stroke and confirmed COVID-19 was reported during the study period. CONCLUSION: Kobe City was able to maintain operation of its stroke care systems thanks to close cooperation among all city PSCs and a temporal decrease in the total number of stroke cases.

[Endogenous endophthalmitis following staphylococcus aureus meningitis].

Endogenous endophthalmitis is a rare complication of acute meningitis. A healthy 82-year-old woman suddenly developed septic bacteremia and meningitis owing to Staphylococcus aureus (methicillin-susceptible Staphylococcus aureus); she presented with a high fever and drowsiness. Improvement of these initial symptoms unveiled impaired bilateral visual acuity. Detailed ophthalmological assessments diagnosed it as probable endogenous endophthalmitis, which was immediately treated with vitrectomy. Subsequently, the patient totally recovered without any neurologic or ophthalmic sequela.

Familial adult-onset Alexander disease with a novel mutation (D78N) in the glial fibrillary acidic protein gene with unusual bilateral basal ganglia involvement.

In this report, we describe the case of a new Japanese family (32 to 64 years old; 2 females and 1 male) affected by adult-onset Alexander disease. Clinically, one member (age at onset, 56 years old) developed cerebellar ataxia, another (age at onset, 55 years old) showed cerebellar ataxia and pseudobulbar signs, and one member (32 years old) was asymptomatic. Marked atrophy of the medulla oblongata and spinal cord was detected in the two symptomatic patients by magnetic resonance imaging (MRI). However, in the asymptomatic patient, cervicomedullary atrophy was mild. Hyperintensity signals in the medulla oblongata were detected in the two symptomatic patients, but not in the asymptomatic patient. In addition, there are symmetrical hyperintensity signals in the posterior part of the globus pallidus on T2-weighted images in the two symptomatic patients, which are rarely observed in adult-onset Alexander disease. Molecular genetic analysis revealed a novel missense mutation (p. D78N) in the glial fibrillary acidic protein (GFAP) gene in this family. The typical atrophy of the medulla oblongata and upper cervical cord detected by MRI is the diagnostic feature of adult-onset Alexander disease. Genetic analysis of the GFAP gene is recommended for all patients with late-onset progressive ataxia and suspected of having adult-onset Alexander disease on the basis of MRI findings. Additionally, these characteristic MRI patterns might even lead to the identification of asymptomatic cases, as in one of our cases.

[Body lateropulsion as the main symptom of pontine vascular disease--comparison with lateral medullary vascular disease].

Lateropulsion of the body is rarely encountered as an isolated or predominant manifestation of pontine cerebrovascular disease. We compared 2 cases of pontine vascular lesions with 3 cases of lateral medullary infarction; all the patients had presented with body lateropulsion. The patients with pontine lesions tended to full on the side contralateral to the lesion,whereas the patients with lateral medullary infarction, fell on the ipsilateral side. Lateropulsion itself improved within 1 or 2 weeks in all the patients. The pontine lesion was localized to the paramedian tegmentum, just ventral to the fourth ventricle, while the medullary infarction was localized to the lateral surface of the medulla oblongata. The present findings support the possibility that body lateropulsion in patients with pontine vascular lesion is attributed to lesions in the graviceptive pathway that ascends through the paramedian pontine tegmentum.

Hepatitis B virus-related vasculitis manifesting as severe peripheral neuropathy following influenza vaccination.

We report the case of a 68-year-old man who developed hepatitis B virus (HBV)-related vasculitis, manifesting as mononeuritis multiplex, 8 days after influenza vaccination. The patient was a carrier of wild-type HBV, and had never received influenza vaccination. Histologic examination of the left sural nerve revealed necrotising vasculitis predominantly involving small blood vessels. HbsAg deposits were observed at a high density around the epineurial blood vessels of the sural nerve. He was treated with prednisone and Lamivudine. His condition improved gradually. However, seroconversion of HBs and HBe Ag was not detected. At the last follow-up 2 years later, his vascilitis did not recur. On the basis of the time of onset of vasculitis, the presumptive immune-mediated pathology of this disorder suggests a possible etiologic link with influenza vaccine. To our knowledge, our case is the first to show vasculitis of peripheral nerves proven pathologically after influenza vaccination.

Delirium in two patients with Bickerstaff's brainstem encephalitis.

In this report, we describe the case of two patients with Bickerstaff's brainstem encephalitis (BBE) who developed delirium manifested as emotional incontinence, restlessness, and aggressive behavior from disease onset. Serum anti-GQ1b and anti-GT1a IgG antibodies were detected in both patients. When unusual psychiatric symptoms are observed, in addition to acute ophthalmoplegia and ataxia, neurologists should take into account the possibility of BBE. Brain MRI findings were normal in both patients and SPECT was performed on only patient 1. SPECT of patient 1 showed reversible hypoperfusion in the brainstem, bilateral thalami, and medial frontal lobe. Brain SPECT appears to be useful for detecting lesions of the brainstem as well as the basal ganglia or cerebrum in BBE.

Paradoxical cerebral embolism as the initial symptom in a patient with ovarian cancer.

This report concerns a 37-year-old patient with ovarian cancer and a paradoxical cerebral embolism as the initial symptom. She developed acute onset of left quadrantic hemianopia during coughing. Brain magnetic resonance imaging showed an acute multiple infarction, and a simultaneous acute pulmonary embolism was observed. Transesophageal echocardiography showed a patent foramen ovale, multidetector row computed tomography an ovarian tumor and infarction of the spleen, whereas multidetector row computed tomography venography showed right iliac vein compression by the ovarian tumor. The diagnosis was stage Ic ovarian cancer. Because blood stasis of the pelvic vein is a major risk factor for venous thrombosis, the presence of a patent foramen ovale should alert physicians to examine not only veins in the lower extremities but also the pelvic and intra-abdominal veins as a source paradoxical embolism.

