Rinsho shinkeigaku = Clinical neurology

Publisher: Nihon Shinkei Gakkai

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Website Rinsho Shinkeigaku / Clinical Neurology website
Other titles Rinshō shinkeigaku, Clinical neurology
ISSN 0009-918X
OCLC 4301256
Material type Periodical
Document type Journal / Magazine / Newspaper

Publications in this journal

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    ABSTRACT: A 73-year-old woman was admitted to our hospital due to a decreased conscious level and a high fever. Six days before her admission, she felt transient numbness in her right lower limb. Brain MRI taken by her local doctor revealed only right parietal cortex lesions. She was diagnosed with transient ischemic attack and started on anti-platelet therapy. One day before her admission, she became drowsy, and left-side weakness developed. She was admitted to a community hospital for treating stroke. On the next day, she was referred to our hospital because of a high fever. Our brain MRI showed new lesions in her right temporal lobe. She had no stroke risk factors, and embolic sources were not detected. Cerebrospinal fluid analysis detected herpes simplex virus DNA. She was diagnosed with herpes simplex encephalitis (HSE). HSE is common encephalitis which develops fever, headache and alteration in mental status. It often involves temporal lobe, but extratemporal lesions alone are not uncommon. Diffusion-weighted images (DWI) of brain are of importance to differentiate HSE from stroke. When it is questionable to diagnose with stroke for patients with cerebral cortex lesions, they must be monitored with close observation. There is the possibility of initial presentation of HSE in that situation even if patients have no typical symptoms.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: We report a 43-year-old man experienced numbness in the distal portion of both legs, which progressed over following two months. Neurological examination showed hypesthesia and muscle weakness in the distal portion of both legs. No abnormal findings were seen on blood test and whole-body contrast enhanced computed tomography (CT). Histopathological findings of the sural nerve and the peroneus brevis muscle showed decreased myelinated nerve fibers with scattered myelin ovoids, vascular occlusion in the epineurium, and inflammatory cell around the arteriole in the muscle bundle. These findings suggested falling in the category as non-systemic vasculitic neuropathy (NSVN). 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) revealed the increase of FDG uptake in the rectum. Inflammatory cell infiltration was found around the arteriole with fibrinoid necrosis in the histopathological specimen of the rectal mucosal biopsy. This result represented the diagnosis as systemic vasculitis. The diagnosis of NSVN may depend on the sensitivity of diagnostic procedure, and 18F-FDG PET CT might be a useful tool to detect small or medium-sized vasculitis.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: A 46-year-old woman presenting to the Department of Hematology with swelling of the mandibular lymph nodes was diagnosed with angioimmunoblastic T-cell lymphoma (AITL) in June 2013. The patient went into complete remission in December 2013 with chemotherapy; however, she was re-evaluated because of mental confusion during May 2014. In addition to the memory disturbances, elevated cerebrospinal fluid cell count and protein were noted. Fluid attenuated inversion recovery cranial magnetic resonance imaging revealed multiple hyperintense areas in both the mammillary bodies and thalamus accompanied by contrast-enhancing in some areas. The diagnosis of recurrent AITL was made based on the brain biopsy. AITL recurrence in the cranium should be considered in patients exhibiting central nervous system symptoms although such recurrences have not been reported previously.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: A 64-year-old man developed right arm weakness and dysarthria, and was admitted to our hospital. Diffusion-weighted magnetic resonance imaging of the brain showed a high intensity area in the frontal lobe. T2*-weighted images showed multiple spotty low intensity lesions in bilateral cerebral hemispheres, mimicking cerebral microbleeds. Cerebral angiography showed multiple aneurysms in the anterior, middle, posterior cerebral arteries and cerebellar arteries. Transthoracic echocardiography revealed a floating structure in the left atrial chamber, indicating cardiac myxoma. We diagnosed cardioembolic ischemic stroke due to left atrial myxoma. Cardiac surgery for excision of a left atrial myxoma was performed on the 3rd hospital day. Multiple aneurysms should be taken into account for differential diagnosis in patients with cardiac myxoma and with atypical spotty low intensity on T2*-weighted images.