The author reviewed the clinical records and neuroradiologic examinations of 86 consecutive patients with orthostatic headache who visited our clinic between April 1995 and December 2014. Fifty-six patients were suspected to have spontaneous intracranial hypotension (SIH). The baseline characteristics of these patients were essentially similar to those reported in other published case series of SIH: female preponderance, mean age of approximately 40 years, and frequent association with nausea, hearing disturbances, or vertigo. In 43 patients who underwent gadolinium-enhanced MRI, 15 had partial dural enhancement and 15 had diffuse enhancement. Of 13 patients who underwent radionuclide cisternography, a direct finding of cerebrospinal fluid (CSF) leakage was demonstrated in six patients. Ordinal scales were formulated for regression of the extent of dural enhancement on cranial MRI (none: 0, partial: 1, diffuse: 2) and severity of orthostatic headache (not so severe: 1, severe: 2). Ordinal logistic regression analysis demonstrated that the extent of dural enhancement was negatively associated with the severity of orthostatic headache. A possible explanation was that patients suspected of having SIH who showed severe orthostatic headache may lack the ability to compensate for CSF loss. Epidural blood patch (EBP) is targeted at the CSF leak site or at the lumbar level when the site of CSF leak has not been determined. The interval from EBP to disappearance of orthostatic headache did not significantly differ in six patients treated with targeted EBP and five patients with lumbar EBP. Linear regression analysis demonstrated that the duration of orthostatic headache was associated with the interval from onset of headache to initial visit to our clinic, with the slope of the regression line 1.243 and intercept 14.8 days. Thus, early diagnosis of SIH appeared to correlate with earlier disappearance of orthostatic headache. No other factors were found to predict the outcome of SIH.

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We experienced 13 cases (29.8 ± 7.0 years) of mumps meningitis and 365 cases of adult aseptic meningitis during 11 years from 2004 to 2014. A small epidemic of mumps occurred for 3–4 years, and the incidence rate of adult mumps meningitis coincided with the epidemic without seasonal fluctuation. Parotitis was observed in 8 of the 13 mumps meningitis patients (61.5%) and orchitis in 2 of 7 male patients (28.6%). There were no differences in clinical manifestations, laboratory findings, and outcome between patients with adult mumps meningitis and those with echovirus 9 meningitis (9 patients), except for the low frequency of nausea/vomiting and a high percentage of mononuclear cells of the cerebrospinal fluid in those with mumps. Eight patients had contact with persons with mumps before the symptomatic stage of meningitis. Only one patient had received mumps vaccination in childhood. On the basis of the values of the anti-mumps IgM and IgG antibodies, we speculated primary infection and the re-infection of mumps in 6 and 2 patients, respectively. Moreover, second vaccine failure was suggested in the vaccinated patient.

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It has been suggested that many physicians feel it is difficult to manage patients with Duchenne muscular dystrophy (DMD) and that support from experts is required. Therefore, to assess the effects of Japanese practical guidelines for DMD, we distributed a survey questionnaire to certified neurologists and child neurologists in Japan. The survey revealed the actual state of medical care for patients with DMD in Japan prior to publication of guidelines. Many pediatric patients visited academic medical centers (AMCs) and general hospitals (GHs). In contrast, adult patients visited mainly National Hospital Organization (NHO) hospitals and other hospitals that can manage cardiopulmonary care and inpatient therapy. Medical insurance approval for steroid therapy was well known by subjects surveyed. Beta-blockers and angiotensin converting enzyme inhibitors were widely used as cardioprotective agents. However, the rates of obtaining written informed consent before genetic testing, regular radiological testing for scoliosis, usage of mechanical-assisted cough, disaster response plan instruction for patients receiving mechanical ventilation, and management of patients with female dystrophinopathy were below satisfactory. More than 20% of doctors surveyed practiced or recommended muscle strengthening training. Although the details of the training were not specified, this may indicate overuse of resistance training exercises. Our goal is to promote an educational campaign to better disseminate best practice care and clinical guidelines. We plan to conduct another round of surveys in several years to assess the effects of the clinical guidelines.

