Recurrent Atopic Myelitis Presenting as an Isolated Lhermitte's Sign / 대한임상신경생리학회지

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.

Acute Exacerbation of Interstitial Lung Disease in Newly Diagnosed Probable Dermatomyositis / 대한임상신경생리학회지

No abstract available.

Peripheral Nerve Axon Involvement in Myotonic Dystrophy Type 1, Measured Using the Automated Nerve Excitability Test

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.

Two Cases of Progressive Myoclonus Epilepsy due to Gaucher's Disease, Type 3

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.

The Transient Syndrome of Headache with Neurologic Deficits and CSF Lymphocytosis

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.

2 Cases of Leber's Hereditory Optic Neuropathy Confirmed by Molecular Genetics

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.

Cerebral infarctions associated with intravenous use of methamphetamine

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.

A case of cerebral infarction caused by occlusive vasculopathy after previous gamma knife radiosurgery for pituitary adenoma

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.

A case of cerebral infarction caused by occlusive vasculopathy after previous gamma knife radiosurgery for pituitary adenoma

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.

Chronic Idiopathic Meningitis

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.

Tarsal Tunnel Syndrome: Electrophysiological Study Using Interdigital Nerve Condition Test

BACKGROUND AND OBJECTIVES: Tarsal tunnel syndrome is a rare compressive neuropathy. Reported cases of the tarsal tunnel syndrome in Korea were diagnosed by conventional nerve conduction and EMG studies, so their sensitivity was low and the differentiation from similar disorder such as lumbosacral radiculopathy was difficult. We report the usefulness of the interdigital nerve conduction study by using needle recording electrodes in the tarsal tunnel syndrome. METHODS: Sensory compound nerve action potentials were recorded orthodromically using the near-nerve needle and signal averaging techniques. Active and reference recording electrodes were inserted behind the medial malleolus above the flexor retinaculum. When the great toe was contracting minimally with less than 3mA for a stimulus of 0.05ms duration, the needle was considered to be adequately positioned. The first and fifth digital nerves were stimulated with ring electrodes and various interdigital nerves with interdigital stimulating surface electrodes which were specially designed. Averaged sensory action potential were recorded 3 times and superimposed after 64 stimuli per each time of recording. Amplitude of sensory nerve action potential, sensory nerve conduction velocity, and the presence of the temporal dispersion were checked. The results were as follow, 1. The causes of the 775 were trauma in 3 cases, sprain in 2 cases, unknown in 5 cases. 2. Associated disease were radiculopathy in 6 cases, rheumatoid arthritis In 1 case, carpal tunnel syndrome In 1 case. 3. Clinical findings included pain on the in 5 cases, tingling sensation in 4 cases, hyperesthesia in 4 cases, numbness in 2 cases and Tinnel's sign in 2 4. Five cases showed absent potential, 5 cases low amplitude, 4 cases dispersion, 2 cases slow nerve conduction velocity and 2 cases low amplitude and slow nerve conduction velocity by Interdigital nerve condution study. 5. Five lumbosacral radiculopathy without tarsal tunnel syndrome showed normal results in interdigital nerve conduction test. We concluded that interdigital nerve conduction test using needle recording electrode is useful methods of diagnosing tarsal tunnel syndrome as well as differentiating to other disorders having similar symptoms.