• The Journal of Korean Physical Therapy
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• v.12 no.1
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• pp.15-21
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• 2000

Rising from a supine position to erect stance is an important milestone in treatment of patients with neurologic dysfunction. The purposes of this study wed to describe the movement patterns that hemiplegic patients use when rising to a standing. position and to investigate whether movement patterns that an proposed to treat of functional activity, Seventeen patients were videotaped performing three trials of rising. Movement patterns were described with three body components : upper extremities, head-trunk, lower extremities. Subjects rose most commonly using a push and reach pattern of the upper extremities-symmetrical interrupted by rotation pattern in the head-trunk-an asymmetrical squat pattern in the lower extremities and a push and reach pattern of the upper extremities-symmetrical interrupted by rotation pattern in the head-trunk-an symmetrical squat with balance step pattern in the lower extremities.

• The Journal of Churna Manual Medicine for Spine and Nerves
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• v.1 no.1
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• pp.15-25
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• 2006

Objectives : This report is about a case of a patient who improvement in general condition, Infection and bowel after and urinary dysfunction. Methods : We diagnosed that symptoms as Wejeung(?證), and employed oriental medical treatments, herb medication, acupuncture, electro-acupuncture, moxibustion and physical therapy. Results : In the first case, neurologic impairment was aggravated despite of 15 days of treatments. In the second case, motor and defecation was improved. Conclusions : There is no specific treatment for acute transverse myelitis in the Western medicine. Several case reports revealed clinical improvement of subacute or chronic transverse myelitis in the oriental medicine. We anticipated that the researches regarding treatment for acute transverse myelitis in the oriental medicine will be encouraged in the near future.

• Annals of Clinical Neurophysiology
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• v.18 no.1
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• pp.11-13
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• 2016

Idiopathic hypertrophic cranial pachymeningitis (ICHP) is diffuse inflammatory process of the dura mater. ICHP can produce similar presentation with Tolosa-Hunt syndrome (THS) if it involves cavernous sinus. A-29-year old male with persistent headache and no definite neurologic dysfunction was noted. Two weeks later, he complained of ophthalmoplegia, and his symptoms were thought to be manifestations of THS. Brain magnetic resonance images revealed diffuse thickened, enhanced pachymeninges in left tentorium. The patient was diagnosed with IHCP. We report a IHCP patient who showed very similar presentation as THS.

• Journal of Trauma and Injury
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• v.18 no.2
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• pp.175-178
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• 2005

Fat embolism syndrome is a collection of respiratory, neurological and cutaneous symptoms and signs associated with trauma and other disparate surgical and medical conditions. The incidence of clinical syndrome is low while the embolization of marrow fat appears to be an almost inevitable consequence of long bone fractures. The pathogenesis is a subject of conjecture and controversy. There are two theories which have gained acceptance(mechanical theory, biochemical theory). Onset of symptom is usually within 12 to 72 hours, but may manifest as early as 6 hours to as late as 10 days. The classic triad of fat embolism syndrome involves pulmonary changes, cerebral dysfunction and petechial rash. The cornerstone of treatment is preventing the stress response, hypovolemia and hypoxia and operative stabilization of fractures. Corticosteroid are the only drugs which have repeatedly shown a positive effect on the prevention and treatment of fat embolism syndrome. We report a case of post-traumatic fat embolism syndrome with severe cerebral involvement without respiratory distress. A 55 years old female had a traffic accident. She sustained pelvic bone fracture and both humerus fracture. Approximately 4 hours after the accident, mental status change developed without a focal neurologic deficits. She had no respiratory symptom and sign. Her brain MRI showed multiple cerebral fat embolism lesion. The patients received supportive treatment with corticosteroid, albumin. Her neurologic status stabilized over several days. After orthopedic surgery, she was discharged 62 days after admission.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.11 no.1
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• pp.88-98
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• 2011

