OBJECTIVE: To investigate the effect of physical therapy (PT) intervention on spasticity in patients with cerebral palsy (CP), and to assess the degree of deterioration of spasticity when regular PT is interrupted in those patients. METHODS: We recruited 35 children with spastic CP who visited our hospital for PT, and whose Modified Tardieu Scale (MTS) scores were serially recorded including before and after a 10-day public holiday time frame period. The outcome measures were the angle of range of motion (ROM) of dorsiflexion of the ankle joint (R1 and R2) in the knee flexion and extension positions as assessed using the MTS. RESULTS: The range of dorsiflexion of the ankle joint (R1 and R2) after the holiday period was significantly decreased as compared with that measured ROM noted before the holiday period, regardless of the knee position, age, or gross motor function. The dynamic component of the MTS (R2–R1) showed a slight decrease in the knee flexion position. CONCLUSION: Interruption of regular PT aggravated spasticity and decreased ankle joint ROM in children with spastic CP. Our findings suggest that regular PT in the care continuum for children with CP is crucial for the maintenance of ROM in the spastic ankle joints.
Ankle Joint ; Cerebral Palsy ; Child ; Continuity of Patient Care ; Holidays ; Humans ; Knee ; Muscle Spasticity ; Outcome Assessment (Health Care) ; Range of Motion, Articular

PURPOSE: Nutritional therapy (NT), such as enteral nutrition (EN) or parenteral nutrition (PN), is essential for the malnourished patients. Although the complications related to NT has been well described, multicenter data on symptoms in the patients with receiving NT during hospitalization are still lacking. METHODS: Nutrition support team (NST) consultations, on which NT-related complications were described, were collected retrospectively for one year. The inclusion criteria were patients who were (1) older than 18 years, (2) hospitalized, and (3) receiving EN or PN at the time of NST consultation. The patients' demographics (age, sex, body mass index [BMI]), type of NT and type of complication were collected. To compare the severity of each complication, the intensive care unit (ICU) admission, hospital stay, and type of discharge were also collected. RESULTS: A total of 14,600 NT-related complications were collected from 13,418 cases from 27 hospitals in Korea. The mean age and BMI were 65.4 years and 21.8 kg/m2. The complications according to the type of NT, calorie deficiency (32.4%, n=1,229) and diarrhea (21.6%, n=820) were most common in EN. Similarly, calorie deficiency (56.8%, n=4,030) and GI problem except for diarrhea (8.6%, n=611) were most common in PN. Regarding the clinical outcomes, 18.7% (n=2,158) finally expired, 58.1% (n=7,027) were admitted to ICU, and the mean hospital days after NT-related complication were 31.3 days. Volume overload (odds ratio [OR]=3.48) and renal abnormality (OR=2.50) were closely associated with hospital death; hyperammonemia (OR=3.09) and renal abnormality (OR=2.77) were associated with ICU admission; “micronutrient and vitamin deficiency” (geometric mean [GM]=2.23) and volume overload (GM=1.61) were associated with a longer hospital stay. CONCLUSION: NT may induce or be associated with several complications, and some of them may seriously affect the patient's outcome. NST personnel in each hospital should be aware of each problem during nutritional support.
Adult ; Body Mass Index ; Demography ; Diarrhea ; Enteral Nutrition ; Hospitalization ; Humans ; Hyperammonemia ; Intensive Care Units ; Korea ; Length of Stay ; Multicenter Studies as Topic ; Nutrition Therapy ; Nutritional Support ; Parenteral Nutrition ; Referral and Consultation ; Retrospective Studies ; Vitamins

A patient with Hepatitis A virus (HAV) infection was notified to the public health authority. The identical HAV was found at a water dispensing outlet at the ground water facility and the toilet close to the facility. Serosurveillance in the community was conducted. Suspicious individuals were asked to visit city public health center and had a serologic test for anti-HAV Ig M. Overall, 100 individuals were tested, and all were negative for the anti-HAV Ig M. In our study cohort, we could not identify additional case of HAV infection.

