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We herein report the distribution of gray matter lesions on magnetic resonance imaging (MRI) in two patients with lymphomatosis cerebri (LC). In our patients, the fluid-attenuated inversion recovery sequence of brain MRI demonstrated a bilateral and diffuse high signal intensity, not only in the white matter but also in the thalamus, globus pallidus, putamen, and hippocampus. Among the deep gray matter, the caudate head and putamen (striatum) were relatively spared when compared with the globus pallidus, thalamus, and hippocampus. Interestingly, we found seven previous reports of similar MRI findings, with relative sparing of the striatum, in patients with LC. This finding may be characteristic of LC and help facilitate its diagnosis. Further investigations of a larger number of LC patients are necessary to confirm these findings.

Introduction: Brachiocephalic artery stenosis rarely causes right hemispheric infarction with associated left hemiparesis. To date, there have been no reported cases of stroke associated with brachiocephalic artery stenosis that were successfully treated with recombinant tissue-type plasminogen activator (rt-PA), alteplase. Case Report: An 80-year-old woman presented with left hemiparesis. Brain computed tomography showed no hemorrhage, and computed tomography angiography demonstrated brachiocephalic artery stenosis. Alteplase was administered based on a diagnosis of ischemic stroke. Brain magnetic resonance imaging showed multiple acute infarctions. Thereafter, the blood pressure of the right arm was found to be lower than that of the left arm. The patient’s neurological deficits gradually improved; she was eventually able to walk again and was thus discharged home. Conclusion: While the combination of left hemiparesis and a decrease in blood pressure in the right arm are well known in patients with stroke associated with Stanford type A aortic dissections, it may also occur in patients with stroke due to brachiocephalic artery stenosis. Unlike stroke associated with Stanford type A aortic dissections, stroke due to brachiocephalic artery stenosis may be treated with alteplase.

Background: Although acute pancreatitis is listed among the exclusion criteria for the administration of recombinant tissue plasminogen activator according to the Japanese Guideline for the Management of Stroke, the co-occurrence of acute pancreatitis and acute ischemic stroke has not been investigated. The present study aimed to assess the incidence rate of acute pancreatitis in patients with acute ischemic stroke. Methods: This study consecutively enrolled all patients with ischemic stroke admitted to the Department of Neurology, JA Toride Medical Center between April 2014 and March 2016. Diagnosis of acute pancreatitis was made according to the revised Atlanta Classification of Acute Pancreatitis. We retrospectively analyzed serum amylase activity and the frequency of acute pancreatitis as a comorbidity of ischemic stroke. Results: A total of 411 ischemic stroke patients were included. Serum amylase activity was measured for 364 patients, 27 of whom presented with amylase activity exceeding the upper limit of normal. In two patients with serum amylase activity greater than three times-fold the upper limit of normal, computed tomography or transabdominal ultrasonography showed no characteristic findings of acute pancreatitis. No patient in the cohort met the diagnostic criteria for acute pancreatitis. Conclusions: Acute pancreatitis is a very rare comorbidity of acute ischemic stroke.

Based on previous reports, we propose a practical guide to choose dabigatran 150 mg twice daily or apixaban 5 mg twice daily for patients with atrial fibrillation. We recommend the use of dabigatran 150 mg twice daily for patients with atrial fibrillation who have a high risk of embolism (e.g., ischemic stroke on other oral anticoagulants, presence of left atrial appendage thrombus) and a low risk of bleeding. However, the prevalence of such patients with atrial fibrillation is considered low because patients with atrial fibrillation with a high risk of embolism usually have a high risk of bleeding. In most other patients with atrial fibrillation, the use of apixaban 5 mg twice daily should be considered.

A 61-year-old woman presented with a 1-month history of decreased activities of daily living. Magnetic resonance imaging revealed abnormal intensities of the bilateral frontal lobes and enhancement of the thickened dura matter. A biopsy of the dura mater revealed multinucleated giant cells. She had sinusitis and hematuria; she was diagnosed with granulomatosis with polyangiitis. Hypertrophic pachymeningitis (HPM) was considered to have interrupted the venous flow and caused vasogenic edema. Bilateral frontal lobe edema resulting from HPM due to granulomatosis with polyangiitis has not been reported. A biopsy and examination for other organ complications were useful for the diagnosis and treatment of our patient.

