Search

To report a case of idiopathic spinal epidural lipomatosis (SEDL) presented with unique radicular pain most likely caused by enlarged veins surrounding nerve roots.A 26-year-old male presented with radicular pain of the right T6-T7 area. He also showed Becker's nevus in the corresponding area. CT myelography and magnetic resonance imaging revealed epidural lipomatosis posterior to T4-T8 of the spinal cord. Surgical removal of adipose tissue and a hemilaminectomy of T4-T7 were performed and resulted in relief of the radicular pain.Lipomatosis was histologically confirmed and surrounded by enlarged veins. These abnormally enlarged veins compressed the nerve roots and were thought to cause radicular pain. Also, Becker's nevus of this case seems to have some relationship with SEDL.

A 39-year-old woman had developed systemic lupus erythematosus(SLE) at the age of 29. She had a long history of immunosuppressant therapies such as corticosteroid. On admission, she presented a headache due to the cryptococcal meningitis which was confirmed by lumbar puncture. Combined medications of amphotericin B and fluconazole were not effective, and combined amphotericin B and flucytosine were replaced. Prednisolone and methotrexate had been tapered gradually. Fifty days after the initial treatment for meningitis Cryptococcal neoformans was not observed in the cerebrospinal fluid. Sixty days after the treatment, thrombocytopenia was observed with positive lupus anticoagulant and anticardiolipin antibody. Following which, thrombophlebitis occurred in the left brachium. We suggest that the provoked pathoimmunological reaction such as antiphospholipid antibody syndrome during the treatment for meningitis needs to be cared during the course of SLE.

A 30-year-old woman presented with ataxic gait and progressive mental deterioration, and 3 years later developed myoclonus in the limbs. Subsequently, she lapsed into an akinetic state and died more than 6 years after the onset of disease. The brain weighed 670 g, and preferential degeneration was found in the medial thalamus and the inferior olivary nucleus. In the cerebrum and cerebellum, gliosis and neuronal depletion were only mild and disintegration of the parenchymal structures was inconspicuous, despite pronounced atrophy. The patient had methionine homozygosity at codon 129 of the PrP gene and protease-resistant PrP type 2 in the brain. On PrP immunostaining, plaque-like deposits were detected in the cerebral and cerebellar cortices. Severe brain atrophy such as in the present case has never been described in the thalamic variant of sporadic CJD.

The patient was 42-year-old woman who had exhibited elevated levels of serum creatine kinase(CK) and intermittent weakness of proximal muscles since her thirties. She had a history of palmoplantar pustulosis, Mondor's disease and recurrent miscarriages. Basedow's disease, which had been treated with antithyroid drugs since 37 years of age, recurred during the fourth pregnancy. After the pregnancy was terminated in the sixth week, weakness and grasp pain in the proximal muscles developed. The biopsy of biceps brachii muscle showed necrosis and reconstruction of muscle fibers with equivocal inflammatory cells, which was compatible with the findings for inflammatory myopathy such as polymyositis(PM). She was treated with prednisolone and the weakness and grasp pain in the proximal muscles were resolved. PM beginning during a woman's reproductive period is rare, and few studies have examined the association between PM and pregnancy. In this case, pregnancy and her past diseases were considered to be linked to an autoimmune abnormality that might have contributed to the inflammatory myopathy.

We studied 10 patients with acute ischemic cerebrovascular disorders presenting paralysis confined to one limb, unaccompanied by sensory signs(pure motor monoparesis, PMM) on diffusion-weighted MR imaging(DWI). DWI revealed fresh ischemic lesions in all patients, except for 2 cases of transient ischemic attack. On DWI, acute infarction in multiple lesions was identified, and small superficial lesions were clearly described. Superficial lesions were seen in 4 patients, and deep lesions were also seen in 4 patients. DWI is useful for lesion analysis in cerebral infarction with PMM.