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: A 50-year-old man was diagnosed with Parkinson’s disease at the age of 43 years. The patient was hospitalized because of frozen gait and tendency to fall and showed abnormal postures of marked antecollis and stooped posture. The angle of the abnormal posture dramatically fluctuated, and the fluctuation coincided with the motor symptoms over the course of a 24-hour period. Treatment with rotigotine was started, and diurnal fluctuations in the gait disorder and abnormal posture were recorded. The time recorded in the Timed Up & Go test decreased after treatment with rotigotine in a dose-dependent manner, particularly early in the morning. The angle of the abnormal posture improved with a 4 mg/24 hr dose of rotigotine but worsened with a dose of 8 mg/24 hr. Because abnormal posture is a known side effect of dopamine agonists such as rotigotine, it is possible that high-dose rotigotine worsened the abnormal posture, whereas the low-dose improved the abnormal posture because of undertreatment. This case highlights the importance of observing the diurnal fluctuation in abnormal posture for developing a strategy for the treatment of Parkinson’s disease.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: A 63-year-old man was admitted to our hospital because of convulsive seizures. Radiological examinations revealed cerebral venous sinus thrombosis in the anterior part of the superior sagittal sinus. He had marked hyperhomocysteinemia (93.5 nmol/ml) due to combined deficiencies of folate and vitamin B12. He was T/T homozygous for methylene tetrahydrofolate reductase C677T polymorphism. He received a supplement therapy of vitamins. First, he was administered folate orally. After 3 months, the serum level of homocysteine decreased to 22.6 nmol/ml (an 86% reduction), but was still above the normal level. Next, an additional supplement therapy of vitamin B12 lowered the homocysteine level to normal (12.3 nmol/ml) after 4 months. These results showed that the increase of homocysteine levels in this patient was mainly caused by the deficiency of folate. Additionally, acquired risk factors like vitamin deficiencies increased the level of serum homocysteine to almost 100 nmol/ml.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: A 65-year-old man with Takayasu arteritis in a stable condition was admitted to our hospital because of rapid progressive dementia. Brain FLAIR/T2-weighted magnetic resonance images revealed high signal intensity in the diffuse subcortical white matter. John Cunningham virus (JCV) genome in cerebrospinal fluid was detected by polymerase chain reaction. Finally, progressive multifocal leukoencephalopathy was diagnosed definitely by brain biopsy. In addition, the patient was found to be complicated by chronic/smoldering adult T-cell leukemia/lymphoma. The administration of mefloquine with mirtazapine was early started within two months after the onset. However, the combination treatment led to no improvement in symptoms and lesion size. The patient died six months after the onset. Therefore, this case suggested that both of HTLV-I infection and B cell abnormalities due to Takasasu arteritis impaired the therapeutic effect.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: A 56-year-old man, who presented with 6 years history of difficulty in walking, was diagnosed as having vascular parkinsonism on the basis of the clinical findings of parkinsonism, pyramidal sign and the brain MRI findings of multiple lacunar infarction. Although he did not have hypertension, he had hyperhomocysteinemia and homozygous methylenetetrahydrofolate reductase (MTHFR) gene variant (C677T) as risk factors for ischemic stroke. Recent studies have shown that hyperhomocysteinemia and MTHFR gene variant are associated with small-vessel disease, suggesting that these risk factors may underlie vascular parkinsonism, particularly in patients lacking hypertension and in those with a relatively younger age at onset of this disease.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: We report the case of a 17-year-old woman with paroxysmal sympathetic storm (PSS), which was successfully treated with clonidine hydrochloride. The patient was hospitalized for acute disseminated encephalomyelitis in June 2006. Dysphagia led to severe aspiration pneumonia in September 2006, and she suffered cardiopulmonary arrest. She survived but had severe brain damage, with her brain MRI showing diffuse hypoxic encephalopathy. From October 2006, she had several episodes of profound tachypnea (> 60/min), tachycardia (160 to 170 beats/min), hypertension (> 140 mmHg), hyperthermia (39°C), and decerebrate posturing. During the attacks, the levels of catecholamines in the patient's blood and urine were markedly elevated. Accordingly, a diagnosis of PSS associated with hypoxic encephalopathy was made. Her PSS clearly improved after the administration of clonidine hydrochloride (900 μg/day). This case suggests that clonidine hydrochloride, an α2 blocker, may be one therapeutic option for PSS.