Because fungi usually spread from the paranasal sinuses to the orbital apex in invasive fungal sinusitis (IFS), IFS often presents as an orbital apex syndrome (OAS) characterized by dysfunction of cranial nerves II, III, IV, V1, and VI. We report a case of sphenoid sinus mucormycosis that presented as isolated retrobulbar optic neuropathy. A 94‐year‐old woman presented with acute blindness in the right eye. Examination revealed the absence of light perception and pupillary reflex in the right eye. Head MRI showed a mass in the right sphenoid sinus, which was contiguous with the right optic nerve. She underwent endoscopic surgery, and a histopathological diagnosis of mucormycosis was established. Treatment with intravenous liposomal amphotericin B reduced the size of the mass. She has survived for more than 1 year without recurrence. Clinicians should consider that IFS can present as isolated retrobulbar optic neuropathy.

A 58-year-old man consulted our hospital due to a 2-year history of dysarthria and a 1-month history of blepharospasm. In addition to the ataxic dysarthria and blepharospasm, a neurological examination demonstrated slight ataxia of the trunk and lower limbs. Brain MRI demonstrated atrophy of the upper portion of the cerebellar vermis. Gene analysis established a diagnosis of spinocerebellar ataxia type 31 (SCA31). Single photon emission computed tomography (SPECT) with the three-dimensional stereotaxic ROI template (3DSRT) software program demonstrated hyperperfusion in the lenticular nucleus and thalamus. Although the association between SCA31 and blepharospasm in our patient remains unclear, we considered that this combination might be more than coincidental.

Objective: Living wills, written types of advanced directives, are now widespread in western countries, but in Japan, their recognition still remains restricted to a small part of the population. As an initial step to introduction of such patient-oriented medicine, we surveyed present recognition and acceptance patterns concerning living wills in a main regional hospital located in a suburban area of Tokyo. Methods: Without any preceding guidance on living wills, the questionnaire on living wills was distributed to all the staff working at JA Toride Medical Center in September 2013, and their responses were collected for analysis within one month. Results: Questionnaires were distributed to all hospital staff, 843 in total, and 674 responses (80.0% of distributed) were obtained. The term of living will was known by 304 (45.1%) of the respondents, and introduction of living wills to patients was accepted in 373 (55.3%) of the respondents, meanwhile, 286 (42.4%) respondents did not indicate their attitude toward living wills. As to styles of document form, 332 respondents (49.3%) supported selection of wanted or unwanted medical treatments and care from a prepared list, and 102 respondents (15.1%) supported description of living wills in free form. As preferred treatment options that should be provided as a checklist, cardiac massage (chest compression) and a ventilator were selected by more than half of the respondents. Based on their responses, we developed an original type of living wills available to patients visiting the hospital. Conclusions: Although not all the respondents were aware of living wills even in this main regional hospital, introduction of living wills to patients was accepted by many of the hospital staff. Awareness programs or information campaigns are needed to introduce living wills to support patient-centered medicine.

A 74-year-old right-handed woman without cognitive impairment suddenly developed nonfluent aphasia. Brain MRI showed acute infarction in the right frontal lobe and insula without involvement of the corpus callosum. A neurological examination demonstrated not only transcortical motor aphasia, but also ideomotor apraxia and right hand predominant constructional apraxia (CA). To date, right hand predominant CA has only been reported in patients with corpus callosum lesions. The right hand predominant CA observed in our patient may be associated with the failure to transfer information on the spatial structure from the right hemisphere to the motor cortex of the left hemisphere.

Progressive supranuclear palsy-Richardson's syndrome (PSP-RS) is a neurodegenerative disease characterized by postural instability and vertical gaze palsy, but the clinical diagnosis of PSP-RS is often difficult in the early stage of the disease. A 64-year-old male experienced frequent falls, followed by dysarthria and dysphagia. Neurological examination at age 64 demonstrated vertical gaze palsy, dysarthria, dysphagia, and retropulsion. At that time, while brain MRI demonstrated no apparent abnormalities, SPECT showed the reduction of the cerebral blood flow in the thalamus as well as the medial frontal lobe cortices. The patient was diagnosed with probable PSP-RS, and died at age 70. On postmortem examination, there were abundant tuft-shaped astrocytes, neurofibrillary tangles, coiled bodies, and argyrophilic threads in the brain, establishing the diagnosis of PSP-RS. Our definite PSP-RS case suggests that thalamic hypoperfusion may provide helpful evidence to support a diagnosis of PSP-RS in the early stage of the disease.