A 47-year-old woman developed consciousness disturbance, and experienced hallucinations while traveling abroad, and then went into critical condition. She was placed in the critical care unit, and had flaccid tetraparesis requiring mechanical ventilation. Haemophilus influenzae was cultured from the sputum. The level of protein of the cerebrospinal fluid was elevated to 114 mg/dl, nerve conduction study showed findings of pure axonal damage, and the sural nerve biopsy revealed severe axonal degeneration. She improved gradually by plasma exchange. The diagnosis of acute motor sensory axonal polyneuropathy (AMSAN) based on autoimmune mechanism was made. We speculate that H. influenzae infection may have elicited AMSAN in this case.

This study evaluated efficacy and safety of donepezil hydrochloride (donepezil) at 5 mg/day in patients with mild to moderately severe Alzheimer's disease for 24 weeks in a double-blind, placebo-controlled comparative trial. In this study, 268 patients were enrolled and 39 of these (15%) were withdrawn. In the evaluable population of efficacy, Protocol-Compatible (PC) analyzed patients (n = 228), better effects than that of placebo were confirmed using two primary efficacy measures: a cognitive performance test, the Japanese version of the Alzheimer's Disease Assessment Scale - cognitive subscale (ADAS-J cog, p = 0.003) and a clinical global assessment, the Japanese version of the Clinical Global Impression of Change (J-CGIC, p = 0.000). The superiority of donepezil was also shown by secondary measures: the Sum of the Boxes of the Clinical Dementia Rating (CDR-SB), the Mental Function Impairment Scale (MENFIS) and the caregiver-rated modified Crichton scale (CMCS). The same results were obtained in the intention-to-treat (ITT) analysis (n = 263). The incidence of drug-related adverse events was 10% (14/136) in the donepezil and 8% (10/131) in the placebo group; no significant difference was seen between the two groups. The main adverse events were gastrointestinal symptoms, and these were almost all mild, and they all disappeared with continued administration or temporary discontinuation of donepezil. These results indicate that the donepezil appears to be effective and well tolerated in patients with mild to moderately severe Alzheimer's disease.

A 15-year-old male developed symmetrical weakness of the limb muscles. He had not had any previous developmental disorders except delayed initiation of walking. Flexion contraction of ankle joint and pes cavus deformity were seen. The cerebrospinal fluid protein concentration was elevated. Nerve conduction study showed severe conduction block and temporal dispersion. A sural nerve biopsy revealed remarkable onion bulb-like changes and perivascular infiltration of inflammatory cells. After high-dose corticosteroid treatment, he showed improvement in muscle strength. Although there were no abnormalities of genes related to hereditary neuropathy, the atypical findings of severe demyelinating changes of peripheral nerves mimicked hereditary neuropathy.

A 59-year-old man was admitted to the hospital due to leg edema. He had multiple sclerosis-like episodes of transient diplopia at the age of 36 years and spastic paraplegia at the age of 38. After admission he showed various fluctuating neuropsychological symptoms (disorientation, hallucination, apraxia, aphasia) and cranial nerve palsy. Magnetic resonance imaging revealed abnormal signal intensity in the right thalamus and deep white matter. The patient was diagnosed as having malignant lymphoma and treated with steroid therapy and chemotherapy. Although partial improvement of clinical findings was observed, recurrent cerebral hemorrhage followed. Autopsy findings revealed perivascular infiltration of T cells in the brain, spinal cord and other general organs.

The patient was a 47-year-old female. A giant meningioma was detected in the right middle cranial fossa by exploration for the cause of right-sided transient monoocular blindness of a 1-2 minute duration accompanied by headache. The headache was localized on the right side, occurred more frequently early in the morning, and continued for several hours. No neurological abnormality was noted except for right-side dominant bilateral choked disc and reduced vision. Both headache and transient monoocular blindness disappeared after total resection of the meningioma. A decrease in the cerebral blood flow associated with an increase in the intracranial pressure and right-sided occurrence of paroxysmal visual impairment due to choked disc were possible mechanisms of the transient monoocular blindness. Amaurosis fugax must be initially first in patients complaining of transient monoocular blindness, and exploration for carotid artery lesions is important. In the present case, however, this symptom led to detection of a giant meningioma.

