Showing papers in "Nō to shinkei Brain and nerve in 2001"

[Study of functional rating scale for amyotrophic lateral sclerosis: revised ALSFRS(ALSFRS-R) Japanese version].

Abstract: Amyotrophic lateral sclerosis(ALS) is progressive, degenerative, fatal disease of the motor neuron. No efficacious therapy is available to slow the progressive loss of function, but several new approaches including neurotrophic factors, antioxidants and glutamate antagonists, are currently being evaluated as potential therapies. Mortality, and/or time to tracheostomy, muscle strength and pulmonary function are used as primary endpoints in clinical trials for treatment of ALS. The effect of new therapies on the quality of patients' lives are also important, so we sought to develop a rating scale to measure it. The revised ALS Functional Rating Scale(ALSFRS-R), which has addition of items to ALSFRS to enhance the ability to assess respiratory symptoms, is an assessment determining the degree of impairment in ALS patients' abilities to function independently in activities of daily living. It consists of 12 items to evaluate bulbar function, motor function and respiratory function and each item is scored from 0(unable) to 4(normal). We translated the English score into Japanese one with minor modification considering the inter cultural difference. And we examined reliability of the translated scale. As a measure of reliability, the intraclass correlation coefficient(ICC) was evaluated for total score and the Kappa coefficient proposed by Cohen and Kraemer was calculated for each item. Moreover, we examined sensitivity to clinical change over time and carried out the factor analysis to analyze the factorial structure. The subjects were 27 ALS patients and each was scored twice for reliability or three times for sensitivity by 2 to 5 neurologists and if possible, nurses. The ICC for total score was 0.97(95% C. I.; 0.94-0.98). Extension of the Kappa coefficients were 0.48 to 1.00 for inter-rater reliability and the averaged Kappa coefficients were 0.63 to 1.00 for intra rater reliability, respectively. Concerning the factorial structure, the contribution of the first factor(the first principal component) were 53.5% principal factor solution. The factor loadings of items were 0.52-0.91 except "salivation" and this factor almost equal to the simple sum of all items was interpreted as the general degree of deterioration. The promax votation revealed the riginally supposed factor structure with 3 factors(groups of items): neuromuscuclar function, respiratory function and bulbar function. The rating scale correlated with Global clinical impression of change(GCIC) scored by neurologists and declined with time, indicating its sensitivity to change. On the bases of these results, ALSFRS-R(Japanese version) is considered to be highly reliable enough for clinical use.

[A case of Hirayama's disease successfully treated by anterior cervical decompression and fusion].

Abstract: The authors report the case of a 16-year-old boy with Hirayama's disease(juvenile muscular atrophy of unilateral upper extremity). The present history began about 6 months previously, when he noticed slowly progressive weakness with atrophy of the left hand and forearm. Neurological examination on admission revealed diffuse distribution of muscular atrophy including the left hypothenar, thenar, forearm, and triceps muscles. However, EMG studies identified neurogenic changes in both upper extremities. There was no long tract sign of objective sensory impairment. Plain spinal radiograms showed abnormal kyphosis of the cervical vertebrae. Cervical MR images in the neutral position demonstrated focal atrophy of the cervical cord at the C 5-6 vertebral levels. When the neck was flexed, the cervical cord was displaced anteriorly and was compressed over the posterior surface of the C 5-6 vertebral bodies. He was diagnosed to have Hirayama's disease(cervical flexion myelopathy). Via an anterior approach, he underwent a C 5 vertebrectomy followed by fixation of C 4-6 vertebral bodies using iliac bone and plate system. He recovered from surgery without any complications and has been well for the past 6 months with remarkable improvement of muscle strength. Application of cervical collar for 3 to 4 years has been generally advocated for the treatment of Hirayama's disease because progression of signs and symptoms is usually expected to cease within several years. However, some patients were reported not to response to conservative treatment for more than 5 years after their onsets. To these patients surgery seems to be beneficial, because it can give rise to permanent stable fixation with much shorter period of external cervical immobilization compared with cervical collar therapy, in which long-term application is frequently unbearable in many patients. In conclusion, the present case emphasizes the importance of surgical treatment in Hirayama's disease not only to improve neurological deficits but regain better quality of life.

[Diffusion-weighted magnetic resonance imaging (MRI) in acute brain stem infarction].

