We report a 44-year-old woman who had anti-aquaporin 4 (AQP4) antibody-positive myelitis and immune thrombocytopenic purpura (ITP). She was admitted to our hospital with paraparesis, dysesthesia below the Th8 dermatome level on her right side and lower extremities, constipation and urinary retention. Magnetic resonance imaging revealed a longitudinally extending lesion at the level of Th4–Th10. Her serum sample was positive for anti-AQP4 antibody. Corticosteroid therapy was initiated, and her symptoms were largely ameliorated. Furthermore, concurrently with the myelitis, her platelet count dropped (99 × 10⁹/l). A diagnosis of ITP was made with positive serum platelet-associated IgG (PA-IgG) and negative work-up for blood malignancies by bone marrow aspiration. Since a causal relationship between Helicobacter pylori (H. pylori) and ITP is suggested by several studies, she was also examined and diagnosed with H. pylori-positive ITP. After the bacteria eradication therapy, her platelet count and PA-IgG returned to normal range. Furthermore, the anti-AQP4 antibody titer declined and her symptoms were almost resolved. We considered that H. pylori might influence progression of the myelitis as well as induction and development of ITP.

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A 35-year-old woman was admitted with subacute intellectual deterioration. Laboratory studies showed elevated total homocysteine and decreased folic acid. MRI revealed leukoencephalopathy with a posterior predominance, and hyperintensity in the pyramidal tracts on T₂-weighted and FLAIR images. The enzyme assay showed a deficiency of methylenetetrahydrofolate reductase (MTHFR) activity with low residual activity of 4.2% of the mean control value in cultured fibroblasts. Sequence analysis of the MTHFR gene demonstrated two homozygous missense mutations, c.677C>T (p.Ala222Val) and c.685A>C (p.Ile225Leu). c.677C>T (p.Ala222Val) is known as a common polymorphism and c.685A>C (p.Ile225Leu) is considered to be a novel polymorphism. A diagnosis of MTHFR deficiency was made. Treatment with folic acid, vitamin B₁₂ and B₆ made significant improvement of intellectual deterioration and reduction in the total homocysteine level. They also made marked resolution of leukoencephalopathy. Posterior-predominant leukoencephalopathy was found to be an excellent marker of MTHFR deficiency, and may help to establish the diagnosis.

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A 60-year-old woman suffered from high fever（38°C）and abnormal behavior, was admitted to our hospital on the seventh day of the fever. At admission, she was stuporous, and a cerebrospinal fluid (CSF) analysis revealed pleocytosis (55/μl, monocytes). Fluid-attenuated inversion recovery (FLAIR) magnetic resonance (MR) images showed high-intensity signals in the medial temporal lobe, inferior surface of the frontal cortex, right cerebellar vermis, and left thalamus. We diagnosed herpes simplex encephalitis, based on the finding of an elevated titer of herpes simplex virus antibody in the CSF (2.90). She was started on treatment with acyclovir and steroid pulse therapy, which was followed by rapid clinical improvement. After recovering from the stupor, the patient exhibited the symptoms of hypersomnia with low orexin level in the CSF. Thus, we should bear in mind that other than consciousness disturbance, patients with herpes simplex encephalitis can also present with rare complications due to the extent of the lesions.

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A 44-year-old woman with a history of transient right hemiparesis presented with personality change. One year later, she was admitted with ophthalmoparesis, dysarthria and regression phenomenon. MRI indicated acute infarction of the paramedian region of the midbrain and a nodular lesion in the interpeduncular fossa with contrast enhancement. Two years later, the patient was admitted with sudden onset of right hemiplegia. MRI showed acute infarction in the left side of the pons, diffuse brain atrophy, and abnormal contrast enhancement in the nodular lesion of interpeduncular fossa and leptomeninges of the ventral pons. MR angiography revealed that cerebral main tracts were intact, and cerebrospinal fluid analysis revealed mild pleocytosis and slightly elevated protein levels. Cervical lymph node biopsy demonstrated caseating granuloma with acid-fast bacilli. The patient was diagnosed with chronic tuberculous meningitis, even though tuberculous bacilli were not detected on polymerase chain reaction (PCR) or in culture. Antituberculous medication resulted in radiological resolution and neurological improvement. Although the patient had mild headache and pyrexia at the first admission, no signs of meningeal irritation were confirmed throughout the clinical course. We suspect that a paucity of tuberculous bacilli released from the tuberculous foci in the meninges to the subarachnoid space caused prolonged clinical course and lack of meningeal irritation signs.

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A 59-year-old man was admitted to our hospital because of sudden weakness in his left foot. He had been treated for lung cancer by chemotherapy and irradiation 3 years earlier. Brain magnetic resonance (MR) imaging revealed multiple acute cerebral infarctions in the area of the right anterior cerebral artery. MR angiography (MRA) revealed that the right anterior cerebral artery was patent, with slight irregularity in the A3 portion. He was treated by administration of aspirin (200 mg/day) and a continuous intravenous unfragmented heparin infusion (10,000 IU/day). Four days after admission, he developed dyspnea. Chest computed tomography (CT) performed 5 days after admission revealed both a marked pericardial effusion and a pleural effusion. Emergency pericardiocentesis was therefore performed. While 1,000 ml of bloody pericardial effusion were aspirated, his dyspnea ameliorated dramatically. Histological examination of the pericardial effusion revealed infiltration of lung adenocarcinoma cells in the pericardium. Intracranial 3D-CT angiography revealed the pearl and string sign in the right anterior cerebral artery 6 days after admission. Anterior cerebral artery dissection was diagnosed as the cause of his cerebral infarction. It is important to recognize the possibility of cardiac tamponade as an uncommon complication of the treatment for acute cerebral infarction.