Central hypoventilation syndrome is a rare and fatal condition resulting from various central nervous system disorders that is characterized by a failure of automatic breathing. We report a case of central hypoventilation syndrome following posterior circulation stroke whose pulmonary function was improved by respiratory rehabilitation. A 59-year-old woman with a history of hemorrhagic stroke of the bilateral cerebellum was hospitalized due to pneumonia. A portable ventilator was applied via tracheostomy, recurrent episodes of apnea and hypercapnia impeded weaning. A respiratory rehabilitation program including chest wall range of motion exercise, air stacking exercise, neuromuscular electrical stimulation (NMES) on abdominal muscles, upper extremity ergometer, locomotor training, high-frequency chest wall oscillator, mechanical insufflation, and exsufflation was employed, as spirometry showed a severe restrictive pattern. A spontaneous breathing trial was started, and a portable ventilator was applied for 8 hours, only during nighttime, to prevent sudden apneic event. After 4 weeks of treatment, follow-up spirometry showed much improved respiratory parameters. This case suggests that respiratory rehabilitation can improve pulmonary function parameters and quality of life in central hypoventilation syndrome.
Abdominal Muscles ; Apnea ; Central Nervous System Diseases ; Cerebellum ; Electric Stimulation ; Female ; Follow-Up Studies ; Humans ; Hypercapnia ; Hypoventilation ; Insufflation ; Middle Aged ; Pneumonia ; Quality of Life ; Range of Motion, Articular ; Rehabilitation ; Respiration ; Respiratory Center ; Spirometry ; Stroke ; Thoracic Wall ; Tracheostomy ; Upper Extremity ; Ventilators, Mechanical ; Weaning

Subtle dysfphagia, which is increased post-swallowing remnants, is a frequent finding in the elderly with various etiologies. These changes in swallowing are frequently overlooked by physicians. On the other hand, subtle changes evident on a videofluoroscopic swallowing study (VFSS) may suggest hidden disease. Therefore, clinicians should evaluate incidental dysphagia. Case 1: A 65-year-old man with no relevant medical history, presented with dysphagia and residual sensation during meals. VFSS showed moderate post-swallowing remnants in the vallecular fossa and pyriformis sinus. Further examination revealed prostate cancer with multiple bone metastases including the skull. Case 2: A 60-year-old man complained of residual sensation after swallowing, which started 2 months ago. He had a history of lung cancer. Pharyngeal residue was observed on VFSS. A brain metastasis was observed on MRI. Post-swallowing residue is often neglected or overlooked by clinicians who regard them as the features of aging. The present cases show that mild dysphagia with increased post-swallowing remnants may be an initial presentation of a hidden malignancy with metastasis. Physicians should consider unexplained dysphagia or tongue atrophy as possible initial presentations of hidden malignancies.
Aged ; Aging ; Atrophy ; Brain ; Deglutition ; Deglutition Disorders* ; Hand ; Humans ; Lung Neoplasms ; Magnetic Resonance Imaging ; Meals ; Middle Aged ; Neoplasm Metastasis ; Prostatic Neoplasms ; Sensation ; Skull ; Tongue

We report a female proband carrying a de novo 5q34-q35.2 deletion breakpoint, and review the unique skeletal phenotype and possible genotype related to this mutation. The patient presented with a persistent head tilt and limited head rotation. Non-contrast-enhanced three-dimensional computed tomography of the cervical spine revealed several malformations including a bone cleft in the right pars interarticularis, a bone defect in both C5 lamina and the transverse foramen at C2–C3, agenesis of the right articular process of C5, bony fusion of C4–C5, and subluxation of the craniocervical joints. Several deformities of the cervical spine seen in this patient have not been associated with the 5q deletion. A review of 5q-related mutations suggests that abnormalities associated with MSX2 gene might cause cervical spine abnormalities.
Chromosome Deletion ; Congenital Abnormalities ; Female ; Genotype ; Head ; Humans ; Joints ; Phenotype ; Spine*