Abstract: Diffusion-weighted magnetic resonance imaging (DWI) provides one of the earliest demonstrations of ischemic lesions. However, some lesions may be missed in the acute stage due to technical limitation of DWI. We therefore conducted the study to clarify the sensitivity of DWI to acute brain stem infarctions. Twenty-eight patients with the final diagnosis of brain stem infarction(midbrain 2, pons 9, medulla oblongata 17) who had been examined by DWI within 24 hours of onset were retrospectively analyzed for how sensitively the initial DWI demonstrated the final ischemic lesion. Only obvious(distinguishable with DWI alone without referring clinical symptoms and other informations) hyperintensity on DWI was regarded to show an ischemic lesion. Sixteen(57.1%) out of 28 patients had brain stem infarctions demonstrated by initial DWI. In the remaining 12 cases, no obvious ischemic lesion was evident on initial DWI. Subsequent MRI studies obtained 127 hours, on average after the onset showed infarction in the medulla oblongata in 11 cases and in the pons in one case. Negative findings of DWI in the acute stage does not exclude possibility of the brain stem infarction, in particulary medulla oblongata infarction.

A case of anaplastic astrocytoma arising 8 years after initial treatment by partial resection and irradiation for central neurocytoma

Abstract: A 31-year-old female developed the anaplastic astrocytoma in the right basal ganglia and temporal lobe 8 years after partial resection and irradiation of the central neurocytoma. Probably the malignant astrocytoma was an irradiation-induced tumor. Postoperative radiation therapy for the central neurocytoma appears to have a good effect on tumor local control. But it should be carefully decided whether to use radiation therapy for residual tumor because of the risk of delayed complications of irradiation, such as the present case.

A case of urinary retention secondary to aseptic meningitis

Abstract: A case of a 37-year-old man with urinary retention secondary to aseptic meningitis is described. He was admitted to our hospital with complaints of fever, nausea, headache, and gait disturbance. He had begun treatment 1 week previously for high fever, headache and joint pain at an outpatient department. On admission, neurological examination revealed neck stiffness, spasticity and hyperreflexia of the extremities, hypesthesia of the lower extremities, and ataxic gait. A spinal fluid examination revealed aseptic meningitis. Disturbed consciousness, diplopia, aphasia, tetraparesis, and urinary retention appeared after admission. Computed tomography and magnetic resonance imaging (MRI) of the head revealed no abnormal lesions. On the 2nd day of hospitalization, a Foley catheter was inserted for urinary retention. The patient was treated with conservative therapy, and his symptoms gradually resolved. The Foley catheter was removed on hospital day 13, but bladder dysfunction was persisted. MRI of the lumbar spine revealed herniated lumbar discs at level L3-4, L4-5, and L5-S1, but the nerve roots were not compressed. The patient was managed with clean intermittent catheterization for more than two weeks. Cystometry revealed atonic bladder. Nineteen cases of urinary retention secondary to aseptic meningitis have been reported in the previous literature. We reviewed their clinical presentation and treatment.

A case of corticobasal degeneration of which movemental disturbances were improved by administration of amantadine

Abstract: Corticobasal degeneration(CBD) is a neurodegenerative disorder characterised clinically by apraxia, cortical sensory loss, alien limb, dementia, oculomotor abnormalities, dysarthria, postural instability, akinesia, rigidity, and pyramidal signs. Brain imaging may demonstrate greater abnormalities contralateral to the more affected side. We reported a case of corticobasal degeneration of which praxic impairments were improved by administration of amantadine. The patient was a 63-year-old right-handed woman. She showed marked dysfunction including rigidity, limb kinetic apraxia, cortical sensory loss, ideomotor apraxia, and dressing apraxia. A brain MRI scan revealed bilateral cortical atrophy centered in the postcentral gyrus, more pronounced in the left hemisphere than the right. A SPECT scan showed a decrease in blood flow in the temporo-parieto-occipital regions, more pronounced in the left hemisphere than the right. An EEG showed a diffuse slowness. L-dopa had no effect on the symptoms of rigidity, limb kinetic apraxia, cortical sensory loss, ideomotor apraxia, and dressing apraxia. By administration of amantadine, rigidity and cortical sensory loss did not improve, but some praxic impairments, such as dressing apraxia and ideomotor apraxia, and the EEG improved. Upon withdrawal of amantadine, the improved symptoms deteriorated. Amitriptyline did not improve the deteriorated symptoms. After amantadine was re-administered, the same praxic impairments and the EEG improved again. This suggested that administration of amantadine had some effect on certain praxic impairments and the EEG.

[The effect of C 1 esterase inhibitor on ischemia: reperfusion injury in the rat brain].

