Klippel-Trenaunnay syndrome is characterized by three typical clinical manifestations; 1) Capillary malformations (port-wine stains), 2) bony and soft tissue hypertrophy, 3) varicosities or venous malforamation, but many other clinical manifestations can be presented. Although many associated clinical manifestations were reported in Klippel-Trenaunay syndrome, peripheral polyneuropathy or any other results of electrodiagonostic study were not reported previously. We experienced a 22 year old male who was transfered in rehabiliation program after surgical management of intra cerebral hemorrhage. During rehabilitation program we diagnosed him as Klippel-Trenaunay syndrome by three typical clinical manifestations associated with dilated cardiomyopathy. He also presented sensory impairment in distal part of all extremites. Electrodiagonostic study revealed peripheral polyneuropathy. We concluded that the possibility of peripheral polyneuropathy should be considered in Klippel-Trenaunnay syndrome.
Capillaries ; Cardiomyopathy, Dilated ; Cerebral Hemorrhage ; Humans ; Hypertrophy ; Klippel-Trenaunay-Weber Syndrome* ; Male ; Polyneuropathies* ; Rehabilitation ; Young Adult

Rett syndrome is a progressive encephalopathy in females that appears during the first 18 months of the life. A few neurophysiologic investigations of peripheral nerve and electrodiagnostic studies in Rett syndrome has proposed mild distal axonopathy. However, the electrodiagnostic study shows demyelination rather than axonopathy in presenting patient with Rett syndrome. An 11-year-old female had normal birth history, no perinatal problems and normal development until age of 18 months. Developmental regression was recognized by her parent and slowly progressed. At physical examination at her age of 7 years, stereotypic hand movements was present and she could walk with frequent fall. Brain Computed Tomography was not specific and electrodiagnostic study revealed slow conduction velocity in all nerves tested. After follow-up for 4 years, hand wringling was remained and she became to be unable to walk alone. Electrodiagnostic study revealed as follows; 1) Compound muscle action potentials and sensory nerve action potentials in all the nerves tested revealed prolonged distal latency with normal amplitude. 2) F waves were evoked with prolonged latency in the all the nerves tested. 3) H-reflex were absent, bilaterally. 4) Facial motor conduction study and blink reflex showed normal findings. 5) Auditory evoked potential and visual evoked potential studies showed normal findings. These electrodiagnostic study indicates demyelinating neuropathy.
Action Potentials ; Blinking ; Brain ; Child ; Demyelinating Diseases ; Electromyography ; Evoked Potentials, Auditory ; Evoked Potentials, Visual ; Female ; Follow-Up Studies ; H-Reflex ; Hand ; Humans ; Parents ; Peripheral Nerves ; Physical Examination ; Reproductive History ; Rett Syndrome

We report the case of a 40-year-old hypoxic encephalopathy patient who suffered from dry mouth and frequent poor oral hygiene secondary to a prominent nasolabial fold and elevated upper lip, exposing the canine teeth at rest. This expression was confirmed secondary to persistent contraction of the levator labii superioris muscle with electromyography (EMG) study. We have injected 6 units of Botulinum toxin A in levator labii superioris muscle with electromyographic guidance. Elevation of upper lip at rest causing exposure of canine teeth has been nearly disappeared 3 days after the injection. We suggest that chemical weakening of the levator labii superioris muscle using Botulinum toxin A could be possibly responsible for the dramatic reduction of elevated upper lip exposing canine teeth in patients with hypoxic encephalopathy.
Adult ; Botulinum Toxins* ; Cuspid ; Electromyography ; Humans ; Hypoxia, Brain ; Lip ; Mouth ; Nasolabial Fold ; Oral Hygiene

