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*

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

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

Multiple sclerosis (MS) is the most common demyelinating disease affecting the central nervous system of young adults living in the western world. MS should be strongly suspected when a young adult develops one or more neurological episodes consistent with damage to white matter within the central nervous system (CNS), especially when these affect the optic nerves, brainstem, or spinal cord. The patient with relapses, each of which can be attributed to demyelination in the CNS, requires no investigation prior to establishing the diagnosis of clinically definite MS. For a diagnosis of MS, separate anatomical sites within the CNS must have been affected on different occasions, typically three. MS in Asian populations is characterized by the selective and dominant involvement of the optic nerve and spinal cord with some incidence of brainstem lesions. 35-40% of MS cases in Korea are of this optico-spinal type with or without brainstem lesions. Reported cases of neuromyelitis optica spectrum disease (NMOSD), causing severe optic neuritis (ON) and/or longitudinally extensive transverse myelitis, either monophase or with a relapse-remitting pattern, some of which were diagnosed previously as the optico-spinal form of MS in Asia, have increased annually in Korea with the development of the NMO-IgG or aquaporin4-antibody detecting technique. NMO-IgG detection is very important in the diagnosis of early stage of NMOSD and the differentiation of MS and other demyelinating disease. Many new convenient oral drugs or very potent intravenous monoclonal antibodies for targeting VLA-4, CD20, and CD52 may decrease the annual relapse rate and burden of brain-spinal cord lesionsin MS.
Antibodies, Monoclonal ; Asia ; Asian Continental Ancestry Group ; Brain Stem ; Central Nervous System ; Demyelinating Diseases ; Humans ; Incidence ; Integrin alpha4beta1 ; Korea ; Multiple Sclerosis ; Myelitis, Transverse ; Neuromyelitis Optica ; Optic Nerve ; Optic Neuritis ; Recurrence ; Spinal Cord ; Western World ; Young Adult

The diagnosis of a cervical cord infarction could be made with clinical manifestations and a neurological examination. The MRI will make a disgnosis. The nerve conduction study and electromyogram were conducted for a better comprehension of the flaccid hands weakness and paraplegia. The nerve conduction study, performed two weeks after stroke, did not show any compound motor action potentials (CMAPs) of the abductor pollicis brevis (APB), abductor digiti quinti (ADQ), or the extensor digitorum communis muscles. Late responses (H-reflexes and F-waves) were not evoked in the lower extremities. The denervation potentials were detected in the APB and ADQ. The diffuse anterior horn cell lesion of the C7-T1 spinal cord did not cause CMAPs in any of the hand muscles. A lack of late-responses in the lower extremity of the cervical cord infarct suggests that the suprasegmental region of the descending tract to the anterior horn cells of the lumbar spinal cord must be needed for the production of a late-response by a signal transduct-ing neurotransmitters or long loop facilitations.
Action Potentials ; Anterior Horn Cells ; Comprehension ; Denervation ; Diagnosis ; H-Reflex ; Hand ; Infarction ; Lower Extremity ; Magnetic Resonance Imaging ; Muscles ; Neural Conduction ; Neurologic Examination ; Neurotransmitter Agents ; Paraplegia ; Spinal Cord* ; Stroke