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: We report the case of a 62-year-old man who presented with malignant lymphoma as recurrent multiple cranial nerve palsy after spontaneous regression of oculomotor nerve palsy. He developed ptosis and diplopia due to right oculomotor nerve palsy. Brain MRI/MRA showed no abnormality, and he recovered with conservative medical management. Three months later, he showed diplopia due to right abducens nerve palsy and facial pain and trigeminal sensory loss. Neurological examination revealed multiple cranial nerve palsy involved cranial nerve III, V, IX, and X of the right side. Serum soluble interleukin-2 receptor levels were normal, and cerebrospinal fluid examination was unremarkable. Steroid and subsequent intravenous immunoglobulin therapy didn't improve his symptoms. Six weeks after his admission, he showed rapid enlargement of the cervical lymph node and the right tonsil, and post-contrast T1-weighted MRI showed enlargement and enhancement of the left infraorbital nerve, the bilateral cavernous sinus, the bilateral facial nerves, and the left trigeminal nerve. The histopathologic examination of the tonsil biopsy revealed diffuse large B cell lymphoma. The cause of these symptoms was thought to be infiltrating the cavernous sinus, and adjacent nerves. Spontaneous regression of malignant lymphoma is an exceptional event, but this possibility should be considered so as to the correct diagnosis and proper treatment.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: A 61-year-old alcoholic man was admitted to our hospital because of disturbance of consciousness. He also exhibited external ophthalmoplegia, diplopia and mild rigidity, but tendon reflex was normal. On brain MRI, diffusion weighted images (DWI) and apparent diffusion coefficient (ADC) map depicted high intensity in the splenium of the corpus callosum. DWI showed high intensity, but ADC map depicted iso-intensity in bilateral precentral gyri. Marchiafava-Bignami disease (MBD) was diagnosed. After intravenous drip of vitamin, his symptoms improved rapidly and the abnormal MRI findings in the splenium of the corpus callosum and bilateral precentral gyri disappeared gradually. MBD is pathologically characterized by demyelination and necrosis in the corpus callosum, which are generally caused by cytotoxic edema. Our case suggests that vasogenic edema may occur at the early stage of the MBD.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: A 71-year-old woman was admitted to our hospital complaining of left orbital pain, headache, diplopia and left-sided ptosis, which she had suffered for two months. On examination, the patient had loss of visual acuity, left-sided ptosis, lateral gaze disturbance, and was diagnosed as having left orbital apex syndrome. An abnormal signal to the left orbital cone was detected on MRI. Serum β-D-glucan was increased, and serum Aspergillus antigen and antibody were both positive. Although antifungal drugs (voriconazole and liposomal amphotericin B) were administered, the symptoms deteriorated. The patient then underwent optic nerve decompression surgery and was treated with intravenous methylprednisolone, which gradually improved the patient's symptoms, Aspergillus hyphae were confirmed by pathological examination. To obtain good prognosis for patients with orbital apex syndrome associated with Aspergillus infection, optic nerve decompression surgery should be considered.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: A 60-year-old woman had transient weakness of the legs and urinary retention for six weeks. She presented with a gait disorder and was admitted to the hospital. She showed symptoms of paraplegia, tingling in the lower extremities, dysuria. She underwent an MRI, and T2-weighted images showed an enlargement of the thoracolumbar spinal cord and high intensity signal from Th3 to the medullary cone, and a contrast-enhanced T1-weighted image showed abnormal vessels anterior to the spine cord. Cervical and spinal angiography documented a dural arteriovenous fistula at the craniocervical junction that was fed by the right vertebral artery and the right ascending pharyngeal arteries and drained into the perimedullary veins. Surgical therapy improved her symptoms and MRI images. Craniocervical junction DAVF with thoracic-medullary cones lesion is rare.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: We report a patient of 68-year-old woman who developed numbness of feet in 2008. Ataxic gait disturbance, truncal ataxia, muscle weakness of lower limbs have gradually appeared and she couldn't walk without assistance in 2013. Her cognitive function declined subacutely in 2014. When she was admitted to our hospital, it was difficult to fully evaluate her neurological symptoms and cognitive function. The tendon reflex were absent and Babinski reflex showed positive in both sides of the lower limbs. Diffusion weighted image of MRI showed high intensity in cerebrocortical area, and variation P102L prion protein gene mutation was detected. We diagnosed her with Gerstmann-Sträussler-Scheinker (GSS) disease. Cerebellar symptom such as ataxic gait occurs as the initial manifestation in 90% of patients with GSS disease. Her initial symptom was numbness of lower limbs and cerebellar symptom gradually appeared during the course of disease. In addition, her cognitive function declined six years after the onset. This case presented atypical clinical course as described above. Consequently, it led to diagnostic delay in GSS disease.