No abstract available.
Dermatomyositis* ; Lung Diseases, Interstitial* ; Pneumonia

Atopic myelitis (AM) is a relatively mild form of myelitis associated with allergic diathesis, and present with predominant sensory manifestations. Lhermitte's sign has been considered as a relatively non-specific clinical sign suggesting demyelinating lesion in cervical cord. Here we report a patient with recurrent AM who presented with isolated Lhermitte's sign, both in first and second attacks. This report suggests that either the diagnosis or recurrence of AM can be frequently underdiagnosed because of its predominant sensory manifestations.
Diagnosis ; Disease Susceptibility ; Humans ; Myelitis* ; Recurrence

BACKGROUND AND PURPOSE: Primary involvement of the peripheral nerves in myotonic dystrophy type I (MyD1) is controversial. We investigated whether the involvement of peripheral nerves is a primary event of MyD1 or secondary to another complication such as diabetes mellitus (DM). METHODS: The subjects comprised 12 patients with MyD1, 12 with DM and no peripheral nerve involvement, and 25 healthy volunteers. We measured multiple excitability indices in the median motor axons. The strength-duration time constant was calculated from the duration-charge curve, the threshold electrotonus and current-threshold relationships were calculated from the sequential subthreshold current, and the recovery cycle was derived from double suprathreshold stimulation. RESULTS: The depolarizing and hyperpolarizing threshold electrotonus were significantly reduced and exhibited increased refractoriness in the MyD1 group compared with the DM and control groups. The SDTC, superexcitability, and subexcitability were not significantly altered in the MyD1 group. CONCLUSIONS: The MyD1 group exhibited a depolarized axonal membrane potential. The significant differences in peripheral nerve excitability between the MyD1 group and the DM and normal control groups suggest that peripheral neuropathy is a primary event in MyD1 rather than a secondary complication of DM.
Axons ; Diabetes Mellitus ; Humans ; Membrane Potentials ; Myotonic Dystrophy ; Peripheral Nerves ; Peripheral Nervous System Diseases ; Sarcosine ; Thiocarbamates

Gaucher's disease is an autosomal recessive disorder caused by a deficiency of beta-glucosidase (glucocerebrosidase) which results in an accumulation of glucocerebroside in various organs and tissues. Type 3 (juvenile or subacute neuro-pathic) Gaucher's disease, presented here as progressive myoclonus epilepsy, occurs more rarely than type 1 (adult or nonneuropathic) or type 2 (infantile or neuropathic) Gaucher's disease. Two patients (brother and sister) with type 3 Gaucher's disease had or was expected to develop typical features of progressive myoclonus epilepsy: myoclonus, seizures, dementia, and cerebellar dysfunction. One of them showed Gaucher cells in a liver biopsy specimen and decreased beta-glucosidase activity (14% of normal) in the cultured skin fibroblasts, which confirmed the clinical diagno-sis of type 3 Gaucher's disease.
beta-Glucosidase ; Biopsy ; Cerebellar Diseases ; Dementia ; Fibroblasts ; Gaucher Disease* ; Humans ; Liver ; Myoclonic Epilepsies, Progressive* ; Myoclonus ; Seizures ; Skin

In 1981, Bartleson et al described seven patients who experienced three to 12 episodes of headache accompanied by neurologic deficits and CSF lymphocytosis. The headache pain was severe and of a type not previously experienced by the patients. The neurologic deficits were temporary, usually lasting hours and at most 3 days. The CSF showed a predominantly lymphocytosis and increased protein. We report a patients of this syndrome. A 39 year-old woman without a history of migraine developed a right-sided severe headache with weakness in the right extremities. Three days earlier she had an upper respiratory infection. At admission she had a fever. Her speech was dysarthric. She had left homonymous hemianopsia, left-sided hemiparesis and hypoesthesia. Lumbar puncture revealed 180 WBCs (90% lymphocytes) and total protein 132 mg/dl. The symptoms and signs cleared completely within 18 hours. Three days later, she experienced left-sided severe headache followed 1 hour later by confusion after cerebral angiogram. Her language showed Wernicke's aphasia. A repeat lumbar puncture revealed similar result to first attack. The symptoms again cleared two days later. She has remained free of symptoms for 28 months. This syndrome is a self-limited condition, but the etiology of this syndrome is unknown. The monophasic nature and a high frequency of an associated fever and viral illness favor infectious origin.
Adult ; Aphasia ; Extremities ; Female ; Fever ; Headache* ; Hemianopsia ; Humans ; Hypesthesia ; Lymphocytosis* ; Migraine Disorders ; Neurologic Manifestations* ; Paresis ; Spinal Puncture