Acute brachial neuropathy (ABN) is a rare disease, characterized by an acute or subacute onset of pain followed by weakness of shoulder or arm muscles without trauma or traction injury. So the diagnosis of this clinical entity is not easy. The purpose of this study was to analyze retrospectively the ABN in 14 cases focusing on the clinical profile and to evaluate the effectiveness of electrophysiologic study in diagnosis of ABN with a new result helpful in localizing a brachial plexus disorder. The most helpful electrophysiologic data of ABN in my patients seemed to be abnormalities of low amplitude, abnormal right to left difference of compound motor action potentials (CMAPs) and sensory nerve action potentials (SNAPs) in axillary nerve, ulnar or median nerves. Results of nerve conduction velocity, terminal and F-wave latency were not as useful. But the electromyogram was most helpful in localization of upper or lower plexus lesions and cervical radiculopathy. The most striking clinical feature of ABN was the rapid onset of pain followed by the development of muscle weakness of shoulder girdle after a variable period or within four days. In contrast to other reports, intrinsic hand muscle weakness was observed in 3 cases with sensory changes in ulnar nerve distribution. The cervical radiculopathies (C5-C7 roots) were simultaneously combined with ipsilateral axillary neuropathy in 3 cases. In this study, decreased amplitude, abnormal right to left difference of SNAPs and CMAPs, and neurogenic EMG findings with normal data of NCV, terminal and F-wave latencies suggest that the pathology of ABN might not be a demyelinating process, but axonopathy.
Adult ; Aged ; Brachial Plexus Neuritis/complications/diagnosis/*physiopathology ; Electromyography ; Electrophysiology ; Evoked Potentials ; Female ; Human ; Male ; Middle Age ; Muscle Weakness/etiology ; *Neural Conduction ; Prognosis ; Retrospective Studies ; Sensation Disorders/etiology ; Skin Temperature ; Ulnar Nerve/physiopathology

Radiation-induced neuropathy is a rare but well recognized clinical entity although peripheral nerves are considered to be relatively resistant to irradiation. We report three patients with radiation-induced lumbosacral plexopathy, whose characteristic clinical and electrophysiological features can be summarized as follows: 1) 55-58 year old women who were previously treated with radiotherapy for uterine cervix carcinoma: 2) the latent period is to 10 from 13 years: 3)predominantly motor involvement with slowly progressive paraparesis, asymmetrical onset and worse in distal muscle group: 4) painless at onset, with variable degree of sensorv changes: 5)decreased or absent knee and ankle jerks :6)axonal damage in electrophysiologic study: 7)frequent myokymic discharges. Myokymic discharges occur in bursts at regular rate of 0.1 to 8 Hz. In myokymic bursts with more spikes, interburst interval is longer but interspike Interval is shorter.
Ankle ; Cervix Uteri ; Female ; Humans ; Knee ; Paraparesis ; Peripheral Nerves ; Radiotherapy

BACKGROUND: The clinical and laboratory findings of five patients with tonic pupil (TP) and neuropathy were reviewed for the comprehension of pathogenesis of TP in neuropathy. METHODS: Immunological and nerve conduction studies (NCS) were performed in three patients with Sjogren's syndrome (SS), Miller-Fisher syndrome (MFS), and Adie's syndrome. RESULTS: Upon initial examination, there were no definite sicca syndromes in patients of SS, despite intolerable sensory symptoms. The TP in MFS was improved after intravenous immunoglobulin. Of the cranial neu-ropathies, trigeminal sensory neuropathy was frequent finding. Deep tendon reflexes were absent in all five patients.Absent sensory nerve action potentials and prolonged R1 and R2 of the blink reflex were detected in two SS patients with syncope and asymmetric sensory loss. CONCLUSIONS: These findings in SS patients implicated the possibility of a selective lesion at the level of the dorsal root- or trigeminal- or autonomic- ganglions complicating the TP. In view of the sensory ataxia, opthalmoplegia, areflexia, slow and decreased sensory NCS in the extremity and prolonged R1 and R2, a demyelinating process of postganglionic parasympathetic nerves were suspected to be the cause of the tonic pupil in MFS. Adie's syndrome along with flushing of the left side of the face and chest after exercise, suggested segmental postganglionic lesions of the sympathetic and parasympathetic peripheral nervous systems. In patients with complicat-ing TP and asymmetric progressive sensory neuropathy, the SS has to be considered even if the patient denies the pres-ence of sicca symptoms at first and SS-A/SS-B autoantibody is negative.
Action Potentials ; Adie Syndrome ; Ataxia ; Autoantibodies ; Blinking ; Comprehension ; Extremities ; Flushing ; Ganglion Cysts ; Humans ; Immunoglobulins ; Miller Fisher Syndrome ; Neural Conduction ; Peripheral Nervous System ; Reflex, Stretch ; Sjogren's Syndrome ; Syncope ; Thorax ; Tonic Pupil*