A 61-year-old Japanese woman presented with a headache and appetite loss lasting for nine days and was admitted to our hospital, where she was diagnosed with pneumococcal meningitis associated with acute sphenoid sinusitis. While the administration of meropenem and dexamethasone ameliorated the meningitis, right third and sixth nerve palsy suddenly developed 10 days after admission. CT angiography subsequently demonstrated an aneurysm in the cavernous portion of the right internal carotid artery. This is the first reported case of sphenoid sinusitis simultaneously complicated by both pneumococcal meningitis and an infectious aneurysm in the intracavernous carotid artery.

Although progressive multifocal leukoencephalopathy (PML) cases showing responses to mefloquine therapy have been reported, the efficacy of mefloquine for PML remains unclear. We report on the failure of mefloquine therapy in two Japanese patients with PML unrelated to human immunodeficiency virus. One of the patients was a 47-year-old male who had been treated with chemotherapy for Waldenstrom macroglobulinemia, and the other was an 81-year-old male with idiopathic CD4 + lymphocytopenia. Diagnosis of PML was established based on MRI findings and increased JC virus DNA in the cerebrospinal fluid in both patients. Mefloquine was initiated about 5months and 2months after the onset of PML, respectively. During mefloquine therapy, clinical and radiological progression was observed, and JC virus DNA in the cerebrospinal fluid was increased in both patients. Both patients died about 4months and 2months after initiation of mefloquine, respectively. Further studies are necessary to clarify the differences between mefloquine responders and non-responders in PML.

High fever, severe neck pain and neck stiffness can result from meningitis. We report a case of pneumococcal pyomyositis of the neck muscles. A 72-year-old man developed high fever and severe neck pain. His chief complaint mimicked bacterial meningitis. Although his condition was initially suspected to be bacterial meningitis, his cerebrospinal fluid did not show pleocytosis. MRI showed areas of high intensity in the posterior deep neck muscles on short tau inversion recovery (STIR) sequences and gadolinium-enhanced T1-weighted images. Pneumococcal myositis should therefore be included in the differential diagnosis of severe neck pain with fever.

A 79-year-old man presented with a slowly progressive gait disturbance. Brain MRI demonstrated ventriculomegaly and the hummingbird sign. A lumbar puncture showed no abnormalities of the cerebrospinal fluid. The improvement of the gait disturbance after the ventriculoperitoneal shunt led to a diagnosis of idiopathic normal pressure hydrocephalus. Interestingly, postoperative brain MRI demonstrated the disappearance of not only ventriculomegaly, but also the hummingbird sign. The disappearance of the hummingbird sign suggests that an increase in the cerebrospinal fluid in the lateral and third ventricles could cause the compression of the superior surface of the midbrain tegmentum, which manifests as the hummingbird sign.

Background: Nasu–Hakola disease (NHD) is a rare autosomal recessive disorder, characterized by a combination of progressive presenile dementia and formation of multifocal bone cysts, caused by genetic mutations of DAP12 and TREM2, which constitute a receptor/adapter signaling complex expressed on osteoclasts, dendritic cells, macrophages, and microglia. No Japanese patients with TREM2 mutations have been reported previously. Methods: We reported three siblings affected with NHD in a Japanese family. Amongst them, two died of NHD during the fourth decade of life. The analysis of genomic DNA, cDNA cloning, and western blot of lymphocyte proteins was performed on samples of the living patient. The transcriptome was studied in the autopsied brain of one patient. Results: We identified a homozygous conversion of a single nucleotide T to C at the second position of intron 3 in the splice-donor consensus site (c.482+2T>C) of the TREM2 gene, resulting in exon 3 skipping and aberrant expression of truncated proteins. We identified 136 upregulated genes involved in inflammatory response and immune cell trafficking and 188 downregulated genes including a battery of GABA receptor subunits and synaptic proteins in the patient’s brain. Conclusions: This is the first report of a Japanese NHD family caused by a splicing mutation of TREM2 that induces both neuroinflammation and neurodegeneration.