Abstract: BACKGROUND Despite the current interest in thrombolytic therapy for acute stroke, ischemia-reperfusion injury remains a potentially hazardous complication. The complement system is thought to play a major role in initiating some of the inflammatory events occurring in the reperfusion injury. This study was conducted to explore the effect of C 1 esterase inhibitor (C 1 INH) on the reperfusion injury in rat middle cerebral artery (MCA) occlusion-reperfusion model. METHODS Twenty-nine male Wistar rats were used. Intraluminal MCA occlusion was performed for 60 minutes. Just before the reperfusion, C 1 INH(50 IU/kg, C 1 INH group, n = 15) or saline (control group, n = 14) was administrated. Forty-eight hours after the reperfusion, infarct volume and myeloperoxidase(MPO) activity of the brain were evaluated. RESULT Infarct volume and MPO activity were significantly smaller in the C 1 INH group(86.5 +/- 76.8 mm3, 0.38 +/- 0.30 U/g) than in control group(179 +/- 92.8 mm3, 1.37 +/- 0.46 U/g) (p < 0.01). CONCLUSION The results of this study provided the first evidence that C 1 INH reduced polymorphonuclear leukocytes(PMN) accumulation and reperfusion damage in the brain.

[Isolated abducens nerve palsy caused by the compression of the basilar artery: a case report].

Abstract: Several pathological etiologies are responsible for abducens nerve palsy, but a neuro-vascular compression of the basilar artery has rarely been recognized as one of the causes. We performed magnetic resonance imaging of the brainstem of 71-year-old female patient with left abducens nerve palsy using constructive interference in the steady state (CISS) method, which allowed high-resolution T 2-weighted imaging and detection of the artery and nerves as high and low intensity area respectively. MRI with CISS revealed the compression of the abducens nerve by the basilar artery at the root exit zone. During the first examination her systolic blood pressure was over 180 mmHg, so we prescribed hypotensive agents until it to normalized. Her symptom of abducens nerve palsy improved after a few days. It is conceivable that compression of the basilar artery may have caused isolated abducens nerve palsy as disclosed by MRI with CISS.

[Diffusion-weighted imaging of brain death: study of apparent diffusion coefficient].

Abstract: DWI(Diffusion-weighted images) of the brain has been revealed to be useful in diagnosis of several clinical conditions. However, little is known about DWI with regard to brain death. We had opportunities to study patients with brain death. Case 1. A 34-year-old woman experienced cardiopulmonary arrest due to severe ventricular fibrillation, and resuscitated after about 120 minutes. After brain death, DWI showed high signals in the cerebral cortex, putamen, thalamus, brain stem and cerebellum, and ADC(apparent diffusion coefficient) values were 30-40% lower than those of normal volunteers. Case 2. A 45-year-old woman experienced cardiopulmonary arrest due to pontine hemorrhage, and was resuscitated after about 20 minutes. Before brain death, DWI showed high signals in the cerebral cortex, putamen, thalamus, brain stem and cerebellum, and ADC values were the same as those of normal volunteers. After brain death, DWI showed more clearly defined in these areas, but ADC values were 30-40% lower than the first values. DWI and ADC mapping shows areas corresponding to edema of a cytotoxic nature and to ischemic tissue. The characteristic views of high signals in the whole area of the brain were present, and an objective evaluation was possible with DWI after the brain death diagnosis by measuring ADC values.

Unusual MR findings of Wernicke encephalopathy with cortical involvement

Abstract: We report a 48-year-old chronic alcoholic man, who developed consciousness disturbance, oculomotor paresis, and flaccid tetraplegia. His dietary habit was very poor since one month prior to the present admission and he was drinking alcoholic beverage. On admission on April 19, 1999, he showed disturbance of consciousness, tetraparesis without sensory disturbance, gaze paresis, and vertical nystagmus on downward gaze. His blood thiamine level was 12 ng/ml (normal range: 23.8-45.9). MRI demonstrated symmetric hyperintense lesions in the motor and premotor cortices bilaterally, in addition to other changes indicating Wernicke's encephalopathy. His motor weakness and oculomotor disturbance improved after treatment with intravenous thiamine. His cortical MRI also normalized. We believe that his cortical abnormality was responsible for his motor paresis and this is an unusual and unique finding for Wernicke's encephalopathy.

[A-56-year-old woman with parkinsonism, whose mother had Parkinson's disease].