A 50-year-old man was admitted, because of motor weakness of the lower limbs, dysesthesia of the left lower extremity, and anuresis. He had an episode of pain in his gluteal region 17 years ago, and then, no abnormalities were detected including myelography in a hospital, followed by slowly progressive muscular atrophy of his lower legs. At 50 years of age, dysuria appeared. He was diagnosed as having neurogenic bladder by urologists, and was admitted to our hospital. On admission, abnormal neurologic findings included: severe muscular atrophy in his lower legs, pes cavus, dysesthesia at the left S1 level, and autonomic bladder. Magnetic resonance imaging (MRI) showed mass lesion involving lower conus and cauda equina. After resection, pathological study revealed the mass was a neurenteric cyst. It is said that the neurenteric cyst causes an asymmetrical and sequential loss of specific neurological functions, with a subsequent return of these functions in the reverse order. That mechanism is not clear. However, in our case, the course of the illness was slowly progressive. We speculate that, because of the cyst's adhesion to cauda equina and perforation through the cyst by a nerve root, the cyst was fixed and caused slowly progressive neurological deficits in proportion to increase of the cyst's size. Our report suggests that a neurenteric cyst, involving the lower conus and cauda equina, can produce severe muscular atrophy in the lower legs.

Prion diseases are now a focus of attention since the diseases are known to disseminate beyond expectation. They are various types of Gerstmann-Sträussler-Scheinker syndrome(GSS), fatal familial insomnia, iatrogenic Creutzfeldt-Jakob disease(CJD), and variant CJD which suspected to have disseminated from bovine spongiform encephalopathy. Abnormal prion protein deposits as amyloid structure in the brain of the patients with these diseases. Mutation of the prion protein gene exists in a part of these diseases. Depending on their kind, patients represent a variety of clinical and neuropathological manifestations. They can be clinically divided into two general groups of the CJD type and the GSS type. For clinical diagnosis of the GSS type prion diseases, analysis of prion protein gene is useful.

We report a patient who developed right sided cogwheel rigidity and resting tremor after left midbrain hemorrhage. Brain magnetic resonance imaging (MRI) showed left midbrain old hemorrhage including substantia nigra. I-123 iodoamphetamine single photon emission computed tomography (IMP-SPECT) images showed reduced radioisotope (RI)-uptake in the left striatum, thalamus and frontal lobe. Our report shows that focal midbrain lesion can produce parkinsonism.

This report concerns an autopsy case of corticobasal degeneration (CBD). The patient was a 64-year-old woman with slowly progressing clumsiness and gait disturbance who died approximately 7 years after the onset of symptoms. The salient neuropathological findings were focal loss of neurons of the cerebral cortex, ballooned neurons and gliosis of the subcortical white matter, thalamus, and basal ganglia. In addition, there were marked neuronal loss and gliosis in the substantia nigra. The ballooned neurons were positively immunostained for phosphorylated neurofilament, alpha B-crystallin and synaptophysin; staining for the latter was intraneuronal. The examination of silver impregnated and Tau immunostained preparations revealed numerous basal neurofibrillary tangles, neuropil threads, and glial inclusions; their presence was most pronounced in the brainstem tegmentum, basal ganglia, and thalamus. Neurofibrillary tangles consisting of straight tubules with a diameter of approximately 13-15 nm and constricted tubules were identified in the substantia nigra. The results of the present study indicate that the cytopathology of the subcortical gray matter and brainstem in CBD patients resembles that of progressive supranuclear palsy.