〔目的〕経口摂取に際し嚥下リハビリテーションなどを含めた専門的な対応が必要と思われる症例を判別する摂食・嚥下フローチャートを作成する。〔対象〕嚥下造影 (videofluorography: 以下VF) を施行した嚥下障害患者28例 (平均年齢78.7±11.3歳)。〔方法〕反復唾液テスト，改訂水飲みテスト，食物テストの代表的テストを評価し，VF所見，藤島の摂食・嚥下能力のグレードとの関連を調査した。〔結果〕反復唾液テスト (感度0.83，特異度0.22)，改訂水飲みテスト (感度0.56，特異度0.72)，食物テスト (感度0.33，特異度0.75)，嚥下造影，藤島の摂食・嚥下能力のグレードを検討した結果，改訂水飲みテスト，食物テストを採用した。しかし改訂水飲みテスト，食物テストがカットオフ値以上であった4例は誤嚥していた。この4例はいずれも脳卒中後遺症のある症例であったことも考慮して，フローチャートを作成した。

脳卒中や骨折の病態が落ち着いたとしても障害を伴った高齢の患者は，転院という環境の変化によって日常生活活動能力が低下する可能性がある。代表的なADL評価であり，信頼性が実証されているBarthel Indexを用いて，環境の変化の影響を評価した。対象は脳卒中と大腿骨頸部骨折を伴う患者で，急性期病院の転院時に療法士が評価した得点と，転院後1週間以内に連携病院にて看護師が評価した得点を検討した。脳卒中の総合得点は58%が低く評価され，差の平均は-5.9±16.0点であった。大腿骨頸部骨折の総合得点は68%が低く評価され，差の平均は-7.3±14.1点であった。脳卒中の順位相関係数は0.91 (p

We report on three elderly patients with stroke-like onset of atypical Miller Fisher syndrome (MFS). The serum titer of anti-GQ1b IgG was markedly elevated in these patients. Their prognoses were sufficiently good with immunoadsorption therapy with or without intravenous immune globulin (IVIg) therapy. However, some neurological findings were not characteristic of typical MFS. Patient 1 suffered from prolonged dysesthesia in her left extremities, and Patients 2 and 3 showed no ataxia. Moreover, complete bilateral gaze limitation is rare in MFS. The sudden stroke-like onset along with the gaze limitation of these patients suggests that the unexpected elevation in the serum titer of anti-GQ1b IgG due to unknown immune dysregulation might have severely affected the third, fourth, and sixth nerves and this potent antibody rapidly attacked these nerves and induced stroke-like clinical features and complete ophthalmoplegia.

We report on a right-handed 43-year-old policeman with atypical Marchiafava-Bignami disease (MBD). The typical clinical manifestations of MBD are reduced consciousness, confusion, seizures, psychotic and emotional symptoms, hemiparesis, dysarthria, ataxia, and coma and death. However, our patient had not developed any of the above symptoms except for slowly progressive cognitive impairment mimicking that of Alzheimer disease. The incidence of MBD may be higher and its prognosis less severe than generally believed. MBD has been underdiagnosed and underreported, and nonspecific general symptoms and encephalopathy in an alcoholic might indicate undiagnosed MBD.

BACKGROUND AND PURPOSE: Predicting response to rtPA is essential in the era of endovascular therapy for stroke. The purpose of this study was to elucidate prognostic factors of early neurologic improvement and long-term outcome with respect to the development and reversion of leptomeningeal collaterals in recanalization therapy after acute ischemic stroke. MATERIALS AND METHODS: We analyzed consecutive patients with proximal MCA occlusion treated with rtPA from 2007 to 2012 at 2 hospital stroke centers. All patients routinely underwent brain MR imaging before rtPA. To assess the reversion of collateral signs, we included patients who underwent follow-up MR imaging. We assessed the development and reversion of collaterals by using a combination of 2 MR imaging collateral markers, the hyperintense vessel sign and the posterior cerebral artery laterality sign. Early neurologic improvement was defined as a decrease in the NIHSS score of ≥10 or a score of ≤2 at 24 hours of treatment. RESULTS: Early neurologic improvement was observed in 22 of 48 eligible patients. The development of collaterals at arrival (15/22 versus 9/26, P = .042) was significantly associated with early neurologic improvement. Multivariate analysis adjusting for other variables showed that the development of collaterals at arrival (OR, 4.82; 95% CI, 1.34–19.98; P = .015) was independently associated with early neurologic improvement. Reversion of collaterals was significantly associated with successful recanalization (P < .001), and multivariate analysis showed that the reversion of collaterals was an independent prognostic factor of long-term functional outcome (OR, 5.07; 95% CI, 1.38–22.09; P = .013). CONCLUSIONS: Our results indicate that the development of leptomeningeal collaterals plays a crucial role in achieving early neurologic improvement, and reversion of collaterals predicts a favorable outcome via arterial recanalization after rtPA treatment for acute stroke.