The syndrome of inappropriate secretion of antidiuretic hormone (SIADH) is a frequent complication of severe head trauma. Fluid restriction is treatment choice of SIADH in patients with traumatic brain injury (TBI), but fluid restriction is limited because they need sufficient calories. We described a patient who, on the four months after a head injury, presented with deterioration of consciousness, which coincided with the development of the SIADH, and which rapidly reversed with the correction of the hyponatremia by demeclocycline with minimal fluid restriction. We suggest that SIADH should be included in the differential diagnosis of deterioration of consciousness during the recovery period of the patients suffering from head injury because unexpected clinical deterioration may often have a reVersible cause. Also, demeclocycline will be useful, which allows for increased fluid liberalization and for provision of adequate calories, in the treatment of the SIADH in patients with TBI.
Brain Injuries ; Brain* ; Consciousness ; Craniocerebral Trauma ; Demeclocycline* ; Diagnosis, Differential ; Humans ; Hyponatremia ; Inappropriate ADH Syndrome

OBJECTIVE: To investigate the effect of cervical orthosis upon swallowing and the differences of bracing effect between normal people and spinal cord injured patients. METHOD: 12 normal adults and 32 cervical cord injured patients who were wearing one of the three common cervical orthoses (soft neck collar, Philadelphia brace, and Minerva brace) were recruited. Swallowing function was evaluated by videofluoroscopic swallowing study (VFSS) without cervical bracing for the baseline data and with cervical bracing to compare with baseline data. The parameters observed were oropharyngeal diameter, initiation point of swallowing, hyoid bone movement, laryngeal penetration, aspiration, and residual volume after swallowing. RESULTS: Cervical orthoses decreased oropharyngeal diameter, reduced hyoid bone movement, increased residual volume, and changed initiation point of swallowing. Minerva brace revealed to give more influence than other braces. Normal adult group and patient group showed no difference in cervical bracing effect. CONCLUSION: Attention should be paid to swallowing function when cervical bracing is needed because cervical bracing itself can increase the risk of aspiration.
Adult ; Braces ; Deglutition* ; Humans ; Hyoid Bone ; Neck ; Orthotic Devices* ; Residual Volume ; Spinal Cord

Most of axillary nerve injury develops after dislocation of glenohumeral joint, proximal humeral fracture and direct blow to the deltoid muscle. Some cases in volleyball players and athletes playing contact sports like hockey, football have been reported. But axillary nerve injury after swimming with butterfly stroke has not been reported previously. We experienced a 34 year old female who had weakness in abduction and sensory impairment in lateral aspect of right arm after butterfly stroke. She was transferred from local clinic to our Rehabilitation Department because symptoms were not improved despite conservative treatment. We diagnosed her as axillary nerve injury by typical clinical manifestations and electrodiagnostic study. Additionally, we detected type II superior laburum anterior posterior lesion combined with axillary nerve injury in shoulder magnetic resonance image. We should consider possibility of axillary nerve injury in a patient with shoulder pain and weakness after swimming like butterfly stroke.
Adult ; Arm ; Athletes ; Butterflies* ; Deltoid Muscle ; Dislocations ; Female ; Football ; Hockey ; Humans ; Rehabilitation ; Shoulder ; Shoulder Fractures ; Shoulder Joint ; Shoulder Pain ; Sports ; Stroke* ; Swimming* ; Volleyball

The study of blink reflexes was carried out to demonstrate the correlations, if there were, between the stage of diffuse axonal injury(DAI) and the abnormality of blink reflexes. The blink reflex was recorded in 20 healthy adult subjects and 22 patients with DAI who were classified according to Adams' classification(DAI I; 7, DAI II; 9 and DAI III; 6). The latencies and amplitudes of R1 and R2 in patients with DAI were compared with those of healthy subjects. The results were as follows; 1) In 20 subjects of patient group, the latencies of R1 were all within a normal range. In 2 subjects, the difference in latencies between the two sides was above 1.4 msec. 2) In 15 subjects, R2 was absent or delayed, and reduced in the size of amplitude in all. Nine were affected bilaterally, and 4 were unilaterally. 3) Seventy one percent of patients in each stage represented abnormal findings. 4) There were no correlations between the DAI stage and the blink reflex. This study demonstrated that the polysynaptic R2 was more profoundly suppressed than the oligosynaptic R1 in a diffuse axonal injury because of a loss or decrease of suprapontine facillitation, which influenced the trigeminal spinal complex and the interneuron of lateral reticular formation.
Adult ; Axons ; Blinking* ; Diffuse Axonal Injury* ; Humans ; Interneurons ; Reference Values ; Reticular Formation

