Epilepsy is a common disease with a high incidence of about one percent. Knowledge of seizure semiology and correct reading of EEG findings are important for diagnosis of epilepsy. Because the primary therapy for epilepsy is antiepileptic drugs (AEDs), including several ones that are newly permitted in Japan, we need to prescribe them based on an understanding of their actions and interaction mechanisms. However, we also need to consider early surgical treatment for temporal lobe epilepsy with hippocampal sclerosis. In the therapeutic decision for adult epilepsy patients many factors such as employment, marriage, child bearing, and co-existent disease need to be considered. The present review provides an overview of the basis of epilepsy practice for neurologists treating adults with epilepsy, including a discussion of new AEDs, epilepsy surgery, women with epilepsy, and epilepsy in the elderly.

View full abstract

Muscle pathology plays a central role in the diagnosis of muscle diseases. Proper handling, fixation and transportation of muscle biopsy specimens are necessary to avoid artifacts. Hematoxylin and eosin provides information on basic morphological abnormalities, including general structural changes, fiber size variation, necrosis and regeneration, endomysial fibrosis and lymphocyte infiltration. Modified Gomori trichrome is useful to detect abnormal structures including protein aggregates. NADH-tetrazolium reductase highlights intermyofibrillar network, thus serving to detect myofibrillar disorganization. Myosin ATPase is used for evaluating fiber types. Selective type 1 fiber atrophy reflects myopathic process while fiber type grouping reinnervating process. The final interpretation whether the biopsy demonstrates myopathic or neuropathic changes is based upon all these findings.

View full abstract

An anonymous postal survey of cardiologists, diabetologists, gynecologists, and ophthalmologists in Osaka was performed to assess the medical care-seeking behaviors of and problems associated with the medical management of patients with myotonic dystrophy (DM). The questionnaires were sent to 927 cardiologists, 357 diabetologists, 882 gynecologists, and 915 ophthalmologists. Of these, 172 cardiologists, 85 diabetologists, 220 gynecologists, and 154 ophthalmologists responded. More than 30% of responders had provided care to DM patients, and approximately 10% had experience diagnosing DM patients. These facts suggest that DM patients receive medical care from various specialists due to complications involving multiple systems and some of them visit other specialists prior to neurologists. Some patients were diagnosed after perinatal or perioperative difficulties. Therefore, it seems important to improve the ability of physicians to identify DM patients. Because specialists with experience diagnosing DM paid more attention to the characteristic features of DM, such as grip myotonia and hatchet face, a simple screening test may be useful for detecting DM. Some responders pointed out the negative attitude of DM patients toward medical care and the lack of neurologists for consultation as problems in the medical management of DM patients. Cooperation among neurologists and other specialists and education of DM patients are important to improve the medical management of DM patients.

View full abstract

We report two patients with encephalitis associated with antibodies against NR1-NR2 heteromers of the NMDA receptor that showed dramatic improvement after immunomodulating therapies. A 38-year old woman (case 1) suddenly developed seizures and short term memory loss. Brain MRI appeared almost normal except for a small number of high intensity spots of white matter on T₂ weighted images. Cerebrospinal fluid examination (CFS) disclosed lymphocytic pleocytosis (61/μl) and Qualitative analysis of NR1-NR2 antibodies in both CFS and serum were positive. Although an initial treatment with high-dose methylprednisolone was not beneficial for clinical improvement, intravenous immunoglobulin (IVIg) therapy led to complete recovery from her neurological problems. Repeated general surveys showed no evidence of tumors including ovarian teratoma.
A 71-year old man (case 2) suddenly developed seizures and short-term memory loss three days after receiving an influenza vaccination. Brain MRI appeared normal. CSF analysis revealed no pleocytosis and a slight elevation of protein value accompanying oligoclonal IgG band. Qualitative analysis of NR1-NR2 antibodies in both CFS and serum were positive. Intravenous high-dose methylprednisolone caused dramatic improvement and his neurological problems immediately disappeared. Repeated general surveys showed no evidence of tumors, as in case 1.
These two cases showed relatively benign clinical courses with no evidence of tumors and were quite different from the well-known encephalitis associated with antibodies against NR1-NR2 heteromers of the NMDA receptor. Our clinical experience in these two cases suggests that the disease spectrum of anti-NMDA-receptor associated encephalitis might be broader than was once considered.