介護保険制度創設のねらいとして「介護の社会化」が掲げられているが，重度要介護者の在宅介護においては，今なお，家族介護者の果たす役割は大きく，家族介護者の献身的な役割意識に支えられていると考える。今回私達は，在宅家族介護の実態と，介護負担感に関連するQuality of Life(QOL)要因を明らかにするため，当居宅支援事業所利用者の主介護者に対し，調査を行なった。これまで，介護負担感に関する研究は多くあるが，介護負担感に関連するQOL要因をQOLの領域別に分析した報告はなかった。調査項目は，主介護者の基本情報，要介護者の基本情報，WHO/QOL-26調査票，介護負担感調査票とした。調査の結果，介護期間6か月未満と5年以上の介護者が負担感を強く感じていた，また，要介護者の認知症が重度になると介護負担感が強くなる傾向であった。介護負担感に関連するQOL要因は，痛みや不快，睡眠と休養，活力と疲労感などの身体的QOL要因であることが明らかとなった。また，介護に関する新しい情報や技術の習得，住宅環境などの環境的QOL要因は，身体的QOLを高める関連要因であることが明らかになった。心理・社会的QOL要因は，介護負担感と有意な関連性がみられなかったが，これは，調査対象者の居住地域が，農村部を含む地方都市であり，主介護者に対する親族の支援が比較的得られていたこと，調査対象者の多くが高齢の女性であり，介護に対する役割意識・義務感とこれらに関連する地域の特性が心理・社会的QOL要因に影響したことによると推測する。以上の結果は，在宅家族介護者を支援するために有用な示唆であると考える。

Background: Diffusion-weighted magnetic resonance (MR) imaging (DWI) is an excellent examination for detecting acute ischemic stroke, but false-negative cases have been reported recently.Patients and Methods: Since the present MR scanner (1.5-T, Siemens Symphony) was introduced to our hospital, a prospective study was designed in the Departments of Neurology and Radiology to evaluate the DWI findings in patients tentatively diagnosed to have an acute infarction and in those with stroke-like episodes. During the 31 months between June 2000 and December 2002, 572 consecutive patients with acute cerebral infarction or presenting conditions mimicking ischemic stroke, including transient ischemic attack (TIA), sudden-onset isolated vertigo, and loss of consciousness (LOC) with or without seizure, underwent DWI.Results: Four of 366 patients with a cerebral infarction (1.1%) had false-negative DWI in the acute stage, and 10 of 206 patients with conditions mimicking ischemic stroke (4.9%) had false-positive DWI in the acute stage. Of these 10 patients, there were five cases with TIA, four with sudden-onset isolated vertigo, and 1 with LOC with seizure. Sensitivity and specificity values were 98.9% and 97.6%, respectively, when DWIs were performed to diagnose acute cerebral infarction.Conclusion: DWI rarely fails to detect an acute-stage cerebral infarction, but further confirmatory measures may be necessary when there is a negative examination using a clinical or computed tomographic diagnosis to the contrary.