Abstract: We report a 56-year-old woman with progressive gait disturbance. Her mother had Parkinson's disease with onset at age 70. She died at age 74 and the post-mortem examination confirmed the diagnosis of Lewy body positive Parkinson's disease. The patient was well until the age of 50(1995) when she noted an onset of resting tremor and difficulty of gait. She also developed delusional ideation and was admitted to a psychiatric service of another hospital, where a major tranquilizer was given. The delusion disappeared but she developed marked rigidity. The major tranquilizer was discontinued and an anticholinergic and amantadine HCl were given. She showed marked improvement to Hoehn and Yahr stage II and was discharged. In 1995, when she was 52 years of the age, she developed delusion again and a major tranquilizer was given. She developed marked parkinsonism again and became Hoehn and Yahr stage V. The major tranquilizer was discontinued and she was treated with levodopa/carbidopa, trihexyphenidyl, bromocriptine, and dops. She improved remarkably to stage II. She was admitted to our service on October 8, 1996 for drug adjustment. She was alert and not demented. She was anxious but delusion or hallucination was noted. Higher cerebral functions were intact. Cranial nerve functions were also intact except for masked face and small voice. Her posture was stooped and steps were small. She showed retropulsion and moderate bradykinesia. Resting tremor was noted in her left hand. Rigidity was noted in both legs. No cerebellar ataxia or weakness was noted. Deep tendon reflexes were within normal range and sensation was intact. Her cranial MRI revealed some atrophic changes in the putamen, in which a T 2-high signal linear lesion was seen along the lateral border of the putamen bilaterally. In addition, posterior part of the putamen showed T 2-low signal intensity change. She was treated with 1.6 mg of talipexole, 6 mg of trihexyphenidyl, and 100 mg of L-dops. She was in stage III of Hoehn and Yahr. She developed neurogenic bladder with a large amount of residual urine for which she required catheterization. She was transferred to another hospital. Despite drug adjustment, she lost response to levodopa and her parkinsonism deteriorated gradually. She also developed syncope orthostatic hypotension. In April of 1998, she developed intracerebral hemorrhage and was admitted again on April 19, 1998. She was unable to stand and showed marked akinesia and rigidity. She was in stage V of Hoehn and Yahr. Her cranial CT scan revealed bilateral high-density lesions in the posterior parietal lobes. She developed dysphagia for which she required gastrostomy. She was transferred to another hospital but her clinical condition deteriorated further. On December 22, 1999, she developed fever and dyspnea and was admitted to our service again. She developed cardial arrest at the emergency room from hypoxia. She was resuscitated; however, she was comatose with loss of brain stem reflexes. Later on she developed generalized myoclonus. She developed cardiac arrest and pronounced dead on December 28, 1999. The patient was discussed in a neurological CPC. The chief discussant arrived at the conclusion that the patient had striatonigral degeneration because of poor response to levodopa in the later course, autonomic failures, and MRI changes. Some other participants thought that the patient had a form of familial Parkinson's disease. Opinions were divided into these two possibilities. Post-mortem examination revealed that the substantia nigra showed intense neuronal loss and gliosis, however, no Lewy bodies were seen. In addition, intracytoplasmic inclusions were seen in oligodendrocytes. The putamen was markedly atrophic in its posterior part with marked gliosis and neuronal loss. The ventromedial part of the pontine nucleus also showed neuronal loss and intracytoplasmic glial inclusions. Pathologic diagnosis was multiple system atrophy. In the parietal lobe, an arteriovenous malformation with bleeding was noted. This is very unique case. Although her mother had Lewy body-positive Parkinson's disease, the patient had Lewy body-negative multiple system atrophy with a-synuclein-positive glial inclusions. Whether this is just a coincidental occurrence or the presence of a genetic load for Parkinson's disease might triggered her multiple system atrophy is an interesting question to be answered in future.

[Prevalence of Martin-Gruber anastomosis on motor nerve conduction studies].

Abstract: Prevalence of median to ulnar anastomosis in the forearm(Martin-Gruber anastomosis; MGA) to the first dorsal interosseous(FDI), abductor digiti quinti (ADQ) and adductor pollicis(AP) was investigated. Subjects contained 106 patients with normal nerve conduction or patients with various neuropathies. Recording electrodes were placed on the motor point of FDI, ADQ and AP. Supramaximal stimulations were given to the median and ulnar nerves at the wrist or above the elbow. The diagnosis of MGA was made by the following criteria; amplitude of compound muscle action potential(CMAP) increased after elbow stimulation as compared with the wrist stimulation in median nerve conduction studies. The corresponding decrease in CMAP amplitude was found after above elbow stimulation as compared with the wrist stimulation in ulnar nerve conduction studies. No MGA was found in 80(75%) out of 106 patients. MGA to FDI was found in all 26 patients who had MGA. MGA to ADQ and AP was found in 11% and 10% of the patients, respectively. Only 8 out of 26 patients had MGA to all 3 muscles. In the presence of MGA median motor nerve conduction studies demonstrate larger CMAP, with a small initial positivity, after elbow stimulation than after wrist stimulation. And moreover, ulnar motor nerve conduction studies reveal a conduction block-like finding in the forearm. In this study MGA was found in 25% of the patient to FDI, in 11% to ADQ and in 10% to AP. Although a very small MGA might be overlooked in our method, such a small MGA doesn't mislead us into erroneous interpretation of motor nerve conduction studies.

