No abstract available.
Foot* ; Hand* ; Paresthesia*

Authors report a typical case of congenital fiber type disproportion (CFTD) with unique clinicopathologic characteristics. The patient was a 13-year-old boy who presented with weakness of lower extremities, especially proximal muscle, since his infancy. He has suffered from severe scoliosis which got worse since the age of 12. He showed mild dysarthria, high arched palate, and fish face. All routine laboratory data were within normal limits. EMG findings suggested myopathy. The muscle biopsy revealed fiber type disproportion with type 1 predominance. While most of the type 1 myofibers were atrophic or normal in size, the type 2 fibers showed universal hypertrophy. The difference of mean diameter between the larger and the smaller fibers was 27.9%. The patient's clinicopathologic settings fulfilled the criteria of CFTD.
Adolescent ; Biopsy ; Dysarthria ; Humans ; Hypertrophy ; Lower Extremity ; Male ; Muscular Diseases* ; Myopathies, Structural, Congenital* ; Palate ; Scoliosis

No abstract available.
Anterior Cruciate Ligament* ; Knee*

Multiple Sclerosis (MS) is the most common demyelinating disease affecting the central nervous system of young adults living in western countries. The clinical suspicion of demyelination, as a pathological process, is high when a young adult develops one or more neurological episodes indicating a damage to white matter tracts within the central nervous system, especially when the optic nerve, brainstem, or spinal cord is involved. The patient with episodes disseminating in time, each of which can be attributed to demyelination, requires no investigation prior to establishing the diagnosis of clinically definite MS, if the age of clinical presentation falls between 20 and 50 years, if different anatomical sites within the central nervous system have necessarily been affected on separate occasions, and if the clinical manifestation is typical for MS. MS in Asian populations is characterized by selective and dominant involvement of the optic nerve and spinal cords, as well as some incidence of brainstem lesion and symptoms. About 35~40% of cases of MS in Korea are of this optico-spinal type. Optico-spinal MS (OSMS) generally has a higher female-to-male ratio than conventional MS. OSMS is also characterized by frequent relapses, severe disability, few brain lesions on MRI, and a very lower incidence of oligoclonal bands in CSF. Neuromyelitis Optica (NMO) (Devic syndrome), which causes severe optic neuritis (ON) and longitudinally extensive transverse myelits either with a monophase or relapse-remitting pattern, is rare in Korean. NMO- IgG was reported to be helpful for the diagnosis of early-stage NMO and differential diagnoses of MS.
Asian Continental Ancestry Group ; Brain ; Brain Stem ; Central Nervous System ; Demyelinating Diseases ; Diagnosis ; Diagnosis, Differential ; Humans ; Immunoglobulin G ; Incidence ; Korea ; Magnetic Resonance Imaging ; Multiple Sclerosis* ; Neuromyelitis Optica ; Oligoclonal Bands ; Optic Nerve ; Optic Neuritis ; Recurrence ; Spinal Cord ; Young Adult

No abstract available.
Korea* ; Lung Neoplasms* ; Lung*

Paraneoplastic syndrome is a rare neurologic disorder caused not by the direct invasion or metastasis of cancer, but by the remote effects of cancer. The central- and peripheral-nervous system or neuromuscular junction area were involved in this syndrome. The pathogenesis was thought as the autoimmune disease, the result of an immunologic response to cancer and to cross-react with self-cells of the nervous system or of the neuromuscular junction, causing neuronal dam-age. Specific forms of this syndrome are often associated with specific paraneoplastic autoantibodies and cancer. The onset of neurological symptoms and detection of these antibodies often precede the diagnosis of the cancer; therefore, detection of these antibodies greatly assists the diagnosis of this syndrome and prompts investigations for the underly-ing cancer. Some paraneoplastic central neurological syndromes, such as cerebellar degeneration, limbic encephalitis, and necrotizing myelitis, were not improved by putative pathogenic autoantibodies, or by immunosuppresant or tumor removal, inspite of improvement in other peripheral neurologic syndrome, Lambert-Eaton myasthenic syndrome, neu-romyotonia, and Stiff-man syndrome. A more detailed understanding of the relationship between the cancer and the neural involvement from the molecular biological standpoint may lead to rational tumor therapy and elucidation of the mechanism of neural death. Here, major clinical forms with well-known antineuronal antibodies and specific cancers are reviewed.
Antibodies ; Autoantibodies ; Autoimmune Diseases ; Diagnosis ; Lambert-Eaton Myasthenic Syndrome ; Limbic Encephalitis ; Myelitis, Transverse ; Neoplasm Metastasis ; Nervous System ; Nervous System Diseases ; Neuromuscular Junction ; Neurons ; Paraneoplastic Syndromes* ; Small Cell Lung Carcinoma ; Stiff-Person Syndrome

In this article, I review various causes of exogenous myoglobinuria(MU) and its pathogenesis in 26 consecutive patients admitted to emergency room, Asan Medical Center and determine whether there is a relationship between concentration of urine myoglobin(Mb) and acute renal failure(ARF) as a complication of MU. Serum and urine Mb were measured by RIA using myoglobin kit (Daiichi, Inc., Tokyo, Japan). The most common disorder of MU was septic shock with hypotension, followed by crush syndrome, major arterial occlusion by thormbosis, alcohol intoxication with status epilepticus, intoxication of unidentified snake venom and drug ingestion. On the basis of this limited amount of data, there is a significant association between high concentration in urine Mb(> 300 ng/ml) and ARF(Fisher's exact test, p< 0.005). To minimize the chances of development of ARF, routine urine Mb levels should be checked on patients at risk, especially septic shock with hypotension.
Adult ; Aged ; Female ; Human ; Kidney Failure, Acute/complications ; Male ; Middle Age ; Myoglobinuria/complications/*etiology ; Retrospective Studies