Background The Japanese have become the longest-lived nation population in the world, and numbers of elderly who require medical and nursing care are increasing. The capacity of nursing homes and nursing institutions is sharply limited in Japan; further, as a group, elderly Japanese patients prefer home care to institutional care. For these reasons, the home health care system in Japan has been increasingly important. Objective We sought to identify factors determining long-term survival in Japanese patients receiving home health care for neurologic disorders. Patients and methods We retrospectively evaluated 180 patients with neurologic disease, who received home health care conducted by our hospital between 1992 and 2001. Factors considered were age; gender; illnesses; prognosis; follow-up period; activities of daily living (ADL); behavioral, cognitive, and communicative functions; swallowing function; feeding method; serum nutritional values (total protein, albumin, and total cholesterol); hemoglobin concentration; and social care services provided at home. Results Variables affecting long-term survival in 180 patients with neurologic disease were age (P Conclusion Maintenance of swallowing function and adequate nutrition through a variety of feeding methods that can be provided by a home health care program are important for long-term survival of patients with stroke and also that of patients with other neurologic diseases.

This article represents the update of ‘European Stroke Initiative Recommendations for Stroke Management’, first published in this Journal in 2000. The recommendations are endorsed by the 3 European societies which are represented in the European Stroke Initiative: the European Stroke Council, the European Neurological Society and the European Federation of Neurological Societies.

A 42-year-old man presented with fever, headache and liver dysfunction, and was diagnosed as having aseptic meningitis by lumbar puncture. The PCR detected Epstein-Barr virus (EBV)-DNA in the peripheral blood and cerebrospinal fluid. About 20 days after onset, the patient presented with hearing impairment in the right ear, which was confirmed by a pure tone audiogram. The hearing acuity improved after the initiation of hydrocortisone sodium phosphate. We presume that the hearing impairment was due to auditory nerve neuritis related to extension of inflammation of the meninges. This is the first reported case of EBV-associated meningitis showing hearing impairment.

Thirteen elderly patients with polymyalgia rheumatica (PMR) are presented. The clinical and laboratory findings suggest that many progressive symptoms are due to the non-specific inflammatory changes in various organs of the body, especially in muscles and joints. An immunofluorescence study of muscle biopsy specimens revealed IgG, IgA, and fibrinogen deposits in the perifascicular area of the perimysium. This finding suggests that immune complexes play a role in the pathogenesis of this condition and that the pathophysiology of PMR is an interstitial inflammatory process. We think that the inflammatory findings affecting the interstitial tissue of muscles in the immunofluorescence study are relatively specific to PMR, and will be affected by steroid treatment.

A previously healthy 50-year-old man developed aseptic meningoencephalitis with clinical manifestations including fever, headache, seizure, Wernicke aphasia, right hemiplegia, and blindness in the left eye. One and one-half months after remission of meningoencephalitis, marked ataxia and psychiatric symptoms became apparent. Magnetic resonance imaging revealed multiple new lesions involving the basal ganglia, thalamus, white matter, and cerebellum. Despite these developments, cerebrospinal fluid findings continued to improve except for excessive content of myelin basic protein. Within 2 weeks, steroid therapy dramatically resolved the ataxic symptoms and disseminated lesions.

Objective: Loss of consciousness (LOC) is caused by a variety of conditions including epileptic, cardiac, psychiatric, and autonomic disorders. We investigated the prevalence of presenting attacks of genuine epilepsy among patients of Emergency Department and Department of Cardiology evaluated for an episode of LOC with or without a convulsion. Patients and methods: We retrospectively studied 371 adults presenting to the Emergency Department and Department of Cardiology of our hospital from 1991 to 1999 with a chief complaint of an episode of LOC with or without a convulsion. Ages ranged from 15 to 78 years. Patients were free of severe chronic illnesses, drug abuse, and alcoholism. LOC was considered to represent genuine epilepsy either when the interictal electroencephalogram (EEG) showed epileptiform discharges in the absence of imaging abnormalities, or when both the EEG and imaging studies were unrevealing but one or more previous attacks had occurred and administration of an anticonvulsant prevented subsequent attacks. Results: Patients included 302 patients without a convulsion, and 69 patients with a convulsion. Of the former, 14 subjects had epileptiform discharges on EEG, and three subjects had no epileptiform discharges but had three or four attacks of LOC that were abolished by anticonvulsant therapy. Of the 69 patients with a convulsion, seven had epileptiform discharges, and 12 had two to five attacks, no epileptiform discharges, and a response to anticonvulsant therapy. Conclusions: The prevalence of presenting attacks of genuine epilepsy in 371 adult patients with an episode of LOC was remarkably high (9.7%: 36 subjects). © 2001 Elsevier Science B.V. All rights reserved.

