The upper chest wall does not grow properly in children with spinal muscular atrophy (SMA) with paradoxical breathing. This suggests that long-term inability to take a deep breath in developing children may result in underdevelopment of the upper chest wall. In addition, a rapid and paradoxical breathing pattern is frequently observed in children with severe cerebral palsy (CP), which often corresponds to the underdevelopment of the upper chest wall. The present study is designed to evaluate the ratio of the upper to lower chest wall in children with severe spastic quadriplegic CP, compared with normal children. We compared normal children with children that had spastic quadriplegic CP who did not have kyphosis or scoliosis. Test subjects were matched in terms of age, height, and weight. The diameters of upper chest (D(apex)) and of lower chest (D(base)) were measured on the anteroposterior (AP) view of a chest X-ray and the D(apex) to D(base) ratio was calculated. In selected cases the forced vital capacity (FVC) was measured using a Wright Respirometer. The D(apex) to D(base) ratio was significantly lower in the CP group than in the control group (p < 0.001). The ratio increased linearly with age (p < 0.001) in both CP (R = 0.372) and control groups (R = 0.477). The FVC/preFVC showed significant correlation with the D(apex) to D(base) ratio (R = 0.542, p < 0.01). The results of this study suggest a deviation of optimal chest wall structure in children with spastic quadriplegic CP.
X-Rays ; Vital Capacity ; Time Factors ; Thoracic Wall ; Thoracic Cavity ; Respiratory Tract Diseases/*pathology/physiopathology ; *Respiration ; Quadriplegia/*pathology ; Muscle Spasticity/*pathology ; Male ; Lung/pathology ; Humans ; Forced Expiratory Volume ; Female ; Child, Preschool ; Child ; Cerebral Palsy/*pathology ; Case-Control Studies

Critical illness myopathy (CIM) is characterized by generalized flaccid weakness occurring in patients with severe systemic illness. A 27-year-old woman presented with loss of consciousness and dyspnea. The brain MRI suggested Wernicke's encephalopathy. During treatment, she experienced progressive quadriparesis and respiratory muscle weakness, which required the mechanical ventilation. The muscle pathology of vastus lateralis revealed type 2 muscle fiber atrophy on histochemical stain and selective loss of myosin on electron microscopic study, and suggested clinical diagnosis of critical illness myopathy.
Adult ; Atrophy ; Brain ; Critical Illness* ; Diagnosis ; Dyspnea ; Female ; Humans ; Magnetic Resonance Imaging ; Muscular Diseases* ; Myosins ; Pathology ; Quadriceps Muscle ; Quadriplegia ; Respiration, Artificial ; Respiratory Muscles ; Unconsciousness ; Wernicke Encephalopathy

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

Intramedullary spinal cord abscess is rare condition, and that, the diagnosis of an abscess is rarely established before operative interference is indicated. A case of spinal cord abscess is reported upon 42 year-old male who was admitted to this hospital because of severe pain in the posterior lower neck and weakness in all 4 extremities. The patient have no history of infection or operation in recent past. On admission the subject was in state of quadriparesis, and the superficial sensory impairement was up to the level of T1 3 spinal dermatome. In an early course of the illness the motor weakness had started to appear in the left arm and the impairement of pain and temperature sensations in the right leg, indicating a picture of Brown-Sequard's syndrome. Radiographic examinations disclosed a localized enlargement of lower cervical canal with a widening of interpedicular distance and showed a defect and obstruction of dye column at C6 spinal level, which were suggestive of picture of intramedullary tumor. Operation the exposed spinal laminae and dura showed nothing abnormal, but the dura mater was under the grerat pressure. Opening the dura the spinal cord was swollen, tense and nonpulsatile with dusky red discoloration on a surface of the cord. Pus contents were obtained by needle aspiration and through a myelotomy opening the contents were evacuated completely. Culture of the pus contents produced no organisms. Pathology reported a nonspecific suppurative inflammation on the biopsy. The surgical treatment was combined with the administration of antibiotics, in full theapeutic doses and steroid postoperatively. Two weeks after the surgery he showed significant recovery from the neurological dysfunctions in his legs.
Abscess* ; Adult ; Anti-Bacterial Agents ; Arm ; Biopsy ; Brown-Sequard Syndrome ; Diagnosis ; Dura Mater ; Extremities ; Humans ; Inflammation ; Leg ; Male ; Neck ; Needles ; Pathology ; Quadriplegia ; Sensation ; Spinal Cord ; Suppuration ; Transcutaneous Electric Nerve Stimulation