BACKGROUND: Organophosphate induced delayed polyneuropathy(OIDP) by ingestion is not common, and the mechanism is not well known. In this study, we present clinical characteristics, electrophysiological findings and pathology of sural nerve in our four cases with OIDP. METHODS: Retrospectively, we reviewed 38 patients diagnosed as organophosphate intoxication at Asan Medical Center from January, 1990 to July, 1998. Among these patients we present four patients with OIDP, who received electrophysiological and pathological studies and discuss similar cases from the literature. RESULTS: OIDP occurred usually 2-4 weeks after exposure. They complained quadriplegia, paresthesia and pain mainly in distal extremities. Two patients had facial diplegia. No definite pyramidal sign was found in all patients. Elelctrophysiological study showed sensorimotor(predominantly motor) axonal polyneuropathy with marked denervation potentials in all tested muscles. Follow-up electrophysiological study after two years showed slightly increased amplitude of sensory nerve or compound motor action potentials with persistent denervation potentials in the distal muscles. Sural nerve biopsy confirmed severe axonal neuropathy with marked decrease of large and small myelinated fibers with myelin ovoids. CONCLUSIONS: OIDP was a cause of severe generalized weakness and paresthesia, decreased sensation in distal extremities after high dose organophosphate ingestion and usually occurred two to four weeks later. The prognosis was poor in patients who had severe weakness of four extremities with facial diplegia at the initial examination.
Action Potentials ; Axons ; Biopsy ; Chungcheongnam-do ; Denervation ; Eating ; Extremities ; Follow-Up Studies ; Humans ; Muscles ; Myelin Sheath ; Organophosphate Poisoning* ; Paresthesia ; Pathology ; Polyneuropathies* ; Prognosis ; Quadriplegia ; Retrospective Studies ; Sensation ; Sural Nerve

BACKGROUND: Critical illness polyneuropathy(CIP) is a recognized cause of muscle weakness and failure of weaning from a ventilator during the course of sepsis and multi-organ failure. We experienced seven patients of polyneuropathy associated with critical illness, and reviewed the cases in order to characterize the clinical features of CIP. METHODS: We evaluated seven patients who developed polyneuropathy for the first time during intensive care at the Asan Medical Center from Feb, 1998 to Mar, 1999. RESULTS: CIP occurred usually 2-8 weeks after admission to the intensive care unit. All patients received ventilator care due to severe pulmonary problems, which included pneumonia, ARDS, and empyema. Five of them had sepsis. All patients had quadriparesis prominently in the distal area, muscle atrophy, decreased tendon reflexes, and distal hypoesthesia. Electrophysiological and pathologic studies were compatible with axonal polyneuropathy. Five patients recovered from the underlying critical illness and regained their muscle power with improved findings on follow-up nerve conduction studies. CONCLUSIONS: Critical illness should be considered as a cause of polyneuropathy in severely ill patients, especially if associated with sepsis. After recovery from illness, motor weakness as well as electrophysiological findings improved. Failure of weaning from a ventilator may be more affected by pre-existing cardiopulmonary problems than CIP.
Axons ; Chungcheongnam-do ; Critical Illness* ; Empyema ; Follow-Up Studies ; Humans ; Hypesthesia ; Critical Care ; Intensive Care Units ; Muscle Weakness ; Muscular Atrophy ; Neural Conduction ; Pneumonia ; Polyneuropathies* ; Quadriplegia ; Reflex, Stretch ; Sepsis ; Ventilator Weaning ; Ventilators, Mechanical ; Weaning

BACKGROUND: Critical illness polyneuropathy(CIP) is a recognized cause of muscle weakness and failure of weaning from a ventilator during the course of sepsis and multi-organ failure. We experienced seven patients of polyneuropathy associated with critical illness, and reviewed the cases in order to characterize the clinical features of CIP. METHODS: We evaluated seven patients who developed polyneuropathy for the first time during intensive care at the Asan Medical Center from Feb, 1998 to Mar, 1999. RESULTS: CIP occurred usually 2-8 weeks after admission to the intensive care unit. All patients received ventilator care due to severe pulmonary problems, which included pneumonia, ARDS, and empyema. Five of them had sepsis. All patients had quadriparesis prominently in the distal area, muscle atrophy, decreased tendon reflexes, and distal hypoesthesia. Electrophysiological and pathologic studies were compatible with axonal polyneuropathy. Five patients recovered from the underlying critical illness and regained their muscle power with improved findings on follow-up nerve conduction studies. CONCLUSIONS: Critical illness should be considered as a cause of polyneuropathy in severely ill patients, especially if associated with sepsis. After recovery from illness, motor weakness as well as electrophysiological findings improved. Failure of weaning from a ventilator may be more affected by pre-existing cardiopulmonary problems than CIP.
Axons ; Chungcheongnam-do ; Critical Illness* ; Empyema ; Follow-Up Studies ; Humans ; Hypesthesia ; Critical Care ; Intensive Care Units ; Muscle Weakness ; Muscular Atrophy ; Neural Conduction ; Pneumonia ; Polyneuropathies* ; Quadriplegia ; Reflex, Stretch ; Sepsis ; Ventilator Weaning ; Ventilators, Mechanical ; Weaning