We report an autopsy case of amyotrophic lateral sclerosis (ALS) clinically diagnosed as spinal progressive muscular atrophy (SPMA). The patient was a Japanese woman without hereditary burden. She developed muscle weakness of the distal part of the left lower extremity at age 42, followed by muscle weakness and atrophy of the right lower extremity and upper extremities. At age 57, she needed transient ventilatory support. Slight weakness in the facial muscles and fasciculation of the tongue appeared at age 60. At age 61, she died of sudden respiratory arrest. During the clinical course, neurological examination revealed neither Babinski signs nor hyperreflexia. The neuropathological examination revealed not only neuronal loss with gliosis in the facial nucleus, hypoglossal nucleus, and anterior horns of the spinal cord, but also loss of Betz cells and degeneration of the pyramidal tracts. Based on these clinicopathological findings and review of literature, we conclude that sporadic ALS mimicking SPMA is present.

for jaw jerk. The Babinski refl exes were bilaterally positive. He needed a urinary catheter due to urinary retention problems. Routine laboratory examination was normal except for an erythrocyte sedimentation rate of 17 mm in 1 h. Both HIV and HTLV-1 serum antibodies were negative. Tumor markers, such as CEA and CA19-9, were in the normal range. Cerebrospinal fl uid examination showed the increased cell count of 42 cells/ l and elevated protein of 61 mg/dl. The IgG index was at the upper limit of the normal range at 0.7, suggesting that intrathecal synthesis of IgG did not increase signifi cantly. Myelin basic protein was increased at 489 pg/ml. All bacterial, fungal, and viral cultures were negative. Repeated cytology was negative. Genotyping for HLA showed that he had DRB1*1501 and DQB1*0602. MRI of the spine revealed multiple hyperintense lesions throughout C 3–5 , T 2–3 , 9–12 and conus medullaris on T 2 weighted images ( fi g. 1 ). Those lesions mostly exhibited multiple enhancements after intravenous administration of gadolinium. Only a small hyperintense lesion was seen in the brain white matter without gadolinium enhancement ( fi g. 2 ). The patient was intravenously given methylprednisolone, 1,000 mg/day for 3 days, followed by prednisolone orally, 50 mg/day. The dose was tapered off over 4 Dear Sir, Multifocal infl ammatory leukoencephalopathy (MIL), a central nervous system disorder characterized by demyelination with perivascular infl ammation, has recently been reported in several patients treated with the combination of fl uorouracil (FU) and levamisole chemotherapy [1–9] . However, there have been no reports mentioning spinal lesions, whereas demyelinating lesions have been frequently found in other diseases such as multiple sclerosis (MS), acute transverse myelitis, and acute disseminated encephalomyelitis. We report the fi rst case of a patient with acute disseminated encephalomyelitis associated with 5-FU and l -leucovorin treatment and discuss its putative pathogenesis.

Background To examine the effects of percutaneous endoscopic gastrostomy (PEG) on quality of life (QOL) in patients with dementia. Methods We retrospectively included 53 Japanese community and tertiary hospitals to investigate the relationship between the newly developed PEG and consecutive dementia patients with swallowing difficulty between Jan 1st 2006 and Dec 31st 2008. We set improvements in 1) the level of independent living, 2) pneumonia, 3) peroral intake as outcome measures of QOL and explored the factors associated with these improvements. Results Till October 31st 2010, 1,353 patients with Alzheimer’s dementia (33.1%), vascular dementia (61.7%), dementia with Lewy body disease (2.0%), Pick disease (0.6%) and others were followed-up for a median of 847 days (mean 805 ± 542 days). A total of 509 deaths were observed (mortality 59%) in full-followed patients. After multivariate adjustments, improvement in the level of independent living was observed in milder dementia, or those who can live independently with someone, compared with advanced dementia, characterized by those who need care by someone: Odds Ratio (OR), 3.90, 95% confidence interval (95%CI), 1.59 - 9.39, P = 0.003. Similarly, improvement of peroral intake was noticed in milder dementia: OR, 2.69, 95%CI, 1.17 - 6.17, P = 0.02. Such significant associations were not observed in improvement of pneumonia. Conclusions These results suggest that improvement of QOL after PEG insertion may be expected more in milder dementia than in advanced dementia.