A 21-year-old woman with gargoyloid face and short trunkal stature showed progressive quadriparesis. Cervical spine MRI showed circumferential compression of cervical spinal cord by thickened dura mater. Elevated urinary dermatan sulfate and decreased enzyme activity of -iduronidase revealed that she has mild form of mucopolysaccharidosis type I. Her weakness was improved with cervical laminectomy and duraplasty. In this case, progressing quadriparesis resulted from cervical spinal cord compression by thickened dura mater. Earlier surgical decompression could have been better for the patient.
Decompression, Surgical ; Dermatan Sulfate ; Dura Mater ; Female ; Humans ; Laminectomy ; Magnetic Resonance Imaging ; Mucopolysaccharidoses* ; Mucopolysaccharidosis I* ; Quadriplegia ; Spinal Cord Compression* ; Spinal Cord* ; Spine ; Young Adult

No abstract available.
Allopurinol* ; Animals ; Liver* ; Rats* ; Reperfusion Injury* ; Verapamil*

Vitamin D deficient ricket is now a rare disease in the developed countries because of the generally improved nutrition and medical awareness but occasional cases are found in the special susceptible populations. The ricket is characterized pathophysiologically by a failure of the normal mineralization of bones and epiphyseal cartilages and is susceptible to the fractures because of the weakness and increased plasticity of bones. The fractures occurred in rickets are known to be delayed in union but it should be normal if the ricket is effectively treated. Authors reviewed 7 cases of fractures occurred in the vitamin D deficient rickets which were managed at Daegu Fatima Hospital during the years from 1980 to 1986 and the following results were obtained. 1. The cases were 4 boys and 3 girls having ages from 6 to 11 years who were all in the same group of restricted population of poor nutrition. 2. 10 fractures noted in 7 cases including two cases of multiple fractures and a case of refracture and most of the fractures occurred in femurs except for a humerus and a forearm bones. 3. The causes of fractures were uncertain because the definite histories were unable to obtain but any violent trauma was not suggested. 4. The fractures were treated by the usual methods of skin tractions or cast immobilizations and daily doses of 2,000 U. or 5,000 U. of vitamin Dwere given as soon as the rickets are recognized. 5. The normal healing of the fractures and rickets occurred in the cases who were given vitamin D initially when the diagnosis of rickets were made. 6. The healing of fractures were very delayed in 2 cases who were missed to recognize the rickets initially and vitamin D was not given.
Daegu ; Developed Countries ; Diagnosis ; Female ; Femur ; Forearm ; Fractures, Multiple ; Growth Plate ; Humans ; Humerus ; Miners ; Plastics ; Rare Diseases ; Rickets ; Skin ; Traction ; Vitamin D ; Vitamins

No abstract available.
Angiotensin II* ; Angiotensins* ; Animals ; Hemorrhage* ; Rats*

BACKGROUND: Sleep-related disturbances and sleep disorders are common in Parkinson's disease (PD) and have a great impact on daily life of PD patients. This study was done to find the sleep characteristics and sleep disturbing factors in PD patients according to disease severity through clinical interview and polysomnographic (PSG) study. METHODS: Fifty patients with PD (22 males, age 60.6 +/- 6.4, Hoehn and Yahr (HY) stage 2.7 +/- 1.0) were recruited and thoroughly interviewed about their sleep. PSG was performed on the patients taking routine antiparkinsonian medications. Patients were grouped into mild and moderate/severe group according to HY stage, and the results were compared between each group. RESULTS: Ninety-four percent of total patients had one or more sleep-related disturbances based on the interview or PSG. On interview, the moderate/severe group complained more insomnia and REM sleep behavior disorder (RBD) than mild group. In PSG findings, the moderate/severe group showed lower sleep efficiency, longer sleep latency, REM sleep latency, waking time after sleep onset, and higher prevalence of RBD. CONCLUSIONS: In this study, most patients with PD had sleep disturbances. Clinical interview and PSG findings revealed deterioration of sleep quality along the disease severity. Our results suggest that sleep disturbances in PD patients are prevalent and warrant clinical attention, especially to the patients with advanced disease.
Humans ; Male ; Parkinson Disease* ; Polysomnography ; Prevalence ; REM Sleep Behavior Disorder ; Sleep Wake Disorders ; Sleep Initiation and Maintenance Disorders ; Sleep, REM

Non-specific periventricular white matter lucencies on computed tomography(leukoaraiosis) were found in 130(40%) of 324 patients with ischemic or hemorrhagic strokes. Patients with leukoaraiosis were significantly older than those without it and correlated with hypertension and lacunar infarction but not with cortical infarction. In patients with hemorrhage, leukoaraiosis occurred sinigificantly more often when aneurysm or arteriovenous malformation were not demonstrated. Our fiindings suggest that leukoaraiosis in cerebrovascular disease is associated with small vessel disease.
Aneurysm ; Arteriovenous Malformations ; Hemorrhage ; Humans ; Hypertension ; Infarction ; Leukoaraiosis* ; Stroke ; Stroke, Lacunar

No abstract available.
Vasodilation*

Hemiparkinsonism with hemiatrophy (HP-HA) syndrome is characterized by a predominant unilateral parkisonism associated with ipsilateral body and/or contralateral brain abnormality. We report three cases of Hemiparkinsonism- hemiatrophy, which is a rare form of secondary parkinsonism. One patient revealed motor complication in the involved limb suggesting that the degeneration of nigrostriatal pathway as the underlying mechanism in this disorder. The functional neuro-imaging can play an important role to verify the cortical abnormalities when structural changes are not evident in brain MRI.
Brain ; Extremities ; Humans ; Magnetic Resonance Imaging ; Neuroimaging ; Parkinson Disease, Secondary ; Parkinsonian Disorders

In patients with inflammatory bowel disease (IBD), ocular extraintestinal manifestations (EIM) are less common than EIM of other systems, but they are clinically important because they can lead to complications that can cause catastrophic damage to the visual acuity and ocular structure. Anterior uveitis and episcleritis are the most common ocular EIM. Involvement of the orbit, posterior segment, and optic nerve can also occur. A variety of treatments are available ranging from topical steroids to systemic immunosuppressive therapies. The treatment of IBD is also essential if the activity of inflammatory bowel disease affects the ocular symptoms.
Colitis, Ulcerative ; Crohn Disease ; Humans ; Inflammatory Bowel Diseases ; Ophthalmology ; Optic Nerve ; Orbit ; Scleritis ; Steroids ; Uveitis ; Uveitis, Anterior ; Visual Acuity

In patients with inflammatory bowel disease (IBD), ocular extraintestinal manifestations (EIM) are less common than EIM of other systems, but they are clinically important because they can lead to complications that can cause catastrophic damage to the visual acuity and ocular structure. Anterior uveitis and episcleritis are the most common ocular EIM. Involvement of the orbit, posterior segment, and optic nerve can also occur. A variety of treatments are available ranging from topical steroids to systemic immunosuppressive therapies. The treatment of IBD is also essential if the activity of inflammatory bowel disease affects the ocular symptoms.
Colitis, Ulcerative ; Crohn Disease ; Humans ; Inflammatory Bowel Diseases ; Ophthalmology ; Optic Nerve ; Orbit ; Scleritis ; Steroids ; Uveitis ; Uveitis, Anterior ; Visual Acuity