Leber's Hereditary Optic Neuropathy(LHON) is a maternally inherited disorders that occurs primarily in young males and is characterized by subacute, sequential, bilateral central visual loss, ultimately, optic atrophy. We report 2 cases of molecularly confirmed LHON which reveal 11778 and 14484 mitochondral DNA mutation, respectively but there is no family history of visual loss. So the diagnosis of LHON deserves to be considered in all crypotogenic cases of acute or subacute optic or chiasmal neuropathy. Late or early age at onset, female gender, and a negative family history should not be dissuasive.
Diagnosis ; DNA ; Female ; Humans ; Male ; Molecular Biology* ; Optic Atrophy ; Optic Nerve Diseases*

Methamphetamine is one of the most popular abuse drug in the South Korea. Intracranial hemorrhage or ischemic stroke have been well known as one of the complications with methamphetamine abuse. We experienced two cases with cerebral vasculopathy which had developed after intravenous use of methamphetamine. The first patient was a previously healthy 20-year-old woman, who was admitted because of drowsy mentality with left hemiparesis and dysarthria. The second patient was a 24-year-old man who was admitted because of seizure and left extremity weakness. They reported several intravenous administration of methamphetamine during previous months. In both case, brain CT showed cerebral infarction compatible with vascular territories. And also, carotid angiographys demonstrated that multiple intracranial vessels were involved with narrowing and stenotic nature, consisting with vasculitis in the both cases.
Administration, Intravenous ; Brain ; Cerebral Infarction* ; Dysarthria ; Extremities ; Female ; Humans ; Intracranial Hemorrhages ; Korea ; Methamphetamine* ; Paresis ; Seizures ; Stroke ; Vasculitis ; Young Adult

The radiation-induced occlusive vasculopathy has been known as the one of the side effects of radiation therary The gamma knife for stereotactic radiosurgery has been used popularly as a valuable adjunctive therapeutic option in some patients with pituitary tumors for its accuracy and effectiveness. But the long term complication of gamma knife have not been revealed until now. In spite of the remarkable improvement in radiologic technology, the side effect of radiation induced vasculaopathy remains an unsolved issue. We experienced the occlusion of intracranial portion of the internal carotid artery in one patient with pituitary adenoma, who had been treated with gamma knife radiosurgery previously.
Carotid Artery, Internal ; Cerebral Infarction* ; Humans ; Pituitary Neoplasms* ; Radiosurgery* ; Technology, Radiologic

The radiation-induced occlusive vasculopathy has been known as the one of the side effects of radiation therary The gamma knife for stereotactic radiosurgery has been used popularly as a valuable adjunctive therapeutic option in some patients with pituitary tumors for its accuracy and effectiveness. But the long term complication of gamma knife have not been revealed until now. In spite of the remarkable improvement in radiologic technology, the side effect of radiation induced vasculaopathy remains an unsolved issue. We experienced the occlusion of intracranial portion of the internal carotid artery in one patient with pituitary adenoma, who had been treated with gamma knife radiosurgery previously.
Carotid Artery, Internal ; Cerebral Infarction* ; Humans ; Pituitary Neoplasms* ; Radiosurgery* ; Technology, Radiologic

We report three patients with chronic idiopathic meningitis in whom clinical feature were headache, fever, cranial nerve palsies or CSF showed inflammatory changes. Two patients showed diffuse thickening and enhancement of meninges on CT and MRI, had biopsy-proven intracranial pachymeningitis (diffuse fibrosis with inflammatory cells). In the other patient, MRI and CT showed normal findings. Two patients including one with idiopathic hypertrophic intracranial pachymeningitis improved with corticosteroid and immunosuppressant, while one patient did with lumboperitoneal shunt.
Cranial Nerve Diseases ; Fever ; Fibrosis ; Headache ; Humans ; Magnetic Resonance Imaging ; Meninges ; Meningitis*