[Successful use of methylprednisolone therapy in a case of non-herpetic acute encephalitis with antibodies against ionotropic glutamate receptor epsilon2 and delta2].

This report concerns a 32-year old man with non-herpetic acute limbic encephalitis. He was admitted to our hospital because of high fever and consciousness disturbance. Cranial MRI revealed abnormal signal intensities in the bilateral mesial temporal lobes. On the second hospitalization day, he developed status epilepticus, which necessitated general anesthesia. Following administration of intravenous (IV) methylprednisolone pulse therapy (1 g/day, 3 days), his consciousness disturbance began to improve. Treatment with high-dose IV methylprednisolone was continued for about 2 weeks, but on the 7th day, he showed severe anterograde amnesia and retrograde amnesia regarding the preceding 5 or 6 years. Subsequently, however, his amnesic disorders improved markedly, and on the 46th day, memory dysfunction had disappeared. Autoantibodies against the glutamate receptor subunits epsilon2 and delta2 were detected in both the CSF and serum, but these antibodies in the CSF became normal during the clinical course. The voltage-gated potassium channel antibody was negative. This case report indicates that high-dose IV methylprednisolone therapy may be an affective treatment for non-herpetic acute limbic encephalitis, possibly associated with autoimmune mechanisms.

A case of diabetic amyotrophy with severe atrophy and weakness of shoulder girdle muscles showing good response to intravenous immune globulin.

A 45-year-old man with insulin-dependent diabetic mellitus developed progressive asymmetrical weakness and atrophy of both shoulder girdle muscles within 1 year. In the last month, he also developed slight weakness of both thighs. Neuropathology of the sural nerve showed an axonal degeneration and perivascular inflammation and electromyography revealed neurogenic changes. Because of a diagnosis of suspected diabetic amyotrophy, intravenous immunoglobulin was administered. This treatment produced marked improvement. Physicians should take into account the possibility of diabetic amyotrophy in patients with diabetic mellitus showing primary involvement of shoulder girdle muscles marked by weakness and atrophy.

[A case of acute oropharyngeal palsy with nasal voice as main symptom].

We report a patient with acute oropharyngeal palsy following enteritis. A 19-year-old woman developed increasing nasal voice over a few days. Neurological examination on day 7 of her course showed paretic dysarthria and mild weakness of neck flexion and quadriceps femoris muscle (Medical Research Council grade, 4+). Her palatal movement was diminished, whereas both palatal and pharyngeal reflex was normal. She could swallow water, although she had a slight amount of liquid reflux to her nose on swallowing. High titers of serum anti-Campylobacter jejuni, anti-GQ1b and anti-GT1a IgG antibodies were detected. Her symptoms improved gradually without any treatment, and disappeared by 40 days from neurological onset. Nasal voice with slight swallowing impairment as initial symptom has been rarely reported, but can occur in acute oropharyngeal palsy. Therefore, neurologists should take into account the possibility of Guillain-Barré syndrome and the regional variants in patients who show nasal voice during the initial stage.

Bilateral iliopsoas hematomas complicating anticoagulant therapy.

An 85-year-old woman receiving anticoagulant therapy for transient ischemic attack suddenly developed bilateral femoral nerve palsy and severe pain in the bilateral groin and thighs. Her platelet count, prothrombin time and activated partial thromboplastin time were within the therapeutic range. Hematomas in the bilateral iliopsoas muscles were clearly detectable on CT scan. The right hematoma was larger than the left one and caused more severe femoral neuropathy, but improved gradually without surgical decompression. This case is reported here because bilateralism is exceptional, and iliopsoas hematoma should be suspected when a patient receiving anticoagulant therapy presents with pain in the groin or thigh.

Infectious endocarditis associated with subarachnoid hemorrhage, subdural hematoma and multiple brain abscesses.

Hemorrhagic stroke is a complication of infectious endocarditis (IE), and severe hemorrhage accompanies Staphylococcus aureus IE during early uncontrolled infection. However, subarachnoid hemorrhage (SAH) or subdural hematoma is rare. A case of S. aureus IE associated with SAH and subdural hematoma in the early stage is reported. A 54-year-old man with a history of mitral valve prolapse presented with fever. Two days after the onset, he fell into a confused state with convulsion and left hemiparesis. He became comatose and brain CT and MRI demonstrated SAH and subdural hematoma with severe right hemisphere swelling. Multiple brain abscesses were also observed. No septic aneurysm was detected by cerebral angiography.

[A patient with primary Sjögren's syndrome featuring polyneuropathy and oculomotor paralysis and associated with asymptomatic left middle cerebral artery stenosis].

We report a case of primary Sjögren's syndrome (primary SjS) with polyneuropathy and right oculomotor paralysis associated with middle cerebral artery stenosis. A 39-year-old woman developed progressive numbness and clumsiness of the limbs. Two months later, right third cranial palsy manifested itself and she was admitted to our hospital. A cranial MRA showed left middle cerebral artery stenosis confirmed by transcranial color doppler sonography. A nerve conduction study showed a decrease in the NCV and reduced CMAP, while sural nerve biopsy showed axonal degeneration and infiltration of inflammatory cells around the small blood vessel walls. The patient complained of dry mouth and a salivary gland biopsy revealed inflammatory changes, while salivary gland scintigraphy showed diminished secretion. These findings led to the diagnosis of Sjögren's syndrome. Reports of primary SjS with involvement of large cerebral arteries are rare. In our case, polyneuropathy and oculomotor paralysis were the manifest symptoms, but middle cerebral artery stenosis was also observed. This indicates that, even in the absence of CNS symptoms, cerebral artery involvement may be present in primary SjS.