A 49-year-old woman developed slowly progressive muscle weakness of the right upper and lower limbs. Physical examination revealed exaggeration of deep tendon reflexes in bilateral lower extremities and a Th5-Th6 girdle sensation. Weakness in her right upper extremity suggested cervical or intracranial lesion. Neuroradiological studies detected no abnormalities in her cervical cord and cranium. So the symptoms and signs were similar to those of motor neuron disease except for the sensory disturbance. MRI study of thoracic cord demonstrated a thoracic extradural arachnoid cyst. After removal of the cyst, the patient's muscle weakness was prominently relieved. We postulate that the cyst stretched spinal cord and dura mater, which led to affection of her cervical cord. We propose weakness of an upper limb as a pseudo-localizing sign of a thoracic extradural arachnoid cyst.

We report a patient who developed acute cerebellar ataxia, delirium, disseminated intravascular coagulation (DIC), and acute renal failure following typhoid fever. After treatment his general conditions improved except for cerebellar ataxia. Brain magnetic resonance imaging (MRI), before and after intravenous gadolinium administration, showed slight atrophy of the cerebellar vermis. 1-123 iodoamphetamine single photon emission computed tomography (IMP-SPECT) images showed markedly reduced radioisotope (RI) uptake in the cerebellum, and strongly implicated dysfunction of the cerebellum. Our report suggests that severe typhoid fever can produce persistent cerebellar dysfunction.

We performed a vesicourethral function study on seven patients with progressive supranuclear palsy. In storage phase, 6 patients had decreased urinary sensation and overactive detrusor. Although bladder compliance was normal in all patients, maximum cystometric capacity was decreased in 3 patients. In micturition phase, detrusor contraction was underactive in 4 patients and acontractile in 1 patient. Sphincter electromyogram showed detrusor-sphincter-dyssynergia in 1 patient, no decrease in 3 patients and synergistic decrease in 1 patient. Six patients had urinary incontinence partially due to those neurological abnormality, partially due to dementia and lower activity of daily living. To facilitate the care of such functional incontinence, we devised a urinary alarm. The urinary alarm is a device to detect urine in a diaper. One can know the micturition in a diaper without being informed of micturition by the patient and change diapers as soon as possible. It was also useful to examine their frequency/volume chart.

We compared the vesicourethral function between progressive supranuclear palsy (7 cases) and Parkinson's disease (vesicourethral function study in 8 patients with dysuria and questionnaire study in 44 patients). The frequency of urinary incontinence in the 44 patients with Parkinson's disease was 38.7%. Vesicourethral function study showed hypersensitivity, low bladder capacity, detrusor hyperreflexia and normal sphincter EMG. The frequency of urinary incontinence in supranuclear palsy was 85.7%. Vesicourethral function study showed hyposensitivity and detrusor hyperreflexia in 6 cases, and 4 of these 6 cases had residual urine due to impaired detrusor contraction in micturition phase and/or abnormal sphincter EMG. Compliance was normal, but bladder capacity was decreased in progressive cases. Impaired contraction and hyposensitivity made it more difficult to control the urinary incontinence in supranuclear palsy than in Parkinson's disease. Dementia and lower ADL were frequently accompanied with supranuclear palsy (rare in Parkinson's disease). These were other factors that worsen the control of incontinence.

A 48-year-old woman with status epilepticus was admitted to our hospital. On admission, she was comatose with horizontal conjugate deviation of eye balls to the left side. Convulsive seizure of Jacksonian type involved initially the left mouth angle and sequentially the left arm and leg, developing into general convulsion. Routine laboratory examinations revealed no significant abnormality. CT scan was normal but carotid angiogram showed a persistent proatlantal intersegmental artery on the left side. Convulsive seizure lasted five days, followed by left hemiparesis. She recovered completely two weeks later. The origin of her illness remained unclear but the causal relationship between neurological symptoms and the persistent primitive artery was presumed.

