Small deep brain infarcts are often caused by two different vascular pathologies: 1. atheromatous occlusion at the orifice of large caliber penetrating arteries termed branch atheromatous disease (BAD) and 2. lipohyallinotic degenerative changes within the course of penetrating arteries termed lipohyalinitic degeneration. Representative vascular territories of BAD type infarcts are lenticulostriate artery (LSA), pontine paremedian artery (PPA) and less frequently anterior choroidal artery. BAD type infarcts are strongly associated with progressive motor deficits (PMD) leading to a worse functional outcome, because they affect pyramidal tract at corona radiata, pontine base or internal capsule. In our study, female sex and initial severity of motor deficits were common predictors for PMD in both groups with LSA and PPA. Single infarcts without concomitant silent lacunar infarcts and preceding lacunar transient ischemic attack (TIA) in the LSA group, and diabetes mellitus in the PPA group were independent predictors for PMD in penetrating artery infarctions. There were different characteristics between the groups of LSA and PPA. Prevalence of male gender, diabetes mellitus and intracranial atherosclerosis were significantly higher in the PPA group than in the LSA group. The combined treatment of cilostazol and edarabone significantly improved functional outcome especially in the PPA infarct group. Adjoining clopidgrel on aforementioned combined treatment further improved functional outcome in the LSA group. The actions of vasodilatation and endothelial protection in cilostazol and inhibition of shear-induced platelet activation in clopidogrel, as well as scavenging free radicals in edaravone might work effectively.

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We investigated disease notification and self-determination in treatment decisions of Creutzfeldt-Jakob disease (CJD). For this purpose, we retrospectively analyzed 18 patients with CJD (sporadic:familial = 14:4) who were admitted to our hospital. The durations from symptom onset to the time of diagnosis ranged from 0.3 to 48 months (median, 2 months). The Hasegawa Dementia Scale-revised (HDS-R) range was 0 to 29 (median, 5.0); however, 4 patients (22%), including 2 with familial CJD and 2 with sporadic CJD, maintained cognitive function (HDS-R score ≥ 21). Two patients (11%), who satisfied Lo’s criteria for self-determination, were given a disease notification, and both of them archived self-determination in treatment decisions. In conclusion, we demonstrated that patients with CJD could be given a disease notification and archive self-determination in treatment decisions upon early diagnosis using DWI.

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A 46-year-old man developed central respiratory failure in the subacute phase of unilateral lateral medullary infarction. He complained of sudden headache and nausea at first. Neurological examination revealed Wallenberg’s syndrome. Acute right lateral medullary infarction caused by the dissecting right vertebral artery was identified by magnetic resonance images. He was transferred to our hospital on the 3rd day after the onset. He was alert and conscious on admission, and became restless gradually later. He was intubated for sudden respiratory failure on the 9th day. Blood gas analysis showed hypercapnia and hypoxia. Central respiratory failure was indicated by the fact that various examinations showed no change of his infarction, no subarachnoid hemorrhage, or no worsening of pneumonia. Ventilatory support was required for a month because of repetitive CO₂ narcosis. He was weaned from the ventilator on the 39th day. Only a few reports are available on central respiratory failure associated with the subacute phase of unilateral medullary infarction. Delayed central respiratory failure may be lethal. Careful observation is required on the subacute phase of Wallenberg’s syndrome.

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A 61-year-old man developed disturbance of consciousness for 2 weeks. He showed neck stiffness and hyporeflexia. Analysis of his cerebrospinal fluid (CSF) revealed pleocytosis and markedly reduced glucose contents. Adenosine deaminase (ADA) levels in the CSF were elevated (28.8 IU/l). Brain magnetic resonance imagings showed enhancement of the leptomeninges. Tuberculous meningitis was considered, but antituberculous drug was not effective. Repeated cytological analysis of the CSF demonstrated atypical cells with enlarged unevenly distributed nuclei and immunoreactive with glial fibrillary acidic protein. We diagnosed him as leptomeningeal gliomatosis. CSF ADA may be elevated in this rare disorder, and here we emphasize that repeated cytological analysis with immunohistochemical staining was useful for diagnosis.

