The authors experience a case of juvenile type thoracic arteriovenous malformation. It is acute onset and represent spastic paraparesis, sensory Impairment and voiding difficult. Intradural spinal arteriovenous malformation is relatively rare lesion. The authors treated with total excision of A-V malformed vessel with good results.
Arteriovenous Malformations* ; Paraparesis, Spastic

No abstract available.
Brain ; Muscle Spasticity ; Paraparesis, Spastic ; Pyramidal Tracts

Oculodentodigital dysplasia (ODDD) is a rare autosomal dominant inherited disease caused by mutations of the human gap junction alpha 1 gene, which encodes the protein Connexin-43. Patients with ODDD may present with neurological deficits with a typical pleiotropic combination of characteristic craniofacial, ophthalmological, phalangeal, and dental anomalies. In this report, we describe the first genetically confirmed Korean ODDD patient, who presented with spastic paraparesis. We will also review the neurological aspects of ODDD as reported in the literature.
Gap Junctions ; Humans ; Muscle Spasticity* ; Paraparesis, Spastic*

Spastic paraparesis is caused by various etiologies such as autoimmune, infection, genetic and metabolic disorder. Adrenomyeloneuropathy (AMN) is very rare but one of important causes in spastic paraparesis. We experienced a patient presenting with adult-onset progressive spastic paraparesis, who was diagnosed as AMN with hemizygous c.431C>T (p.A144V), a novel mutation in exon1. The level of very long chain fatty acid should be included in diagnostic work-up for patients presenting with adult-onset progressive spastic paraparesis.
Adrenoleukodystrophy ; Humans ; Muscle Spasticity ; Paraparesis, Spastic

We present a case of a 68-year-old female with an atypical form of spinal tuberculosis, which involved posterior elements with multiple foci in two noncontiguous vertebral levels. The lesions caused spastic paraparesis and urinary hesitation. There was no evidence of pulmonary or other extrapulmonary tuberculous disease. Based on clinical and radiographic findings, this atypical spinal tuberculosis was preoperatively misdiagnosed as metastatic spine tumor. The histopathologic finding confirmed tuberculosis and the patient was treated successfully with surgery and antituberculous therapy. In case of a spinal lesion of unknown origin, it is important to be aware that atypical spinal tuberculosis can mimic metastatic spine tumor and tuberculosis should always be considered.
Aged ; Female ; Humans ; Paraparesis, Spastic ; Spine* ; Tuberculosis ; Tuberculosis, Spinal*

A case is presented to demonstrate the image findings of a patient with a recent onset of progressive spinal cord compression caused by a huge arachnoid cyst. A 45-year-old woman with progressive paraparesis and dysaesthesia on both legs had extradural mass on thoracolumbar junction and she was cured successfully with surgery. The tumor mass turned out to be arachnoid cyst histologically. By the time of discharge all neurologic deficits and symptoms were cured. Thoracolumbar extradural arachnoid cyst is rare cause of the spastic paraparesis. It is an unusual expanding lesion in the spinal canal which may communicate with the subarachnoid space. The goal of surgery is to decompress the spinal cord and close the connection with the subarachnoid space. We report a case of huge thoracolumbar arachnoid cyst presenting spastic paraparesis with a review of literatures.
Arachnoid* ; Female ; Humans ; Leg ; Middle Aged ; Neurologic Manifestations ; Paraparesis ; Paraparesis, Spastic ; Spinal Canal ; Spinal Cord ; Spinal Cord Compression ; Subarachnoid Space

A 42-year-old female was admitted with an 11-month history of progressive spastic paraparesis and ataxic gait. Magnetic resonance imaging showed intraspinal space occupying lesion compressing the spinal cord posteriorly, located from C5 to T2 with iso to high signal intensity at T2-weighted images and high signal intensity at T1-weighted images. The patient underwent surgery for decompression of the affected spinal cord because of the progressive neurological deficit. At surgery, the lesion was intradural extramedullary lipoma composed with mature adipose tissue. Partial tumor removal to decompress the neural structures and laminoplasty to avoid postoperative instability and deformity were performed. Postoperatively, she demonstrated improvement in paraparesis and was able to walk without assistance. Though attempts to decrease the size of or even to totally remove a lipoma are not required to achieve satisfactory results and carry considerable risks of surgical morbidity, a careful and limited decompression of the affected spinal cord through a partial removal of the tumor and laminoplasty could result in a significant neurological improvement.
Adipose Tissue ; Adult ; Congenital Abnormalities ; Decompression ; Female ; Gait ; Humans ; Lipoma* ; Magnetic Resonance Imaging ; Paraparesis ; Paraparesis, Spastic ; Spinal Cord

Hepatic myelopathy is a rare neurological complication of chronic liver disease, and is characterized by a progressing spastic paraparesis without any sensory loss. However, a few recent reports suggest that involvement of the sensory system is also possible in hepatic myelopathy. We present a patient with hepatic myelopathy, who had impaired proprioception and a delayed cortical response of somatosensory evoked potentials. This supports the hypothesis that hepatic myelopathy may involve the sensory system as well as the motor system.
Evoked Potentials, Somatosensory ; Humans ; Liver Diseases ; Paraparesis, Spastic ; Proprioception ; Spinal Cord Diseases

We report the first Korean case of HTLV-I associated myelopathy (HAM), which was confirmed by Western blot assay of serum and cerebrospinal fluid. Interestingly, the proband's wife was a Japanese Korean who had lived in the endemic area of HAM, Kyushu. Japan, Investigations revealed significantly elevated anti HTLV-I antibody titers in the serum, but not in the CSF, suggesting that she was a carrier of HTLV-I. Considering that the patient had not had a previous blood transfusion, the most common route of HTLV-I, it is likely that the patient was infected by his wife through sexual intercourse. Although previous reports suggest that the transmission of HTLV-I is essentially from man to man or man to woman, our case suggests that woman to man transmission also occurs.
Adult ; Blotting, Western ; HTLV-I Antibodies/analysis ; Human ; Korea/epidemiology ; Male ; Paraparesis, Tropical Spastic/*epidemiology/immunology

OBJECTIVETo screen all ten genes between D15S971 and D15S1012 in five Chinese families with hereditary spastic paraplegia with thin corpus callosum (HSP-TCC).

METHODSDNA samples from 5 HSP-TCC families were screened for mutations in AK128197, MGC14798, HH114, MEIS2, MGC35118, SPRED1, AK128458, FLJ38426, RASGRP1 and AK093014 on chromosome 15q13-15 between microsatellites D15S971 and D15S1012 by polymerase chain reaction, direct sequencing and cosegreagation analysis.

RESULTSNo disease-causing mutations were found in the 10 genes, but 13 polymorphisms were identified in which two were novel.

CONCLUSIONThis study did not support the ten genes between D15S971 and D15S1012 were the disease-causing genes of the 5 HSP-TCC families.

Adult ; Asian Continental Ancestry Group ; genetics ; Chromosomes, Human, Pair 15 ; Corpus Callosum ; pathology ; Female ; Genes, Recessive ; Humans ; Male ; Paraparesis, Spastic ; genetics ; Spastic Paraplegia, Hereditary ; complications ; genetics