We retrospectively studied the clinical features and the outcome of first acute symptomatic seizure in elderly. The subjects were 457 patients, who were more than 15 years old, and whose electroencephalograms were available in our hospital. The subjects were divided into two groups, the elderly (236 patients; age more than 60 years, mean age; 73.2±8.2, 105 female, 131 men), and non-elderly (221 patients; 15≤age≤59, mean age; 35.7±14.1, 87 female, 134 men), and were diagnosed in accordance with the guidelines of ILAE. We ascertained all episodes of acute symptomatic seizure and unprovoked seizure. Date on age, gender, etiology, status epilepticus (SE), 30-day and one-year mortalities, and subsequent episodes of unprovoked seizure were collected. Acute symptomatic seizures are more likely to occur in elderly group, and showed higher short-/and long-term mortalities than unprovoked seizures in both elderly and non-elderly groups. Acute symptomatic seizures due to multiple causes in elderly group showed the highest mortality. The outcome of patient who had SE was poorer within 30 days, but not within one year among 30-day survivors. Considering the fact that first seizures in the elderly are likely to be provoked by acute illnesses, we need to take special care in diagnosing and treating them.

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The Antihypertensive Treatment for Acute Cerebral Hemorrhage (ATACH)-II Trial (ClinicalTrials.gov no. NCT01176565; (UMIN 000006526) is an international, multicenter, randomized, concurrently-controlled, parallel arm, Phase III trial to determine the therapeutic benefit of early intensive systolic blood pressure (SBP) lowering compared with standard SBP lowering for acute hypertension in patients with spontaneous intracerebral hemorrhage (ICH). The Trial is funded by the National Institutes of Health in the United States and led by Dr. Adnan Qureshi at the University of Minnesota. Seventeen Japanese institutions will participate in this Trial. This article describes the latest version of the study design and our endeavors to develop the Japanese research network for stroke clinical research. The ATACH-II Trial plans to randomize a maximum of 1,280 (approximately 400 from Japan) subjects who have supratentorial ICH (hematoma volume <60cc) with Glasgow Coma Scale ≥5 and SBP of >180mmHg. Subjects undergo a follow-up assessment for functional and quality of life assessment at 90 days post-randomization. The primary research hypothesis of the trial is that intensive SBP reduction (to ≤140mmHg) using intravenous nicardipine infusion for 24 hours post-randomization reduces the proportion of death and disability at 90 days by ≥10% (absolute) compared to the standard SBP reduction (to 140-180mmHg range) among subjects with ICH whose treatment is initiated within 4.5 hours of symptom onset. The ATACH-II Trial could be the seminal research project for stroke researchers in Japan to demonstrate themselves as effective contributing members of investigator-initiated international clinical trials.

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This report presents the case of an 83-year-old female with a tumor in the right temporal lobe. She experienced various epileptic visual auras including visual perseveration. Visual perseveration is classified into polyopia and palinopsia. Epileptic visual perseveration is a rare phenomenon, and the mechanism has not been fully explained. MRI revealed a tumor in the right temporal lobe with edema in the occipital white matter. To reveal mechanisms of epileptic polyopia and palinopsia, we recorded EEG and ¹²³I-IMP-SPECT when she experienced epileptic attacks. EEG showed epileptic discharges beginning at the occipital area, which spread to the temporal and parietal areas. During the EEG recording, the main symptom was an unformed hallucination. SPECT showed that blood flow increased in the right medial temporal and parietal lobes and, to a slightly lesser extent, in the right occipito-temporal area when the polyopia and palinopsia frequently appeared. Involvement of the multiple foci may have caused the different kinds of visual symptoms. The medial temporal and parietal areas were likely responsible for polyopia and palinopsia at least for this patient.

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A 25-years-old man experienced fever and diarrhea. Ten days later he noticed difficulty walking (day 1). On admission neurological examination revealed lethargy, dysarthria and weakness of limbs. Oculocephalic response was not be elicited and extensor toe signs were positive. In spite of treatment with aciclovir and methylprednisolone, he continued to show progressive deterioration developing to coma with decorticate posture. Autonomic symptoms (hyperhidrosis, hypersalivation and fever) and groaning were observed. Brain magnetic resonance image and brainstem evoked potential presented no abnormality, but electroencephalographic study showed a spindle pattern indicating spindle coma. Laboratory tests including cerebrospinal fluids showed no specific results. High-dose immunoglobulin was administered from day 6, and his consciousness level improved. External ophthalomoplegia and ataxic gait were observed after he became more alert. Because he had IgG type anti-GQ1b antibodies in the serum, a diagnosis was made of Bickerstaff's brainstem encephalitis (BBE). Six months after discharge he had complete resolution of his symptoms. This is the first report of spindle coma observed in a case of serologically confirmed BBE.