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We herein report the patient of a 69-year-old woman who presented with the chronic myopathic form of sarcoid myopathy. She had experienced slowly progressive limb muscle weakness for three years. She was found to be thin, but otherwise normal, on a physical examination. Neurologically, proximal muscles are predominantly involved without any sensory or other focal deficits. Electromyography revealed myopathic motor unit potentials exhibiting spontaneous discharge. Muscle biopsy demonstrated extensive connective tissue and few residual muscle fibers with a hint of granuloma formation. Repeated sectioning of the muscle biopsy revealed noncaseatious granuloma with a multinucleated giant cell, confirming the diagnosis. The findings of all imaging studies, including a systemic PET (positron emission tomography) scan, were unremarkable. Without careful pathological observation with repeated sectioning, this patient would have been misdiagnosed with limb-girdle muscular dystrophy．

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We report an 85-year-old man presenting with wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome with cerebellar ataxia and facial dysesthesia. He experienced an abrupt onset of double vision and exotropia of the right eye with unsteady gait and dysesthesia around upper lip. He was admitted to our hospital ten days after the onset of the double vision. On admission, he presented with WEBINO, left limb ataxia, and dysesthesia around upper lip on the right side. His exotropia was prominent on the right side. Diffusion weighted images of MRI revealed a high intensity lesion in the paramedian pontine tegmentum involving bilateral medial longitudinal fasciculus (MLF), consistent with acute ischemic lesion. Four months after the onset, the WEBINO persisted, without cerebellar ataxia and facial dysesthesia. Putative lesions of the WEBINO, cerebellar ataxia and facial dysesthesia were bilateral MLF, left superior cerebellar peduncle and trigeminothalamic tract, respectively, which were broader than the MRI lesion. Neurological examination is critical for evaluation of accurate ischemic area.

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We report a 93-year-old woman with dementia who developed generalized convulsion and involuntary movement of her tongue. She could independently walk and eat meals until 8 months ago, however she turned into bedridden. When she was admitted to our emergency room due to status epilepticus, her tongue intermittently moved from the midline to the left. She could not eat or speak during this episodic tongue movement. MR imaging study revealed brain atrophy in the bilateral mesial temporal lobe, consistent with senile dementia of Alzheimer type. Despite her tongue movements seemingly developing to the generalized convulsion, EEG study did not indicate epileptiform discharges corresponding to this movement. Although antiepileptic drug therapy was effective, we needed polytherapy to control this movement. Paroxysmal tongue movements were previously reported in cases of epilepsy, brain tumor, and stroke, observed bilaterally in most cases. This episodic tongue movement would be rare in terms of the clear laterality. The etiology of this movement was presumed as focal seizure, palatal tremor, dyskinesia or others, but was undetermined. Episodic movements involving tongue decrease the quality of daily life especially in the elderly. Therefore, we should pay more attention to it and try to treat it earlier.

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We report an autopsy case of dementia associated with amyotrophic lateral sclerosis (ALS) in a 73-year-old female. She developed memory impairment at the age of 68 years. Atrophy of her hand muscles was noted at the age of 71 years. She was not aware of her memory impairment or muscle weakness, and was loquacious and euphoric. She was clinically diagnosed as having Alzheimer disease (AD) complicated by ALS with dementia/frontotemporal lobar degeneration with motor neuron disease (ALS-D/FTLD-MND). A neuropathological study confirmed the presence of features of sporadic ALS. Furthermore, severe neuronal loss involving the subiculum and the rostral portion of the medial side of the temporal pole cortex was detected, and TAR DNA-binding protein-43-positive-neuronal cytoplasmic inclusions were identified in the granule cells of the dentate gyrus. These findings were compatible with the pathological features of ALS-D/FTLD-MND. Although many pretangles, neuropil threads and senile plaques were revealed in the degenerated areas, there were few neurofibrillary tangles and typical plaques (Braak stage III, C). Further discussion is required to determine whether AD with ALS-D/FTLD-MND is different from typical AD. This case might be helpful for diagnosing similar cases in the future.