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A 66-year-old man was admitted to our hospital with acute paraplegia. He has suffered from hypertension with renal dysfunction for 7 years. Five months before admission, hemodialysis was introduced to him because of chronic renal failure due to renal sclerosis. One week before hospitalization, he noticed dizziness, sensory disturbance below the chest, and a urinary difficulty. Two days prior to admission, he could not walk independently. Spinal MRI revealed a mass at the 7th level of the cervical spine, showing low intensity on T₁-weighted image and surrounding hypointensity with inner mixed intensity on T₂-weighted image. An urgent surgery removed the cervical epidural mass and the following pathological evaluation established the diagnosis of β2-microglobulin amyloidoma. Hemodialysis-related amyloidoma generally emerges after a long duration of hemodialysis, demonstrates an insidious onset of symptoms, and is very rare. The current case indicates that we need to be aware of the possibility of β2-microglobulin amyloidoma even in patients with a short history of hemodialysis with a rapid presentation.

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A 25-year-old woman developed numbness of the right flank two weeks after a one-month stay in Thailand and Laos, which are known as areas of angiostrongylosis cantonensis infections. The signs were numbness, pain sense disorder, and vibration sense disorder on the region of the 6th to 12th dermatome. On a MRI T₂ weighted image (T₂WI), signal hyperintensity in a longitudinal spinal lesion was seen. In the cerebrospinal fluid (CSF), eosinophils were detected. The patient was diagnosed with eosinophilic meningitis and myelitis, and then treated with intravenous methylprednisolone. This improved her signs, CSF and MRI findings. She took no drugs, did not have any allergies, any vasculitis, or neuromyelitis optica spectrum disorders. Although anti-parasite antibodies were not identified, she was probably infected by angiostrongylus cantonensis from her history and examinations.

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A 73-year-old woman was admitted with severe burning pain, hyperesthesia, and weakness in the right lower extremity. The patient had undergone radio- and chemotherapy after surgery for cervical cancer 17 years earlier. We diagnosed radiation-induced lumbosacral plexopathy because of conduction block in the deep peroneal nerve and myokymic discharge in the tibialis anterior muscle. Pelvic computed tomography and magnetic resonance imaging ruled out recurrent tumor and nerve-compressing lesions. Although radiation-induced lumbosacral plexopathy is usually characterized by lower motor neuron syndrome, we report a rare case presenting with severe pain and hyperesthesia.

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A 69-year-old man developed motor aphasia and right hemiparesis with severe headache, during the treatment of cellulitis and sepsis due to cat bites. Brain CT showed a low density, crescent-shaped lesion in the left subdural space, which was hypointense on brain diffusion-weighted imaging (DWI). One week later, when his neurological symptoms had worsened, the signal of the subdural lesion had changed to hyperintense on DWI. The lesion was capsule-shaped when enhanced by Gadolinium. The signal changes on DWI of the lesion indicated the existing hematoma had changed to an empyema, or so-called infected subdural hematoma, due to a hematogenous bacterial infection. Pasteurella multocida, a resident microbe in the oral cavity of cats, could be the responsible pathogen in this case. The patient recovered completely after treatment with intravenous high dose antibiotics. This is an important case report describing the transformation from a chronic subdural hematoma into a subdural empyema by DWI.

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A 77-year-old woman presented with conus medullaris and cauda equina syndrome following a sudden pain in the bilateral lower abdomen and right buttock. Lumbar magnetic resonance imaging (MRI) showed not only a conus medullaris lesion, but also several lesions in the vertebral bodies (L1, L2), right major psoas muscle, right multifidus muscle and bilateral erector spinae muscles. As these areas receive blood supply from each branch of the same segmental artery, we considered all of the lesions as infarctions that were a result of a single parent vessel occlusion. It is known that a vertebral body lesion can be accompanied by a spinal cord infarction, but in combination with infarction of a muscle has not been reported. This is the first report of a concomitant spinal cord and muscle infarction revealed by MRI. It is noteworthy that a spinal cord infarction could expand not only to neighboring vertebral bodies, but also to muscles.

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A 70-year-old woman was admitted to our hospital because of the right limb pain and gait disturbance. ⁶⁷Ga scintigraphy showed an increased uptake in the spinal cord, mediastinal lymph node and right tibialis anterior muscle. Based on the histopathological findings of epithelioid cell granuloma in endobronchial ultrasound-guided transbronchial needle aspiration of lymph node, she was diagnosed as having probable spinal cord/muscular sarcoidosis. After she was treated with oral prednisolone, her limb pain and gait disturbance improved. Furthermore, uptake in ⁶⁷Ga scintigraphy was reduced after the treatment. In conclusion, ⁶⁷Ga scintigraphy is useful not only for diagnosis, but also for estimating the efficiency of the treatment for sarcoidosis involving multiple organs such as the spinal cord and skeletal muscle.

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[in Japanese]

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[in Japanese]

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[in Japanese]

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