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An 84-year-old Japanese woman with no family history of dementia visited our memory clinic complaining of memory disturbance. Neurological examination revealed no apparent motor abnormalities, focal cerebral signs, parkinsonism, or cerebellar dysfunction. Hasegawa's Dementia Scale-Revised (HDS-R) and Mini mental state examination (MMSE) scores were 24 and 23 points, respectively. MRI revealed left-side-dominant dilatation of the inferior horn of the lateral ventricle. Although egocentric behavior was remarkable, no disturbance of intelligence was apparent at the first examination, and she was diagnosed as having mild cognitive impairment. Her memory disturbance and disorientation gradually worsened. Atrophy of the cerebrum and dilatation of the lateral ventricle advanced gradually on MRI. Two years later, she required care to perform activities of daily living. HDS-R and MMSE scores had dropped to 13 and 18 points, respectively, and conversion to dementia was diagnosed. Ability to perform 3D cube-copying was well preserved. The patient died due to acute myocardial infarction at the age of 87. The clinical diagnosis was Alzheimer disease.
At autopsy, the brain weighed 1,250g, and argyrophilic grains were widely observed in the limbic system, corresponding to Saito's stage III. Neuron loss, gliosis, spongiform change, and tissue rarefaction were recognized in the superficial layer of the parahippocampal gyrus. Ballooned neurons, pretangles, oligodendroglial coiled bodies, and neuropil threads were also observed. Neurofibrillary tangles and senile plaques, mainly consisting of diffuse plaque, were recognized as corresponding to Braak stage III and CERAD stage B, respectively. Neither Lewy nor Pick bodies were observed. Although mild phosphorylated TDP-43 immunoreactivity was observed, it was suspected to be due to secondary degeneration of tau deposition. The patient was diagnosed pathologically as having argyrophilic grain dementia.
The clinical findings of the present patient reveal important observations that help to clinically discriminate between various dementias such as Alzheimer disease and argyrophilic grain dementia.

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A 36-year-old man presented with cognitive impairment and disturbance of short-term memory functions with character change. Cerebrospinal fluid analysis revealed no abnormalities; however, brain MRI revealed high-signal intensity from bilateral hippocampus lesions on fluid attenuated inversion recovery (FLAIR) images and T₂ weighted images. The 18F-fluorodeoxyglucose PET demonstrated high glucose uptake in the bilateral hippocampus lesions. He was diagnosed as limbic encephalitis, and was administered high-dose intravenous methylprednisolone and immune adsorption plasma therapy followed by intravenous immunoglobulin therapy. MRI abnormalities improved after treatment but recent memory disturbance remained. Ma2 antibody, NMDA-receptor antibody, and GluRε2 antibody were positive. Eleven months atter the onset of disease, the tumor was identified in left testicle by ultrasound and removed the tumor. The pathological findings were seminoma. We experienced a case of paraneoplastic limbic encephalitis associated with seminoma with short-term memory disturbance. The occurrence of paraneoplastic limbic encephalitis with antibodies against cell membrane (NMDA-receptor antibody and GluRε2 antibody) and intracellular (Ma2 antibody) is rare even in the literature.

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Delayed posthypoxic leukoencephalopathy (DPL) is a rare and less well known complication of hypoxic brain injury. Although it is well known that anoxic or hypoxic injury produces acute neurologic deficits, DPL typically manifests days to weeks after apparent recovery from an obtunded state, and patients with DPL demonstrate cognitive impairment, high brain dysfunction, parkinsonism, or psychosis. MRI findings of the brain demonstrate deep white matter abnormalities. We report 2 cases of DPL after hypoxia due to benzodiazepine overdose. Both of our patients had normal arylsulfatase A activity. Although DPL is seen in carbon monoxide poisoning, pseudodeficiency of arylsulfatase A activity, or drug overdose with heroin or morphine, there are only some previous studies of DPL caused by an overdose with benzodiazepine. It is unclear whether neurotoxicity from the drug in addition to hypoxia alone is involved, however, it is important to note that overdose of common drugs as sleeping medicine can cause DPL. Since DPL may often be misdiagnosed and be subjected to unnecessary treatments, it is also important to understand its unique clinical course and MRI findings. With prompt recognition of DPL, we expect that more cases of DPL caused by overdose with benzodiazepine will be diagnosed, because benzodiazepine overdoses are common.

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A 21-year-old man complained of severe pain and muscle twitching localized in his right arm. Neurological examination showed muscle fasciculations in his right forearm but no myokymia or myotonia. Needle electromyography revealed fibrillation potentials in his biceps brachii muscle and extensor carpi radialis muscle at rest but no myokymic discharges. His serum anti-voltage-gated potassium channel (VGKC)-complex antibody level was significantly high (194.2pM; controls <100pM). Although anticonvulsant therapy relieved his pain, he was readmitted to our hospital because of severe pain in his left arm and both thighs three months later. A high-dose intravenous immunoglobulin (IVIG) therapy followed by steroid pulse therapy relieved his pain. This case with neither muscle cramp nor myokymia expands the phenotype of anti VGKC-complex antibody associated disorder.

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A 68-year-old man was referred to our hospital with tension-type headaches and a 1-year history of dementia. On neurologic examination, he had ideomotor apraxia and incomplete Gerstmann syndrome that was characterized by acalculia, agraphia, and finger agnosia. On imaging, multiple cystic lesions reported as "unusually dilated perivascular spaces" were observed along the medullary arteries in the left hemisphere; some of them had adjacent hyperintense areas in fluid attenuated inversion recovery images. We assumed that the multiple cystic lesions caused his higher cerebral dysfunction, because ideomotor apraxia and Gerstmann syndrome are usually indicative of a left parietal lobe lesion. MR spectroscopy in the lesion site revealed increased lactate. On MR angiography, the left middle cerebral artery and the left posterior cerebral artery were poorly visualized without localized stenosis. Technetium-99 bicisate single-photon emission computed tomography showed severely decreased cerebral blood flow in the left hemisphere. Electroencephalography showed slow waves in the left hemisphere.

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We report a case of complete atrioventricular (AV) block in a 40-year-old patient with Duchenne muscular dystrophy (DMD). While he was bed-ridden and required mechanical ventilation, his cardiac involvement was mild. He had the deletion of exon 45-52 in the dystrophin gene. He underwent transient complete AV block and came to require pacemaker implantation due to recurrence of complete AV block ten days after the first attack. Electrophysiological study revealed mild prolonged AH and HV interval. Although DMD patients with AV block have been rarely reported so far, attention should be paid to AV block for patients who prolonged their lives.

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[in Japanese]

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[in Japanese]

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