OBJECTIVE: The aim of this study is to evaluate the change of esophageal acidity when feeding via nasogastric tube is replaced by via percutaneous endoscopic gastrostomy (PEG) tube. METHOD: Fourteen patients with brain injury participated in the study. 24-hour pH monitoring was performed during nasogastric tubal feeding. After PEG tube insertion, 24-hour pH monitoring was followed up. There was no difference in medication affecting to esophageal acidity in same patient at both pH monitorings. The results of pH monitorings analyzed with Wilcoxon signed rank test. RESULTS: The total time below pH 4.00 was 135.43+/-190.69 minutes for the patients with nasogastric tube and 25+/-42.74 minutes for PEG tube (p=0.013). The numbers of acid reflux was 42.07+/-47.03 and 21.93+/-22.77 respectively (p=0.074). Of the 14 patients, 9 had acid reflux in nasogastric tubal feeding, which was improved in all 9 patients after PEG. Of the 14 patients, 5 had no acid reflux in nasogastric tubal feeding but 3 of the 5 developed new acid reflux in PEG tubal feeding. CONCLUSION: Percutaneous endoscopic gastrostmy tube feeding was better for acid reflex control. But careful observation is needed after PEG because PEG can develop new acid reflux.
Brain Injuries ; Brain* ; Enteral Nutrition* ; Gastrostomy* ; Humans ; Hydrogen-Ion Concentration ; Reflex

OBJECTIVE: The purposes of this study are to estimate the proper replacement time of percutaneous endoscopic gastrostomy Foley catheter for prevention of accidental expulsion from the stomach, and to identify factors influencing deflation of balloon. METHOD: Silicone Foley catheters (22 Fr) were placed and compared in the different environments: 1) different acidity (pH 1, 2, 3, 4, 7), 2) static versus dynamic (100 RPM) environment. The balloon capacity of 30 ml versus 5 ml inflated with 5 ml of normal saline were compared. Mean time interval of deflation of balloon down to the capacity of 2.5 ml and 1 ml was estimated and compared respectively. RESULTS: The results showed no significant difference of the decrease of the balloon of the Foley catheters in each acidity except for pH 1 and dynamic environment. But capacity of balloon could affect deflation. The mean days of deflation of total Foley catheter down to 2.5 ml and 1 ml were 23.5+/-5.3 and 42.2+/-7.2 days respectively. CONCLUSION: Physiological gastric acidity and dynamic environment did not affect the deflation of the Foley catheter significantly, but the capacity of the balloon affected it. And suggested proper time of the replacement of the Foley catheter gastrostomy tube is ranged from 24 to 42 days after exchange.
Catheters* ; Gastric Acid ; Gastrostomy* ; Hydrogen-Ion Concentration ; Peristalsis ; Silicones ; Stomach

OBJECTIVE: To help adjusting the proper timing of replacement of the percutaneous endoscopic gastrostomy (PEG) Foley catheter in brain injured patients. METHOD: Twenty one brain injured patients with PEG Foley catheter were studied and divided into three groups by indwelling duration. The balloon of Foley catheter was inflated with 5 ml of normal saline. We calculated the deflating velocity of the balloon by estimating remained amount of normal saline and indwelling duration at the time of replacement. RESULTS: In 6 patients, the PEG Foley catheter was pulled out easily before aspiration of remained normal saline. In all of these cases, remained amount of normal saline was less than 2 ml. The deflating velocities of the balloons were 0.057 +/- 0.024 ml/day in cases with 3 to 4weeks of indwelling duration, 0.066 +/- 0.005 ml/day with 4 to 5 weeks, 0.067 +/- 0.012 ml/day with above 5weeks. The mean deflating velocity was 0.063 +/- 0.016 ml/day in human. The deflating velocity was slower than that of the previous study in vitro. CONCLUSION: The mean days of deflation of the balloon of total PEG Foley catheter down to 2.5 ml were 42.1 days. We suggest that the PEG Foley catheter would be replaced within 42 days after exchange.
Brain* ; Catheters* ; Gastrostomy* ; Humans