VATER syndrome is defined as the combinations of three or more of the followings; Vertebral defect, Anal atresia, Esophageal atresia and/or Tracheo-Esophageal fistula, Renal dysplasia, and Radial-ray limb anomalies. A 21-month old female patient was admitted. She had the history of anastomosis of trcheo-esophageal fistula, V-P shunt operation due to hydrocephalus, and spina bifida. Upon admission, physical examinations showed increased deep tendon reflexes of all four extremities with grade 1 spasticity and popliteal angle being 45 degrees, bilaterally. The overall development of the patient was delayed. The imaging study showed hydrocephalus, periventricular leukomalatic change, underdevelopment of the cerebral cortex, atrophy of corpus callosum, Arnold-Chiary malformation, a vascular malformation along the intradural space from lower cervical to nearly entire thoracic vertebral level, multiple vertebral anomalies, and the fusion of multiple ribs. Furthermore hydronephrosis of the left kidney was detected. Follow-up of natural history and management of the patient is needed.
Anus, Imperforate ; Atrophy ; Cerebral Cortex ; Corpus Callosum ; Esophageal Atresia ; Esophagus ; Extremities ; Female ; Fistula ; Heart Defects, Congenital ; Humans ; Hydrocephalus ; Hydronephrosis ; Kidney ; Muscle Spasticity ; Natural History ; Physical Examination ; Radius ; Reflex, Stretch ; Ribs ; Spinal Dysraphism ; Spine ; Trachea ; Vascular Malformations

Freezing of gait (FOG), which is the most common symptoms in Parkinson's disease, is a unique gait disorder that patients are unable to initiate or continue locomotion. However, the pathophysiology of FOG has been poorly understood. We report two cases, one case is a 26-year old man and the second case is a 65-year old man, who showed FOG following hypoxic brain injuries caused by sudden cardiac arrest and hypovolemic shock, respectively. Brain F-18 FDG-PET images demonstrated the diffuse cortical hypometabolism in case 1 patient, and the decreased metabolism of the subcortical structures in case 2 patient. Two patients showed the typical features of FOG (turning, destination, and tight quarter hesitations combined with kinesia paradoxa) and the abnormal patterns of temporospatial data in kinematic gait analysis. We present two cases of FOG following hypoxic brain injury with reviewing of some literatures.
Brain ; Brain Injuries ; Death, Sudden, Cardiac ; Freezing ; Gait ; Humans ; Locomotion ; Parkinson Disease ; Shock ; Weather

Anterior spinal artery syndrome refers to the paralysis of the bilateral upper extremities, bladder dysfunction and the sensory deficit of pain and temperature below the level of injury. A 64 year-old female got a cardiac arrest event after stent insertion into the coronary artery. After CPR, she underwent the motor deficit (Z-T) of the bilateral upper extremities without any sensory deficit; proprioception, vibration and pain. The brain MRI showed no abnormality, but high signal intensity was detected in C3-C7 level by T2 sagittal plane and at the anterior horn area of gray matter by axial view of spine MRI. The ventilator has been applied after CPR. By the fluoroscopy, the movement of the diaphragm was decreased, and the nerve conduction study of both phrenic nerves showed no responses.
Animals ; Anterior Spinal Artery Syndrome ; Brain ; Cardiopulmonary Resuscitation ; Coronary Vessels ; Diaphragm ; Female ; Fluoroscopy ; Heart Arrest ; Horns ; Humans ; Neural Conduction ; Paralysis ; Phrenic Nerve ; Proprioception ; Respiratory Paralysis ; Spinal Cord ; Spinal Cord Ischemia ; Spine ; Stents ; Upper Extremity ; Urinary Bladder ; Ventilators, Mechanical ; Vibration

Cerebral air embolism is an unusual event that is mainly an iatrogenic cause, such as open heart surgery. We present a case of cerebral air embolism in a patient undergoing ASD patch repair with cardiopulmonary bypass. He had a status epilepticus, loss of consciousness and marked left limb weakness immediately after the operation. Diffusion-weighted MRI with angiography showed acute infarction in right entire hemisphere with patent internal carotid and intracranial arteries, and glucose PET brain scan showed severe decreased uptakes in right hemisphere. He recovered markedly with mild motor impairment of left upper and lower limbs in the 6 months after onset.
Angiography ; Arteries ; Brain ; Cardiopulmonary Bypass ; Embolism, Air ; Extremities ; Glucose ; Heart ; Humans ; Infarction ; Lower Extremity ; Status Epilepticus ; Thoracic Surgery ; Unconsciousness