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients exhibit normal liver functions and structures. The factors associated with INCPH include the following: Umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and autoimmune diseases. Approximately 70% of patients present a history of major variceal bleeding, and treatment relies on the prevention of complications related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Therfore, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varices with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review.
Arsenic ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Biopsy ; Esophageal and Gastric Varices ; Fibrosis ; Hepatic Veins ; Humans ; Hypertension, Portal ; Liver ; Liver Cirrhosis ; Lupus Erythematosus, Systemic ; Portal Pressure ; Scleroderma, Systemic ; Spondylitis, Ankylosing ; Varicose Veins ; Vinyl Chloride

Idiopathic non-cirrhotic portal hypertension (INCPH) is a disease with an uncertain etiology consisting of non-cirrhotic portal hypertension and portal pressure increase in the absence of liver cirrhosis. In INCPH, patients exhibit normal liver functions and structures. The factors associated with INCPH include the following: Umbilical/portal pyremia, bacterial diseases, prothrombic states, chronic exposure to arsenic, vinyl chloride monomers, genetic disorders, and autoimmune diseases. Approximately 70% of patients present a history of major variceal bleeding, and treatment relies on the prevention of complications related to portal hypertension. Autoimmune disorders associated with INCPH are mainly systemic sclerosis, systemic lupus erythematosus and rheumatoid arthritis. To the best of our knowledge, a case of ankylosing spondylitis (AS) associated with INCPH has not been reported thus far. Therfore, we report our experience of a patient with AS accompanied by INCPH, who showed perisplenic varices with patent spleno-portal axis and hepatic veins along with no evidence of cirrhosis on liver biopsy, and provide a brief literature review.

Gitelman syndrome is a condition caused by a mutation of the thiazide sensitive Na-Cl cotransporter gene on the distal convoluted tubule. It results in a variety of clinical features, including hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis. It is often diagnosed in asymptomatic adults presented with unexplained hypokalemia; however, it is sometimes associated with muscular cramps, numbness, fatigue, weakness, or paralysis. We experienced a case of rheumatoid arthritis accompanied by Gitelman syndrome, presented with hand tremor. We diagnosed her using renal clearance study and genetic analysis. Here, we report our experiences regarding this case along with a literature review.
Adult ; Alkalosis ; Arthritis, Rheumatoid* ; Fatigue ; Furosemide ; Genetic Testing ; Gitelman Syndrome* ; Hand ; Humans ; Hypesthesia ; Hypokalemia ; Muscle Cramp ; Paralysis ; Solute Carrier Family 12, Member 3 ; Thiazides ; Tremor

Neuropathic pain is usually managed pharmacologically, rather than with botulinum toxin type A (BTX-A). However, medications commonly fail to relieve pain effectively or have intolerable side effects. We present the case of a 62-year-old man diagnosed with an intracranial chondrosarcoma, which was removed surgically and treated with radiation therapy. He suffered from neuropathic pain despite combined pharmacological therapy with gabapentin, amitriptyline, tramadol, diazepam, and duloxetine because of adverse effects. BTX-A (100 units) was injected subcutaneously in the most painful area in the posterior left thigh. Immediately after the injection, his pain decreased significantly from 6/10 to 2/10 on a visual analogue scale. Pain relief lasted for 12 weeks. This case report describes intractable neuropathic pain caused by a brain tumor that was treated with subcutaneous BTX-A, which is a useful addition for the management of neuropathic pain related to a brain tumor.
Amitriptyline ; Botulinum Toxins* ; Botulinum Toxins, Type A* ; Brain Neoplasms* ; Brain* ; Chondrosarcoma ; Diazepam ; Duloxetine Hydrochloride ; Humans ; Middle Aged ; Neuralgia* ; Thigh ; Tramadol