[Magnetic resonance imaging findings of postresuscitation encephalopathy: sequential change and correlation with clinical outcome].

Abstract: OBJECTIVE The purpose of this study was to describe the findings of sequential magnetic resonance imaging (MRI) in postresuscitation encephalopathy. Although its outcome is known to be overwhelming, but its acute findings by variable imaging methods are subtle and show only limited values. The correlation of the findings of MRI with clinical outcome were also analyzed. METHODS Twelve patients with global cerebral anoxia who underwent MRI with conventional and diffusion-weighted imaging were enrolled in this study. Compared with normal MRI images, abnormal signal regions were checked and described in cortex, basal ganglia and white matter. Also medical records were carefully reviewed to study the cause, the time necessary for resuscitation and long term clinical outcome. RESULTS The earliest finding was obtained by diffusion-weighted image less than 24 hours (acute period) in bilateral cerebral cortex as bright high signal intensity regions. Similar abnormality of bright high signal area in FLAIR and T 2 was followed according to the time elapsed in early subacute period (1-13 days). Succeedingly, white matter was involved and laminar necrosis in cortical area was observed in late subacute period (14-20 days). Finally, diffuse brain atrophy and obtundation of gray-white matter junction were seen in chronic stage (after 21 days). These MR findings were coincided well with histopathological findings reported in literatures. The poor outcome was closely and significantly correlated with abnormality in MR images. CONCLUSION MRI was a useful diagnostic modality to diagnose the whole brain ischemic encephalopathy and to predict the prognosis.

Relationship between hyperglycemia following head injury and neurological outcome

Abstract: Severe head injury is associated with a stress response that includes hyperglycemia, which has been shown in both experimental and clinical studies to exacerbate the severity of brain injury during ischemic conditions To define the relationship between serum glucose levels and the outcome of patients suffering from closed head injury, we retrospectively reviewed the clinical courses of 88 consecutive head-injured patients The patients were divided into two groups according to their GCS score on admission: severely head-injured group (GCS score of 8 or less) in 36 patients; moderately head-injured group (GCS score of 9 to 12) in 52 patients Severely head-injured group had significantly higher serum glucose levels than moderately head-injured group (mean +/- standard error of the mean; 201 +/- 46 mg/dl vs 1714 +/- 38 mg/dl) (p < 001) Patients who subsequently resulted in severe disability, vegetative state, or death had significantly higher serum glucose levels than patients who had good recovery or moderate disability (2049 +/- 59 mg/dl vs 1629 +/- 51 mg/dl) (p < 001) Cases with a fatal clinical course were mostly associated with high glucose levels All patients who showed a serum glucose level greater than 240 mg/dl on admission were dead These data suggest that the hyperglycemia on admission is a frequent component of the stress response to head injury, a significant indicator of severity of injury and a potent predictor of the outcome from head injury

Atypical Fisher syndrome with optic nerve involvement

Abstract: We report a 38-year-old woman who developed what appeared to be Fisher syndrome associated with optic nerve involvement. One week after a common cold, she developed double vision and left facial palsy. Four days after the onset, she developed bilateral blurred vision, painful total ophthalmoplegia, and ataxic gait. Brain CT and MRI findings were normal. Her vision worsened but the optic fundi were normal. Serum anti-GQ 1 b antibody was elevated. She received steroid therapy at another hospital, and her vision, facial palsy and ocular pain improved. She was transferred to our hospital and we treated her by plasma exchange. She showed near complete recovery. Human optic and ocular nerves contain high amount of GQ 1 b. This may be a underlying mechanism for optic nerve involvement in Fisher syndrome. We thought that she had an atypical Fisher syndrome associated with optic nerve involvement.

Minimally invasive stereotactic functional surgery using an intravenous anesthetic propofol and applying Image Fusion and AtlasPlan

Abstract: Image-guided stereotactic surgery of the ventralis intermedius nucleus of the thalamus, globus pallidus, and subthalamic nucleus is a prevailing modality as a treatment of movement disorders. This technical note describes a method of minimally-invasive stereotactic functional surgery for patients with parkinsonian symptom or various tremors. Patients were administered propofol, an intravenous anesthetic, during placement of a burr hole and a stereotactic frame, but not the period when the patients were necessary to be awake. The intravenous anesthesia was very beneficial to relieve local pain and mental stress of the patients. Radionics ImageFusion and AtlasPlan were used for defining the target localization without an intraoperative ventriculography. ImageFusion efficiently fused high-resolution MR images on CT images. AtlasPlan accurately corrected the localization of the tentative target point after measurements from the midpoint of the anterior commissure-posterior commissure line on the modified MRI, and enabled us to reduce the degree of an intraoperative correction to fix a final target. Stereotactic functional surgery has been thought to be less-invasive, however further modifications of surgical procedure and intraoperative medication can make this type of surgery much more less-invasive.

