Central pontine myelinolysis (CPM) classically occurs in alcoholics, malnourished individuals, chronic liver diseases, and rapid correction of hyponatremia. This report presents locked-in syndrome due to CPM following rapid correction of hyponatremia. A 44-year-old male came to the hospital due to a short period of loss of consciousness. He was alert and had no focal neurological abnormalities at admission. The serum sodium concentration was 118 mEq/L and was corrected to 134 mEq/L in the first 18 hours. One week later, progressive weakness in limbs developed and he progressed to a complete quadriplegic state and bulbar palsy, with only eye blinking preserved. Brain magnetic resonance imaging revealed a characteristic hyperintense signal abnormality in both pons, so he was diagnosed to locked-in syndrome caused by CPM. The patient gradually improved following continuous intensive rehabilitation for more than 2 years. He was able to move all joint muscles against gravity in generally and he could gait under supervision.
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Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive disorder characterized by dysmyelination of the central nervous system (CNS) caused by mutations in the proteolipid protein (PLP) gene. PLP is located at Xq22 and its mutation result in abnormal expression or production of PLP, the most abundant protein in CNS myelin. We present a case of PMD in the 7-year-old boy with nystagmus, ataxia, spastic quadriplegia and severe psychomotor delay. His brain MRI revealed totally dysmyelinated white matter
involving entire supratentorial region, atrophic change, and overaccumulation of the iron in both basal ganglia. He also showed soft-tissue contractures of the hip adductors, associated hip dislocations and equinovarus foot deformities due to severe spasticity of lower extremities. Orthopaedic surgery was performed on both hips. Antispastic medication and physical therapy were maintained for reduction of spasticity. We report this case with the review of literatures. (J Korean Acad Rehab Med 2002; 26: 108-112)
Lissencephaly results from a neuromigrational arrest during first and second trimester of pregnancy and shows hypotonia, marked mental retardation and seizure as predominant features. Myelination is a perinatal process and co-occurence of migrational disorder with myelination disorder is rare. We report a 17-month-old male with mixed quadriplegia and mental retardation with type 1 lissencephaly and dysmyelination of cerebral white matter diagnosed by magnetic resonance imaging.
Fumarase catalyzes the conversion of fumarate to malate in the Krebs cycle. Fumarase deficiency is a rare inborn error of metabolism and is inherited in an autosomal recessive manner. It causes mitochondrial encephalomyopathy. The symptom is characterized by developmental delay and hypotonia.
We report here a case of a 32-month-old child who was initially refered because of spastic quadriplegia, delayed development and poor feeding.
Objective: To evaluate the effect of different kind of abdominal corsets on pulmonary function and energy consumption in the patients with cervical spinal cord injury.
Method: Five subjects with quadriplegia due to cervical spinal cord injury were enrolled. A quantitative evaluation of vital capacity, tidal volume, and oxygen consumption was done using K4b2 (COSMED, Italy) under following six conditions; 1) supine position without abdominal corset, 2) sitting position without abdominal corset, 3) supine position with non-elastic abdominal corset, 4) sitting position with non-elastic abdominal corset, 5) supine position with elastic abdominal corset, 6) sitting position with elastic abdominal corset. Wilcoxon signed-rank test was applied for statistical assessment of group difference.
Results: Vital capacity and tidal volume in the condition with elastic abdominal corset were significantly increased than those of the condition without corset (p＜0.05) or of the condition with non-elastic corset (p＜0.01) in both sitting and supine position. Vital capacity and tidal volume in the condition with non-elastic abdominal corset were significantly decreased than those of the condition without corset (p＜0.05) in both sitting and supine position. Oxygen consumption was least in the condition with elastic abdominal corset (p＜0.05).
Conclusion: These results demonstrated that the elastic abdominal corset is beneficial in improving the efficiency of breathing for the patients with cervical spinal cord injury. However, non-elastic abdominal corset is harmful to these patients.
Objective: To describe the videofluoroscopic findings of quadriplegic patients with dysphagia and to assess the predisposing factors of dysphagia in quadriplegic patients.
Method: Six quadriplegic patients with a dysphagia within 3 months from the injury were included in this study and videofluoroscopic evaluations for both lateral and AP views were performed and evaluated.
Result: A variety of swallowing deficits that involve both oral and pharyngeal phases were detected in the videofluoroscopic study.
The presumed predisposing factors of dysphagia were a surgical stabilization of cervial vertebrae, inadequate neck extension due to cervical orthosis, history of mechanical ventilation, cervical traction, tracheostomy state and cranial nerve injury.
Conclusion: When there are symptoms that suggest a swallowing problem such as a frequent reflex coughing and voice change in quadriplegic patients, videofluoroscopic study will help to prevent the pulmonary complications and to determine the successful swallowing strategies.
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