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: Here, we present a case of right eyelid drooping in a 79-year-old man. Neurological examination revealed ptosis of the right eye without severe painful eyelid swelling and redness. An ocular motility examination of the right eye revealed upward limitation and downward overshoot. The results of routine blood examinations were within normal limits, and no autoantibodies were detected. Orbital magnetic resonance images revealed mild right eyelid swelling and lacrimal gland enlargement, indicating orbital inflammation. The ocular discharge was positive for Staphylococcus hominis by culture and the patient was diagnosed as having acute dacryoadenitis. Treatment with topical and systemic administration of antibiotics rapidly improved symptoms. Ocular infection is not usually suspected in the absence of local severe painful swelling and redness, and painless acute dacryoadenitis presenting as ophthalmoplegia and ptosis may be misdiagnosed. Orbital inflammation may rapidly progress to orbital cellulitis with treatment delay, which may also lead to aggravation of ophthalmic prognosis. Therefore, neurologists should be aware of the possibility of acute dacryoadenitis occurring without the local severe inflammatory findings mimicking neurological diseases, and acute dacryoadenitis should be considered in patients with ophthalmoplegia even in the absence of severe painful eyelid swelling and redness.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: We report the case of a 73-year-old woman presenting with hypersomnia and loss of appetite. She suffered from diabetic nephropathy without receiving dialysis, in addition to hypertension, which was well controlled without marked fluctuation. There were no objective neurological findings. Her laboratory findings showed renal failure with 3.7 mg/dl of serum creatinine and decreased serum sodium and potassium. Brain magnetic resonance imaging (MRI) showed posterior reversible encephalopathy syndrome (PRES) with vasogenic edema, which was distributed in the dorsal midbrain, medial thalamus, and hypothalamus. After we addressed the electrolyte imbalance and dehydration, her symptoms and MRI findings gradually improved, but faint high signals on MRI were still present 3 months later. Orexin in the cerebrospinal fluid was decreased on admission, but improved 6 months later. We diagnosed uremic encephalopathy with atypical form PRES showing functional disturbance of the hypothalamus.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: The patient is a 72-year-old Japanese woman. Seven years prior to admission, multiple nodules in her left lung were found. Bronchoscopic biopsy of the nodules did not provide a confirmative diagnosis, and probable diagnosis of cryptococcosis was made. Follow-up CT scan of the chest revealed reduction in size of the lung nodules. She was admitted to our hospital due to progressive cognitive impairment and difficulty in walking that lasted for 5 months. On admission, athetotic involuntary movement was observed in her lower extremities, predominantly in the right side. Blood and cerebrospinal fluid culture of the patient were positive for Cryptococcus neoformans. Antifungal drugs resolved the cognitive impairment, the difficulty in walking, and the involuntary movement. We assessed the cognitive impairment and observed the clinical improvement of the patient, with the use of neuropsychological examinations. To our knowledge, there has been only a few reported case of cryptococcal meningoencephalitis presenting with treatable cognitive impairment and involuntary movement.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology
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    ABSTRACT: We report the case of a patient with bilateral blephaloptosis associated with a recurrence of diffuse large B-cell lymphoma (DLBCL) in the midbrain. A 70-year-old man experienced acute onset bilateral blephaloptosis; the other ocular movements, except for medial rectus muscle in the right eye, were not impaired. Pupils were isocoric and light reflexes were prompt. Other cranial nerves were intact. Gadolinium-enhanced MRI revealed abnormal enhancement in the midbrain and peri-ventricular regions. FDG-PET revealed an abnormal positive signal in the midbrain, similar to the distribution seen in the MRI scan. Cytology of the cerebrospinal fluid showed large atypical lymphocytes. These findings suggest that the recurrence of DLBCL in the midbrain caused bilateral blephaloptosis. The oculomotor fascicle is localized in the paramedian ventral midbrain. The fascicular fibers are divided topographically into four regions; the lateral, medial, rostral and caudal regions. In three-dimensional arrangement of the oculomotor fascicle, the fibers to the levator palpebrae superioris muscle and medial rectus muscles are located adjacently in caudal regions. Thus, we speculate that recurrence of DLBCL in the midbrain involving the right oculomotor fascicle caused blephaloptosis in the right eye, and then, infiltration of DLBCL to the left oculomotor fascicle subsequently caused blephaloptosis in the left eye. This is a valuable case to be documented in which neurological site of lesions consistent with those are found in radiological study.
    Preview · Article · Jan 2016 · Rinsho shinkeigaku = Clinical neurology