View full abstract

We report a case of tumefactive demyelinating lesion (TDL) diagnosed using ¹H-magnetic resonance spectroscopy (¹H-MRS) and conventional magnetic resonance imaging (MRI). A 7-year-old girl was admitted to our hospital with complaints of sleepiness and clumsiness of the right limbs. Neurological examination showed somnolence, right-sided apraxia, and hemiparesis with enhanced tendon reflexes and Babinski sign. Conventional brain MRI revealed extensive hyperintensity in the subcortical white matter of the left frontal lobe in both T₂ weighted and fluid attenuated inversion recovery images. Gadolinium-enhanced T₁ weighted images showed a tumor-like lesion in this area with interrupted rim enhancement, termed open ring sign, and a periventricular lesion along the inferior horn of the right lateral ventricle and a juxtacortical lesion under the right motor cortex. In ¹H-MRS, both single voxel spectroscopy (SVS) and chemical shift imaging showed elevation of choline and reduction of N-acetylaspartate in the left frontal lobe lesion. Furthermore, SVS with a short echo time revealed elevated peaks for glutamate/glutamine complex in this lesion. These results suggested the demyelinating nature of this tumor-like lesion, in accordance with the concept of TDL. Based on this diagnosis, we treated the patient with three sets of methylprednisolone pulse therapy, which resulted in the reduction of TDL and neurological improvement. A follow-up study using MRI also demonstrated two more lesions in the corona radiata and internal capsule of the left hemisphere, supporting a diagnosis of multiple sclerosis based on the revised McDonald's criteria (2010). We concluded that ¹H-MRS may be beneficial in the differential diagnosis of TDL.

View full abstract

A 49-year-old man presented with fever and pain, redness, swelling, and difficulty in walking. The serum C-reactive protein (CRP), creatin kinase (CK), and endotoxin levels were elevated. A blood culture revealed Edwardsiella tarda(E. tarda). Computed tomography (CT) showed subfascial and subcutaneous low-density areas in the lower legs, suggesting focal abscesses and edema. The patient was likely to have necrotizing fasciitis or cellulitis. He was successfully treated with several antibiotics and discharged after 43 days. Because E. tarda causes sepsis and fulminating necrotizing fasciitis with a high mortality rate in patients with an underlying illness, it should be considered a potentially important pathogen. The lack of an underlying illness may be a factor for a good outcome in this case.

View full abstract

We have reported two patients with posterior spinal artery syndrome. Both of them had sudden onset back pain, paraparesis, loss of deep sensation and bladder-bowel disturbances. MRI disclosed spinal cord lesions positioned at its posterior part including the posterior column or posterior horn at thoracic levels.
Spinal artery syndrome is a rare disorder, especially the posterior spinal artery syndrome (PSAS). In our department, only ten patients had spinal artery syndrome out of 2,064 patients admitted to our hospital these 20 years. All the other 8 patients had anterior spinal artery syndrome. It supports the notion that PSAS is rare. The detection rate of PSAS may increase after the routine use of spinal MRI in clinical practice.
Our two patients had bilateral, symmetric symptoms. These symmetric signs and symptoms are usually seen in PSAS. The bilateral posterior spinal arteries connect with each other through many complex anastomoses. Moderate blood flow insufficiency may produce no clinical symptoms because of compensation by these anastomoses. When symptoms appear, these anastomoses do not compensate blood flow deficit and may produce bilateral symptoms.

View full abstract

A 36-year-old woman with neuromyelitis optica had been treated with steroids for the prevention of relapse. However, her treatment was not effective and she showed adverse effects such as diabetes mellitus, osteoporosis, compression fractures, and Pneumocystis carinii pneumonia. Therefore, we started her on mitoxantrone treatment. After five courses of mitoxantrone injection, she developed a herpes zoster infection in her thigh followed by aseptic meningitis. PCR for varicella zoster virus (VZV)-DNA was positive in the cerebrospinal fluid. The mechanisms that caused VZV reactivation by mitoxantrone are not known. Opportunistic herpes virus reactivation may occur easily with increasing use of immunosuppressive drugs for both neuromyelitis optica and multiple sclerosis. These drugs must be used under careful supervision.

View full abstract

A 66-year old hypertensive man having a prostate cancer was admitted to our hospital with sudden onset right hemiparesis. On admission, he showed left hemiplegia, hypesthesia, right limb ataxia, and dysarthria. The NIHSS score was 16. Diffusion weighted magnetic resonance imaging showed an acute infarct in the middle pons and magnetic resonance angiography (MRA) revealed basilar artery (BA) occlusion. Carotid Doppler ultrasonography showed distal occlusion pattern of the bilateral vertebral artery. He was treated with intravenous rt-PA at 116 minutes after symptom onset. One hour later, his symptom was not improved and BA was still occluded on follow-up MRA. Therefore, we performed mechanical thrombectomy with Merci^® Retrieval System. At 323 minutes after onset, BA was successfully recanalized and NIHSS score decreased to 4 without hemorrhagic complication. Medication of oral warfarin was started on day 19 because paroxysmal atrial fibrillation was detected by electrocardiogram. The retrieved thrombus was pathologically diagnosed as a organizing mixed thrombus probable cardiac origin. On day 27, he was discharged home without any neurological deficit. Additional thrombectomy with Merci^® Retrieval System is a promising treatment strategy for BA occlusion which is resistant to intravenous rt-PA thrombolysis.

View full abstract