[A case of Marchiafava-Bignami disease caused by anorexia nervosa].

Abstract: A 29-year-old female without alcoholism whose height and weight were 152 cm and 28 kg had complained of severe anorexia since about 10 years ago. The splenium of the corpus callosum showed symmetrically low-intensity area in T1 WI and high-intensity area in T2 WI on her brain MRI. These findings could be reversible by the recovery of her nutritional condition. The findings of neurological and neuropsychological examinations were normal. To our knowledge, this is the first case report of Marchiafava-Bignami disease caused by Anorexia Nervosa.

A case of crossed aphasia with echolalia after the resection of tumor in the right medial frontal lobe

Abstract: We report a right-handed woman, who developed a non-fluent aphasia after resection of astrocytoma (grade III) in the right medial frontal lobe. On admission to the rehabilitation department, neurological examination revealed mild left hemiparesis, hyperreflexia on the left side and grasp reflex on the left hand. Neuropsychologically she showed general inattention, non-fluent aphasia, acalculia, constructional disability, and mild buccofacial apraxia. No other apraxia, unilateral spatial neglect or extinction phenomena were observed. An MRI demonstrated resected areas in the right superior frontal gyrus, subcortical region in the right middle frontal gyrus, anterior part of the cingulate gyrus, a part of supplementary motor area. Surrounding area in the right frontal lobe showed diffuse signal change. She demonstrated non-fluent aprosodic speech with word finding difficulty. No phonemic paraphasia, or anarthria was observed. Auditory comprehension was fair with some difficulty in comprehending complex commands. Naming was good, but verbal fluency tests for a category or phonemic cuing was severely impaired. She could repeat words but not sentences. Reading comprehension was disturbed by semantic paralexia and writing words was poor for both Kana (syllabogram) and Kanji(logogram) characters. A significant feature of her speech was mitigated echolalia. In both free conversation and examination setting, she often repeated phrases spoken to her which she used to start her speech. In addition, she repeated words spoken to others which were totally irrelevant to her conversation. She was aware of her echoing, which always embarrassed her. She described her echolalic tendency as a great nuisance. However, once echoing being forbidden, she could not initiate her speech and made incorrect responses after long delay. Thus, her compulsive echolalia helped to start her speech. Only four patients with crossed aphasia demonstrated echolalia in the literature. They showed severe aphasia with markedly decreased speech and severe comprehension deficit. A patient with a similar lesion in the right medial frontal lobe had aspontaneity in general and language function per se could not be examined properly. Echolalia related to the medial frontal lesion in the language dominant hemisphere was described as a compulsive speech response, because some other 'echoing' phenomena or compulsive behavior were also observed in these patients. On the other hand, some patients with a large lesion in the right hemisphere tended to respond to stimuli directed to other patients, so called 'response-to-next-patient-stimulation'. This behavior was explained by disinhibited shift of attention or perseveration of the set. Both compulsive speech responses and 'response-to-next-patient-stimulation' like phenomena may have contributed to the echolalia phenomena of the present case.

[Risk factor for cerebral hemorrhage for the patient with lacunar infarction: investigation of 5 cases associated with both of symptomatic hemorrhagic and ischemic strokes].

Abstract: Background and purpose Microangiopathy is regarded as an important cause of intracerebral hematoma(ICH) and lacunar infarction Dot-like low intensity spots on T2-weighted echo planar image(EPI) have been regarded as hemosiderin deposit associated with microangiopathy However, clinical significance of dot-like hemosiderin spot(dotHS) is still debated Therefore, we analyzed the number of dotHS on EPI of symptomatic lacunar infarction associated with ICH Methods To investigate how the dotHS or risk factors contributed to hemorrhagic strokes for patients with lacunar infarction, the number of dotHS and various risk factors were made a comparison between 20 cases with symptomatic lacunar infarctions(lacunar group) and 5 cases with both symptomatic lacunar infarction and symptomatic ICH(complicated group) In addition to EPI, fluid attenuated inversion recovery image, and T1- and T2-weighted MR images were performed for differential diagnosis of dot HS Results EPI demonstrated that asymptomatic ICH was significantly more frequent in complicated group (60%) than in lacunar group(10%), and dotHS were significantly more frequent in complicated group(100%) than in lacunar group(50%) The number of dotHS of complicated group was 146 +/- 43, which was significantly larger than that of lacunar group(41 +/- 92) No significant difference between two groups were founded in other risk factors including hypertension, diabetes mellitus, hyperlipidemia, and smoking Conclusion These results suggested that dotHS was one of the risk factors for ICH for patients with symptomatic lacunar infarction, and an increasing number of dotHS was one of the predictive factors of symptomatic and/or asymptomatic ICH

[A patient with Moyamoya-like vessels after radiation therapy for a tumor in the basal ganglia].