Lesch-Nyhan syndrome is a X-linked recessive disorder caused by a deficiency of the enzyme hypoxanthine-guanidine phosphoribosyltransferase (HPRT), enzyme to recycle purines. Case history: born induced vaginal delivery at 40 weeks complicated by premature membrane ruputure, body weight 2.820 gm. He showed failure to thrive showing severe protein aversion like milk products and pink daper. Developmental delay revealing rolling over at 10.5 month, followed by regression. Seizure at 2 months, His poor oral feeding was lifelong problem. Weak crying, spastic, choreoathetoid movement. Self mutilating behavior noted and diagnosed at age 3 years. No family history of consanguinity and neurological disorders. Method: Laboratory test, physical exam, imaging study and molecular. Clinical follow up Treat ment with allopurinol. Result: uric acid 10.5 mg/dL (N 3.5-7.9), APRT 151.1uM/ min/ml pro(25.7-101), HPRT 7.6 (N 233.5-701) and c.151C>T hemizygote (p,Arg51X). Abdominal sonogram showed staghorn calculi in both kidneys, brain MRI brain atrophy. Clinical follow up showed, seizure at 2 mo, developmental delay (head control and, rolling over at at 11mo, pointing body part at 2 yr 7 mo, eye hand coordination at 2 y 11mo,creeping at 3 y 7 mo, speaking words at 6 y 6 mo ),and developmental regression at 3 yr of age. Sleeping problem including insomnia and severe constipation. Self mutilating behavior (lip bite) started at 2.5 yr, neurologic sx including intermittent upward gaze accompanied by swallowing difficulty at 3 y 7 mo grand mal seizure at 4.5 yr and spastic extremity and trunchal hypotonia and choleoathetoid movement and ataxia at 6.5 yr. Scoliosis with severe spasticity at 9 yr 9 mo. Acute life threatening episode with irregular breathing at 9 yr and 9 mo, Emaciation and nephrolithiasis and recurrent pneumonia. Died suddenly at 10 yr 3 mo. Conclusion: life long feeding problem, chronic gut motility dysfunction, sleeping difficulty and progressing neurologic deterioration and nephrolithiasis despite normal serum uric acid maintence by allopurinol treatment.

• Korean Journal of Bronchoesophagology
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• v.13 no.1
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• pp.23-28
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• 2007

Background and Objectives: Since intractable aspiration in patients with impaired protective function of the larynx often results in multiple episode of aspiration pneumonia, repeated hospitalizations and expensive nursing care. The authors reported the preliminary results of laryngotracheal separation(LTS) in patient with chronic intractable aspiration. The purpose of this study was to report the follow up results of patient outcome with the LTS. Materials and Methods: A retrospective review of 15 patients who underwent LTS between 1996 and 2006 was conducted. Ages ranged from 3 to 72 years. Results: Eight patients had morbid aspiration as a consequence of acquired neurologic injuries and seven patients with congenital neurologic injuries. Two patients had a postoperative fistula, which was well controlled with local wound care. Following LTS, aspiration was effectively controlled in all patients and eight were able to tolerate a regular diet. Conclusion: LTS is a low-risk, successful, definitive procedure which decreases the potential for aspiration, pulmonary complications, duration of hospitalizations and increases quality of life, especially in patent with irreversible upper airway dysfunction and poor speech potential.

• Archives of Plastic Surgery
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• v.34 no.4
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• pp.478-484
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• 2007

Purpose: Multiple symmetric lipomatosis (MSL) is a relatively rare disorder characterized by presence of multiple, symmetric, nonencapsulated fat masses in face, neck, shoulder and other areas. There has been only a few cases reported in Korea. The main purpose of this research is to examine the Korean patients to see what kinds of special characteristics occurred due to this disease and to decide the proper treatment.Methods: A total of 16 patients were evaluated retrospectively. 5 patients were treated at our hospital. The other patients were reviewed from literature. We analyzed the biological characters of patients, location of fat deposit, morphologic characters of patients, clinical evidence of neuropathy, associated metabolic disorders and treatment modality.Results: All cases were male patient. The mean age of onset was 47.43 years. All patients were moderate to heavy alcoholics. The most common location of fat deposition was posterior neck and abdomen. In neurologic exam of 9 patients, 5 patients showed muscle weakness, tremor, pain and autonomic nerve dysfunction. In metabolic studies of 9 patients, total cholesterol values were higher in 1 patient. A glucose tolerance test was abnormal in 1 patient. In treatment modality, 14 patients were treated with surgical resection, 1 patient was treated with liposuction and surgical excision, 1 patient was treated only with liposuction. Conclusion: To treat MSL patients successfully, we should concentrate not only on the removal of the fatty tissue but also on neurologic abnormities, metabolic disorders and associated diseases.