One patient who underwent radiation therapy for laryngeal cancer two years earlier developed chronic progressive radiation myelopathy and exhibited localized swelling of the cervical spinal cord at the C4 level and resultant blocking of the subarachnoid space on myelography and CT myelography. The condition responded markedly to the administration of corticosteroid except for persisting mild spasticity and sensory disturbances. On myelography and CT myelography half a year later, the cervical spinal cord showed no swelling. The corticosteroid therapy is considered to be useful for radiation myelopathy.

A 67-year-old woman with medial medullary infarction is reported, including clinical manifestations, MRI and angiographical findings, and results of evoked potentials. She suffered from contralateral hemiplegia and disturbance of deep sensation. Motor paresis of the tongue was absent. Magnetic resonance imaging revealed a lesion in the medial portion of the medulla oblongata. The 17 cases previously reported with medial medullary infarction are reviewed. Only 3 cases had triad of medial medullary infarction, contralateral hemiparesis, deep sensory disturbance, and ipsilateral hypoglossal paresis. Therefore, lesion detection is necessary to diagnose medial medullary infarction. Most infarctions limited to the upper third of the medulla were caused by occlusions of vertebral arteries or their branches and prognosis was good. In contrast, infarctions in the lower two thirds were caused by occlusions of anterior spinal arteries and their branches and the prognosis was poor. Thus localization of the lesion using MRI plays an important role to predict the prognosis.

A morphological study using a modified Golgi impregnation technique was carried out on Betz cells in the motor area of eight patients with motor neuron disease (MND) and age-matched controls without abnormal neuropathological findings during routine examination. Betz cells were categorized as normal (type I), or those with moderate (type II) or marked (type III) degeneration of dendrites. Types II and III Betz cells tended to be dominant in the cases of MND, whereas type I Betz cells constituted the highest percentage in most of the control cases. The frequently observed morphological alterations of Betz cells in MND were accumulation of lipofuscin, degeneration or loss of dendrites, and reactive astrocytic gliosis around the Betz cell soma. It was suggested that Betz cell changes in MND are not only due to aging but also to evident pathological processes, since the above-mentioned findings were more extensive in the MND cases than in the control cases. Also, the so-called lower motor neuron disease belongs to one basic disease termed "motor neuron disease", because the pathological changes of Betz cells were observed even in cases of spinal progressive muscular atrophy alone or associated with progressive bulbar palsy, which are devoid of pyramidal tract degeneration and signs of upper motor neuron lesion.

我々は,脊髄小脳変性症の症例で,本人,母親,妹に血清vitamin B6濃度の低下を認め, vitamin B6静脈内投与により,症状の一過性の改善を認めた. vitamin B6と脊髄小脳変性症の関連について述べた報告はほとんどなく,貴重な症例と考えられる.

To examine the metabolic products of thyroid hormones in the central nervous system, total and free rT3 as well as T4 in human cerebrospinal fluid (CSF) were determined in 41 patients. Total T4, T3, and rT3 concentrations in CSF were determined by RIA, and free T4, T3, and rT3 fractions were analyzed by the magnesium precipitation method. The mean (+/- SE) total T4 and T3 concentrations in CSF were 166 +/- 24 and 2.6 +/- 1.5 ng/dl, respectively; the values were approximatey 2% of those in normal control pooled sera. Total rT3 concentrations in CSF from 29 clinically euthyroid patients who were apparently in a normal nutritional state ranged from 5.2-23 ng/dl (mean +/- SE, 11.2 +/- 0.9 ng/dl); the value was approximately 40% of that in sera. A patient with primary hypothyroidism had a markedly diminished CSF rT3 concentration (1.1 ng/dl), and the remaining 11 patients, suffering from severe diseases such as meningitis and cerebrovascular accident, had significantly higher CSF rT3 concentrations (37.5 +/- 6.6 ng/dl) than the clinically euthyroid patients. Although free T4 and T3 concentrations in CSF (5.9 +/- 1.1 and 0.16 +/- 0.05 ng/dl, respectively) were similar to those in serum (2.1 +/- 0.2 and 0.22 +/- 0.01 ng/dl, respectively), the free rT3 concentration in CSF (0.70 +/- 0.12 ng/dl) was approximately 20 times higher than that (0.044 +/- 0.016 ng/dl) in serum. Furthermore, the reciprocal relationship was observed between CSF total rT3 concentrations and free rT3 percentages in CSF. These data suggest the possibility that rT3 may be produced from T4 in human brain and that there may be a transport mechanism regulating free rT3 in CSF.