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The patient is a 66-year-old man with hereditary telangiectasia. He was diagnosed with pulmonary arteriovenous malformation (PAVM), which was revealed by contrast-enhanced chest computed tomography at the age of 65. He developed headache, right homonymous hemianopsia, and right hemiparesis and was admitted to our hospital. Contrast-enhanced magnetic resonance imaging revealed multiple lesions in the left hemisphere, which indicates brain abscesses. Thus, the diagnosis of brain abscess mediated through PAVM was established. Following management with drainage and coil embolization, all neurological symptoms resolved. Therefore, coil embolization should be considered for PAVM at an early stage to prevent brain abscess, even if it is asymptomatic.

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To investigate the need for pre- and post-graduate education for neurologists, the subcommittee of the Japanese Society of Neurology for education performed a questionnaire-based survey in 80 medical universities throughout Japan. The response rate to the questionnaire was 82.5%. Textbooks for lectures for medical students were used in only 22.7% of those universities. If the Japanese Society of Neurology (JSN) made a standard text, 77.8% of universities would like to use it. Most of the training programs for residents were compatible with the minimum requirements of the JSN. Just 66.7% of those training programs were completed in their own institute, and 77.3% of universities required help from the JSN.

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To evaluate postgraduate neurological education, a questionnaire-based survey regarding junior and senior doctor-in-training and the Board Certification Examination in Neurology was carried out on the training supervisors of 690 insitutes, excluding 80 university hospitals. The institutes included 243 teaching hospitals, 326 semi-teaching hospitals and 121 education-associated institutes authorized by the Japanese Society of Neurology (JSN). The results were obtained from 388 institutes, and the response rate was 56.2%. The percentage of junior doctorsin-training that received training in neurology was 68.6% (the average of 2.1 months during 2 years). More than half of the institutes did not have any teaching programs for junior doctors-in-training who did not train in neurology. In senior doctors-in-training, the number of senior doctors-in-trainings per year per institute was 0.44 and was only able to experience limited types of disorders. Also, many institutes could not achieve training goals by the institutes themselves (56%). The problems were due to lack of teaching staffs and manpower, and there were many requests to the Society regarding training methods. As for the Board Certification Examination in Neurology by the Society, it was revealed that there were small number of candidates per year per institute, and over half of institutes could not sufficiently teach and support them. Most requests to the Society were regarding teaching seminars and hands-on courses, and some institutes asked small group meetings for arts and techniques of neurology to be held the Regional Society. In conclusion, there are problems that cannot be solved by individual institutes alone, and we need procedures for postgraduate training in neurology that is organized by the Regional and JSN working as the central organization.

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To understand the status of postgraduate education in neurology in Japan, the Committee for the Education of Undergraduate Students and Junior Residents within the Japanese Society of Neurology investigated the four-year trend at 80 medical schools from 2009 to 2012. The mean number of new students to each postgraduate school increased from 1.24 to 1.67 during these four years. After training clinical neurology, more than half of the neurological residents entered the postgraduate schools. Students in the postgraduate schools seemed to be researching major neurological diseases using various methods at each neurology laboratory. However, some problems were suggested. First, the mean number of newcomers to the neurology departments of the universities decreased gradually from 2.29/year to 1.96/year. Second, many of the postgraduate students were working in patient services at university hospitals or as part-time workers at other hospitals, and may not have sufficient time for their research projects. Third, many of the postgraduate students were carrying out research at each affiliated department of neurology, and may not have the opportunity to work in laboratories specializing in basic science. Finally, there may not be sufficient opportunities for further research at other laboratories in Japan or overseas after they finished their work at postgraduate school.

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[in Japanese]

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[in Japanese]

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