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We report a case of reversible hepatic myelopathy. A 42-year-old female patient with 3-year history of alcoholic liver cirrhosis developed spastic gait, hyperreflexia and mild somatosensory disturbance in her lower extremities. The increased level of serum ammonia and the deficits of N30 and P38 in the tibial somatosensory evoked potentials (SEP) in conjunction with exclusion of the other known causes of myelopathy supported the diagnosis of her hepatic myelopathy. The ammonia lowering therapy by the oral administration of lactulose successfully improved the spastic gait accompanied with the emergence of N30 and P38 in the tibial SEP. Although liver transplantation was known to be the only therapy for hepatic myelopathy in the literatures, our case showed that the ammonia lowering therapy can be effective for the early stage of hepatic myelopathy.

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A 24-year-old woman was referred to our hospital because of impaired consciousness after influenza virus B infection. Neurological examination revealed mild disturbance of consciousness without other neurological abnormalities. Laboratory tests showed elevated serum CRP, IL-6 and TNF-α levels. The level of IL-6 in the cerebrospinal fluid was also slightly elevated. Electroencephalography (EEG) disclosed almost continuous generalized spike and wave complexes and multiple spikes and wave complexes at 1.5–3 Hz. Brain MRI was normal. She was diagnosed as having influenza encephalopathy presenting non-convulsive status epilepticus (NCSE), and commenced methylprednisolone pulse therapy followed by prednisolone with gradual tapering. She was also treated with intravenous phenytoin and oral sodium valproate for NCSE. The next day, her consciousness level had improved. Although she became alert, epileptic discharges on EEG were still observed on the seventh hospital day, and levetiracetam was added. Then, her epileptic discharges almost completely disappeared on the twelfth hospital day. She was discharged without any neurological deficit. We consider this patient to be a case of transient NCSE due to influenza encephalopathy; alternatively, she may have epileptic traits and her NCSE may have been provoked by influenza virus infection.

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We report a 58-year-old woman with bronchial asthma. The onset of the disease was marked by numbness in the right lower extremity, for which she was hospitalized 10 days later. The patient presented with sensory impairment and muscle weakness in the distal regions of both lower limbs, acute pain, purpura, and a leukocyte count of 2.4 × 10⁴/μl (59.2% eosinophils). Nerve conduction tests revealed a decrease in the amplitude of the compound muscle action potential in all 4 extremities. Skin biopsy results led to the diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Steroid pulse therapy and oral steroid therapy were initiated but did not resolve the acute pain or numbness. However, intravenous immunoglobulin (IVIg) was administered at day 28 after the beginning of the steroid treatment, and the pain started to improve immediately afterward. In some cases, IVIg can be effective in the treatment of intense pain in peripheral neuropathy associated with steroid-resistant EGPA.

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A 67-year-old man living alone was admitted for acute disturbance of consciousness during winter. He presented with semicoma, a decorticate posture, and exaggerated tendon reflexes of the limbs, but brainstem reflexes were intact. The carboxyhemoglobin (COHb) level was normal in arterial blood gas on admission, and protein in cerebrospinal fluid was increased without pleocytosis. Brain MRI showed diffuse T₂ high intensities in the deep white matter bilaterally without a contrast effect and abnormal T₁ intensity in the pallidum. ¹H-MR spectroscopy (MRS) of the white matter lesion demonstrated findings suggesting demyelination as an increased choline peak, enhanced anaerobic metabolism as increased lactate and lipids peaks, and reduced neurons as a decreased N-acetylaspartate peak, which corresponded to delayed encephalopathy due to the interval form of carbon monoxide (CO) poisoning. The possibility of CO exposure due to coal briquette use 2 weeks before the symptomatic onset was indicated by his family, so he was diagnosed with CO poisoning. His consciousness slightly improved with corticosteroid therapy and repetitive hyperbaric oxygen therapy, but brain MRI and MRS findings did not improve. Characteristic MRS findings of leukoencephalopathy are helpful for diagnosing the interval form of CO poisoning in the case of a normal COHb level.

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A 72-year-old male with heart failure was admitted to our hospital. Treatment with dabigatran (220 mg per day) was initiated because of atrial fibrillation. On the third day, the patient developed left-sided hemiparesis and dysarthria at 4.5 hr after the last dose of dabigatran. The activated partial thromboplastin time (APTT) was 39.1 sec, and the NIHSS (NIH Stroke Scale) was 11. An intravenous infusion of rt-PA was administered at 160 min after the onset of hemiparesis (at 7 hr after the last dose of dabigatran). Although diffusion weighted MRI revealed a minor infarction in the right lower frontal gyrus, the patient was discharged without hemorrhage (NIHSS 0), and the score on the modified Rankin scale assessed 3 months later was 0. The outcomes have been good in 8 out of 9 reported cases, including the present case; the remaining severe case developed complicating intracranial hemorrhage. Thrombolytic therapy could be safe, if it is performed more than 7 hr after the last dose of dabigatran and the APTT is less than 40 sec.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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