Velopharyngeal insufficiency after surgical resection of soft palate cancer can be troublesome. This report concerns a male suffered from severe dysphagia following combined treatment for soft palate cancer. Sequential videofluoroscopic swallowing studies (VFSS) were used to assess his swallowing function and plan the interventional strategies. Initial VFSS showed huge nasal regurgitation, increased oral transit time, residues in oral cavity, delayed swallowing reflex, pharyngeal residue, impaired laryngeal elevation, and aspiration in semisolid and liquid trials. Obturator prosthesis was fabricated to minimize velopahryngeal insufficiency. After application of obturator prosthesis, swallowing dysfunction in oral and pharyngeal stages was markedly improved. Nasal regurgitation was not shown. Oral residue, oral transit time in oral stage also improved. Residue on vallaculae and pyriform sinuses decreased in pharyngeal stage. Aspiration also decreased. We reported successful obturator prosthesis application with sequential changes of clinical and VFSS findings in our case.
Deglutition ; Deglutition Disorders ; Gagging ; Humans ; Male ; Mouth ; Oral Stage ; Palate, Soft ; Prostheses and Implants ; Pyriform Sinus ; Velopharyngeal Insufficiency

Bilateral phrenic nerve palsy after open cardiac surgery is an extremely rare complication, but serious enough to induce respiratory failure or cardiac arrest. We report a 76-year-old male patient presented weaning difficulty after elective aortic valve replacement and thymic resection under hypothermic cardiopulmonary bypass. Bilateral phrenic nerve palsy was diagnosed through chest radiograph and electrophysiological studies of the diaphragm. After receiving tracheostomy, invasive intermittent positive-pressure ventilation (IPPV) was applied to him at intensive care unit. He was referred to our hospital because of weaning difficulty which lasted for 6 months after the surgery. He received extensive pulmonary rehabilitation which includes non-invasive positive-pressure ventilation (NIPPV), and then he finally succeeded in weaning from ventilator after 8 months. Applying NIPPV to patients with bilateral phrenic nerve palsy could minimize the duration of IPPV, reduce its disadvantages, and facilitate weaning of artificial ventilation.
Aged ; Aortic Valve ; Cardiopulmonary Bypass ; Diaphragm ; Heart Arrest ; Humans ; Intensive Care Units ; Intermittent Positive-Pressure Ventilation ; Male ; Paralysis ; Phrenic Nerve ; Positive-Pressure Respiration ; Respiratory Insufficiency ; Thoracic Surgery ; Thorax ; Tracheostomy ; Ventilation ; Ventilators, Mechanical ; Weaning

"Trigger finger at the wrist" is a rare phenomenon. The main symptom consists in a triggering at the wrist produced by finger motion. Its etiology and presentation may vary. One of etiology, some anomalies of muscle can cause this disease. Triggering finger at the wrist with carpal tunnel syndrome by abnormal the belly of the flexor digitorum superficialis has been reported. But most cases of it have been reported by orthopedic surgeons. We are presenting the case of 28 year old male patient who had abnormal flexor digitorum superficialis in carpal tunnel which was diagnosed by using ultrasonography.
Carpal Tunnel Syndrome ; Fingers ; Humans ; Male ; Muscles ; Orthopedics ; Wrist