Abstract: A patient with Moyamoya-like vessels after radiation therapy for treatment of a tumor in the basal ganglia is reported. He was diagnosed as Down syndrome at birth. He had a tumor in the left basal ganglionic region at 12 years of the age. The tumor increased in size at age 14. He underwent cerebral angiography, which did not show a stenosis nor occlusion of the internal carotid artery, anterior cerebral artery, nor the middle cerebral artery. He received radiation therapy with a total dose of 56 Gy. He presented a dressing apraxia at age 19. MRI showed cerebral infarction in the left temporo-occipital region. Right internal carotid angiography revealed a severe stenosis of the internal carotid artery and anterior cerebral artery as well as a severe stenosis of the middle cerebral artery on the right side. Moyamoya-like vessels were seen in the basal ganglionic region. Left internal carotid angiography also showed a stenosis of the internal carotid artery and anterior cerebral artery as well as a severe stenosis of the middle cerebral artery on the left side. Moyamoya-like vessels were seen in the basal ganglionic region. Leptomeningeal anastomose and transdural anastomose were bilaterally seen. These arterial occlusion and stenotic phenomenon corresponded to a previous radiation field. These Moyamoya-like vessels with arterial stenosis and occlusion were thought to be due to radiation-induced vasculopathy, because a previous cerebral angiography showed a normal caliber of cerebral arteries. This patient showed that patients with radiation therapy in their early childhood should be carefully observed considering the possibility of this phenomenon.

Placement of stent for internal carotid artery stenosis in the cervical portion improved ocular ischemic syndrome

Abstract: We report a case of ocular ischemic syndrome due to severe stenosis of the internal carotid artery. The patient was a 67-year-old man with a hemispheric transient ischemic attack and an amaurosis fugax. Ocular examination showed multiple soft exudates and dilatation of the retinal veins. Carotid stenting was performed to reconstruct the internal carotid artery. The multiple soft exudates were almost diminished and the visual acuity was improved. These results suggest that carotid stenting for severe stenosis of the internal carotid artery should be one of the less invasive methods to improve the microcirculation of the retina.

[Hand motor cortical area reorganization following cerebral infarction evaluated with functional MRI, near infrared spectroscopic imaging, and transcranial magnetic stimulation].

Abstract: A 60-year-old, right-handed man suffered from left hemiparesis with upper limb dominance. CT and MRI revealed cerebral infarction of the entire right middle cerebral artery territory. His hemiparesis recovered excellently and residual neurological deficits 6 years later were left hand weakness(grasping power 9 kg vs. 35 kg in the right) and clumsiness. Functional MRI was performed. During right(normal) hand grasping, activation was seen in the left sensorimotor cortex and supplementary motor area. During left(paretic) hand grasping, activation was seen in the left (ipsilateral) sensorimotor cortex, right parietal cortex, and bilateral supplementary motor areas. Near infrared spectroscopic imaging showed similar results. During right hand grasping, left sensorimotor cortex was activated, and during left hand grasping, bilateral sensorimotor cortices were activated with ipsilateral predominance. Transcranial magnetic stimulation of the left motor hand area evoked right hand movement and stimulation of a point near that area evoked ipsilateral left hand muscle movement. Thus, the findings of the three techniques consistently suggest that the recovery of left hemiparesis of this patient was promoted by motor cortical area reorganization including the ipsilateral motor cortex.

Symptomatic subependymoma of the lateral ventricle in a young female

Abstract: We present a case of a symptomatic subependymoma (SE) in a young. The patient was a 20-year-old female who suffered from severe headache and vomiting. Precontrast CT scans revealed a large spotty-calcified isodensity mass lesion in the right anterior lateral ventricle, showing scarce contrast enhancement at the center of the mass and with marked hydrocephalus. MRI demonstrated the lesion as slightly hypo-intensity on T1-weighted image, hyper-intensity on T2-weighted image. On FLAIR imaging, the lesion indicated remarkable hyperintensity and was clearly distinguishable from surrounding brain parenchyma and the cerebrospinal fluid. After Gd-DTPA infusion central vein was revealed, but no tumor enhancement was seen. The tumor was totally extirpated through the transcortical approach. Histological diagnosis was SE, and high MIB-1 staining index (9.3%) was seen. FLAIR imaging is useful for the preoperative diagnosis and the postoperative assessment of SEs.

[A case of brainstem encephalitis associated with Epstein-Barr virus infection: differentiation of acute disseminated encephalomyelitis and Bickerstaff's brainstem encephalitis].