Among 39 cases with acute-onset amnestic syndrome having unilateral localized infarct, 8 cases with anteromedial thalamic infarct ("thalamic" amnesia), and 18 cases with medial temporal lobe infarct including hippocampus in the posterior cerebral artery territory ("PCA" amnesia) were studied in terms of X-CT and MRI findings and neuropsychological examinations. Results were as follows: 7 out of 8 cases with thalamic amnesia (88%), and 15 of 19 cases with PCA amnesia (78%) showed left side lesions on CT scan. All groups showed a prolonged recent memory loss with little loss of immediate recall and remote memory, and disorientation and dyscalculia. In both types of amnesia, patients having a left sided lesion showed recent memory loss with new learning disabilities of verbal materials. Patients having a right sided lesion showed recent memory loss with new learning disabilities of both verbal and visuospatial materials. Judging from the X-CT and MRI findings, the lesions most probably causing amnesia in these cases seemed to be the anterior and dorsomedial nuclei of the thalamus in thalamic amnesia and hippocampus in PCA amnesia. Differential diagnosis in amnestic syndrome with localized infarct is also discussed.

Experimental evidence has shown that the amount of 5-HIAA in the CSF reflects the metabolism of serotonin in the brain if this metabolite is eliminated from the brain and flows into the CSF at a constant rate. We studied the concentration of 5-HIAA in the lumbar CSF in several neurological diseases to elucidate the alteration in abnormalities of serotonin metabolism. The concentration of 5-HIAA in the CSF was measured in 94 patients with cerebral infarction, 30 with vascular dementia, 25 with dementia of the Alzheimer type, 28 with Parkinson's disease and 6 with hypoxic encephalopathy. Patients with cerebral infarction were classified into 24 with a solitary cerebral infarct and 70 with multiple cerebral infarcts. Patients with Parkinson's disease were subdivided into 12 with various psychiatric symptoms including depressive state, hallucination and/or intellectual impairment and 16 without psychiatric symptoms. Patients with hypoxic encephalopathy consisted of 5 with apallic syndrome and one patient with Lance-Adams syndrome. The concentration of 5-HIAA in solitary cerebral infarct, multiple cerebral infarcts and vascular dementia did not exhibit a significant difference from that in control cases (54.6 +/- 23.1 ng/ml). But patients with dementia of the Alzheimer type (34.5 +/- 10.9, p less than 0.001) showed a significantly lower concentration. This fact seems to reflect the pathological finding that the number of large neurons is decreased and neurofibrillary tangles are increased in the nucleus raphe dorsalis of patients with Alzheimer type dementia.(ABSTRACT TRUNCATED AT 250 WORDS)

The site of lesions in Miller Fisher syndrome, especially those causing ataxia, has been controversial. A 50 year old man with features of Miller Fisher syndrome in whom MRI showed enhancing lesions in the spinocerebellar tracts at the level of the lower medulla is reported. Peripheral involvement of cranial nerves was also indicated by an abnormal blink reflex and by clinical manifestations: complete external ophthalmoplegia, bilateral peripheral facial weakness, convergence disturbance, absence of Bell's phenomenon, oculocephalic, and oculovestibular reflex. Abnormal lesions on MRI disappeared and the blink reflex became normal with clinical improvement. The case is regarded as Miller Fisher-Guillain-Barré overlap syndrome, a postinfectious allergic reaction involving both peripheral nerves in the cranium and neuraxis in the spinocerebellar tract. The lesions in the spinocerebellar tracts are responsible for cerebellar ataxia in this syndrome.