Metronidazole can induce serious neurologic problems including peripheral neuropathy, seizures, and encephalopathy. We examined a patient with acute Wernicke's encephalopathy and peripheral polyneuropathy that had developed after prolonged metronidazole therapy without a history of chronic alcoholism or poor nutritional intake. The 68-year-old man had been hospitalized for a brain abscess and was treated for 10 weeks with metronidazole (2 grams per day). This patient, who showed symptoms of numbness and tingling in the legs, was referred for electromyography (EMG) and was diagnosed with peripheral polyneuropathy. A few days later, he developed sudden ataxia, dizziness, and diplopia. The neurologic examination revealed nystagmus and ophthalmoplegia, and the FLAIR brain MRI showed symmetrical high signal intensity lesions in the cerebellar dentate nucleus, midbrain, tegmentum around the periaqueductal gray matter, and tectum. After administering intravenous thiamine and stopping the metronidazole therapy, he recovered from the ophthalmoplegia and ataxia. Brain MR showed complete recovery within 3 weeks; however the EMG remained abnormal for a further 6 months, although the symptoms were almost completely resolved by this time.
Aged ; Alcoholism ; Ataxia ; Brain ; Brain Abscess ; Cerebellar Nuclei ; Diplopia ; Dizziness ; Electromyography ; Humans ; Hypesthesia ; Leg ; Mesencephalon ; Metronidazole ; Neurologic Examination ; Ophthalmoplegia ; Periaqueductal Gray ; Peripheral Nervous System Diseases ; Polyneuropathies ; Seizures ; Thiamine ; Wernicke Encephalopathy

OBJECTIVE: To evaluate the efficiency of powered wheelchair and scooter and to investigate the factors affecting the accidents and injuries. METHOD: We investigated 90 subjects with mobility disability who had used powered wheelchair or scooter more than a year. The subjects responded to a questionnaire of the 34 items. RESULTS: Seventy (77.7%) of respondents used powered wheelchair or scooter more than 3 days a week. More than 50% of them regarded the obstacles such as curbs or thresholds as the most common hindering factor to outside mobility. The causes of breakdown were due to battery, wheel, gear box and motor. About a third of 58 respondents were dissatisfied with the result of repair. The high cost of the repair was regarded as the leading cause of dissatisfaction. Contusions were the most common, with abrasion and sprain accounting for most of the remainder. Of the 18 users who were affected by injuries, only 11.1% wore a safety seatbelt. Only 25 of 89 respondents had a safety education. CONCLUSION: Our study showed that the efficiency of power wheelchair and scooter was relatively high. But the obstacles such as curbs, recurrent breakdown, and concerns about the accident were regarded as the hindering factors to the outside mobility. The degree of satisfaction to the safety education is relatively low, so more systemic and practical programs need to be devised.
Accounting ; Contusions ; Surveys and Questionnaires ; Questionnaires ; Self-Help Devices ; Sprains and Strains ; Wheelchairs

OBJECTIVE: To emphasize the need for precise diagnosis of amyotrophic lateral sclerosis (ALS), a progressive and degenerative disease of upper and lower motor neurons that often present initially with weakness at the upper or lower extremities, and frequently misdiagnosed as myelopathy, radiculopathy, peripheral neuropathy or arthropathy that may ultimately lead to unnecessary treatments including surgical procedures. METHOD: We retrospectively reviewed medical records of 331 ALS patients who visited our hospital between 1998 and 2008. Symptoms at onset, progression of disease, radiologic findings, surgeries prior to diagnosis of ALS, outcome after surgery or conservative treatments, and electrodiagnostic study results were reviewed. RESULTS: Among the 331 patients with ALS, 34 (10.3%) had a history of surgical procedure and 37 (11.1%) underwent conservative treatment prior to diagnosis of ALS. 34 patients with a mean disease duration at diagnosis of 20.0+/-14.9 months, had surgery for symptoms that were later attributable to ALS. In 30 of the 34 patients, symptoms did not resolve after the intervention. 37 patients with a mean disease duration at diagnosis of 16.6+/-14.3 months, underwent conservative treatments such as physical therapy prior to diagnosis of ALS. Only in one patient (2.7%), symptoms improved after conservative treatment. CONCLUSION: In the absence of a single confirmatory study for the diagnosis of ALS, clinical findings may be misinterpreted, leading to an erroneous diagnosis. Therefore, closer and more careful follow-up is necessary for patients with limb weakness in the absence of sensory symptoms, or bulbar abnormalities such as dysarthria and dysphagia.
Amyotrophic Lateral Sclerosis ; Deglutition Disorders ; Dysarthria ; Extremities ; Follow-Up Studies ; Humans ; Lower Extremity ; Medical Records ; Motor Neurons ; Peripheral Nervous System Diseases ; Radiculopathy ; Retrospective Studies ; Spinal Cord Diseases