• Journal of Chest Surgery
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• v.25 no.5
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• pp.511-517
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• 1992

From January 1988 to December 1990, 18 adult patients with aortic disease underwent surgical repair using hypothermia and total circulatory arrest. The age at operation ranged from 17 years to 64 years[mean 45.2$\pm$10.7 years]. We disease entities included aortic dissection in 12, aortoannuloectasia in 3 and thoracic aortic aneurysm in 3 cases. Partial cardiopulmonary bypass via femoral vessels along with surface cooling was used upon the induction of deep hypothermia[18~20oC]. Modified Bentall operation was performed in 7 cases, ascending aorta replacement in 6, graft interposition in descending thoracic aorta in 3 and others in 2 cases. The circulatory arrest was maintained for periods of 2 minutes to 86 mimutes[mean 34.7$\pm$5.0 minutes]. Overall hospital mortality was 27.8%[5/18]: brain damage was responsible for the death of 2 patients. 4 patients out of 13 survivors experienced postoperative neurologic dysfunction, which was proved to be self-limited except one case showing left hemiparesis. 12 patients were followed up postoperatively with the mean follow-up period 22.7$\pm$10.1 months. There was no death. No new neurologic problems were observed during follow-up period. All but one patient showing recurrent dissection and aortic regurgitation are in exellent clinical condition. These clinical data suggests that the principle of deep hypothermia and total circulatory arrest can be applied rather safely in adult patients, especially in the treatment of patients with aortic disease, it can be a valuable adjunct with better clinical results.

• Journal of Genetic Medicine
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• v.19 no.1
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• pp.22-26
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• 2022

Cerebrotendinous xanthomatosis (CTX) is a rare genetic disease caused by a deficiency of enzymes for the synthesis of bile acid, resulting in the accumulation of cholestanol with reduced chenodeoxycholic acid (CDCA) production and causing various symptoms such as chronic diarrhea in infancy, juvenile cataracts in childhood, tendon xanthomas in adolescence and young adulthood, and progressive neurologic dysfunction in adulthood. Because oral CDCA replacement therapy can effectively prevent disease progression, early diagnosis and treatment are critical in CTX. This study reports the case of CTX in a 10-year-old male who presented with Achilles tendon xanthoma and mild intellectual disability. Biochemical testing showed normal cholesterol and sitosterol levels but elevated cholestanol levels. Genetic testing showed compound heterozygous variants of CYP27A1, c.379C>T (p.Arg127Trp), and c.1214G>A (p.Arg405Gln), which confirmed the diagnosis of CTX. The patient had neither cataracts nor other focal neurologic deficits and showed no abnormalities on brain imaging. The patient received oral CDCA replacement therapy without any adverse effects; thereafter, the cholestanol level decreased and no disease progression was noted. The diagnostic possibility of CTX should be considered in patients with tendon xanthoma and normolipidemic conditions to prevent neurological deterioration.

• Journal of Oral Medicine and Pain
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• v.39 no.1
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• pp.10-14
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• 2014

Purpose: The aim of this study is to evaluate the difference between gustatory functions in a sleep disordered breathing (SDB) group and a control group. The pathogenesis of SDB has not been fully understood. Though the precise contributions of neuromuscular and anatomical factors on SDB pathogenesis are still debated, we hypothesized that the gustatory dysfunction could be predisposed to SDB. Methods: All patients were diagnosed as SDB by polysomnography (PSG). On the basis of PSG results, patients were divided into 3 groups: snoring, mixed, and obstructive sleep apnea (OSA). The control group comprised healthy volunteers who were the same age as those of the SDB group and whose breathing was verified as normal using a portable sleep monitor device. The patient group and the control group were evaluated for gustatory functions with an electrogustometry (EGM). The electrical taste thresholds were measured in the anterior, midlateral, and posterior sides of the tongue and soft palatal regions, both sides. To find out the difference in EGM scores, statistical analysis was performed using the Kruskal-wallis and Mann-Whitney U test with 95% confidence interval and p<0.05 significance level. Results: The patients with SDB had higher EGM scores than the control group at all spots tested, except for the right midlateral of the tongue, and there was a statistical significance in the comparison between the control group and the divided SDB groups, respectively. Among the divided SDB groups, the snoring group had the most significant differences in the number of the measured spots, but there was no difference among the snoring, mixed, and OSA groups. Conclusions: These results may suggest that neurologic alterations with sleep disordered breathing could be associated with gustatory dysfunction. In the future, further systemic studies will be needed to confirm this study.