Abstract: A 28-year-old woman initially suffered high fever and headache (day 1). Aseptic meningitis was diagnosed on day 3. Limb ataxia, however, appeared on day 7, and external ophthalmoplegia and drowsiness were recognized on day 8. Urinary disturbance and orthostatic hypotension appeared on day 13. Cerebrospinal fluid showed moderately high cell counts(mononuclear cells, 51/microliter; polynuclear cells, 9/microliter). MRI T2-weighted images showed high intensity lesions in the pons and mesencephalon. No serum anti-GQ1b IgG antibody was detected on day 4. Epstein-Barr virus (EBV) viral capsid antigen-IgG antibody was positive, and EBV determined nuclear antigen antibody was seroconverted. EBV-DNA was detected in the CSF by PCR. These findings indicate prior infection by EBV. After intravenous dexamethasone therapy, these symptoms rapidly disappeared. Our patient showed external ophthalmoplegia, ataxia, and disturbance of consciousness, which are the cardinal signs in Bickerstaff's brainstem encephalitis. The time course of her neurological symptoms, the presence of meningitis, and the MRI findings, however, indicated the pathogenesis of acute disseminated encephalomyelitis rather than Bickerstaff's brainstem encephalitis. We diagnosed this patient as a brainstem encephalitis associated with EBV infection.

Innervation pattern to the extensor digitorum brevis by deep peroneal nerve and accessory deep peroneal nerve

Abstract: On the electrophysiological basis, extensor digitorum brevis(EDB) muscle is innervated electrophysiologically not only by deep peroneal nerve(DPN) but also by accessory deep peroneal nerve(ADPN), an anomalous branch of superficial peroneal nerve, with a prevalence of 17-28%. We investigated 23 patients who had both DPN and sufficient ADPN innervation to the EDB on the intramuscular distribution of DPN and ADPN innervation to the medial and lateral side of the EDB. Recording electrodes were placed on the medial and lateral edges of the EDB with a supramaximal stimulation to the anterior or lateral ankle, compound muscle action potential (CMAP) of DPN or ADPN innervation was recorded. In 19 patients (83%) the DPN innervation was larger than or equal to the ADPN innervation. Only in 4 patients (17%) the ADPN innervation obviously exceeded the DPN innervation. DPN enters to the EDB from the medial side, and ADPN from the lateral side of the EDB. In 16 patients(70%) the DPN innervation was relatively large and the ADPN innervation was relatively small at the medial side of the EDB, and vice versa at the lateral side of the EDB. These distributions were almost uniform in 5 patients(22%). This study clarified that a biased larger DPN innervation and smaller ADPN innervation to the medial side of the EDB, and vice versa to the lateral side of the EDB in the majority cases. In some cases diffuse innervation to the EDB was found.

The role of transplanted astrocytes for the regeneration of CNS axons

Abstract: Long tract axons in the mammalian CNS do not normally regenerate for appreciable distance after they transected. But we reported transplantation of Schwann cells(SCs) or olfactory ensheathing cells induced regeneration of transected rat dorsal column (DC) axons and improved the conduction. Scar formation(gliosis), for which astrocytes(ACs) play an important role, may be one of strong and physical barriers for the regeneration of CNS axon. Oligodendrocyte and myelin associated protein or products also inhibit the regeneration of the axons, as chemical barriers. To investigate how effective the promotion or the reduction of scar or myelin formation may be for axonal regeneration, we transplanted AC into transected DCs, or radiated(X-ray) the DCs, and compared to normal DCs or regenerated DCs following by SC transplantation. DCs of adult rats were transected at Th 11 and transplanted with SCs(6 x 10(4)) of adult rats or ACs(6 x 10(4)) of neonatal rats. Five to six weeks later, the spinal cords were removed and pinned in a recording chamber, and compound action potentials (CAPs) along the DC through the transected lesion were recorded, to investigate conduction properties(conduction velocity and response after high frequency stimulations). Following transplantation of SCs or ACs, histological examination revealed regenerated axons with SC-like patterns of remyelination in transected DCs. X-ray irradiation did not enhance the regeneration of DC axons. SC transplantation improved the conduction properties of transected DCs and increased the number of regenerated axons, compared to transected DCs without cell transplantation. AC transplantation resulted in improvement of the conduction properties, but the number of regenerated axons was similar to that of transected DCs without the transplantation. X-ray irradiation (40 Gy) three days before DC transection and AC transplantation prevented the electrophysiological continuity of axons through the transected lesion. This evidence revealed that AC transplantation secondarily enhanced the regeneration of axons, probably endogeneous SCs of dorsal roots migrated into the transected lesion and enhanced the axonal regeneration.