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Biomedical subjects

L S Ro

Publications and source records attributed to L S Ro.

30 records · Page 2Linked to original sources

Central gadolinium enhancement of an acute spontaneous spinal epidural haematoma.

Spontaneous spinal epidural haematoma (SSEH) is uncommon, but central gadolinium (Gd) enhancement of the haematoma is even rarer. To our knowledge, only one case has been reported, but no explanation was given concerning the mechanism of this enhancement. We present a case of SSEH with unusual central Gd enhancement which we believe is due to oozing from the injured posterior internal venous plexuses. Therefore, although rare, central Gd enhancement of an epidural mass should not rule out the possibility of a haematoma.

Female↗

Juvenile-onset of Dejerine-Sottas disease in a Taiwanese woman.

Dejerine-Sottas disease is an uncommon hereditary neuropathy which has not been reported in Taiwan. We describe a 57-year-old woman who had slowly progressive weakness in her four limbs since adolescence. None of her close relatives had the disease and no consanguinity was noted. Neurologic examination showed severe weakness and vibratory sensation loss in the four limbs. The tendon reflexes were generally absent. Electrophysiologic studies suggested a systemic myelinopathic process. Light and electron microscopy of the sural nerve biopsy specimens revealed many onion-bulb shapes formed by the Schwann cell processes or basement membranes without any evidence of myelin sheaths around the axons, which are characteristic features of Dejerine-Sottas disease.

Age of Onset↗

Vocal cord paralysis as an initial sign of multiple system atrophy in the central nervous system.

Multiple system atrophy (MSA) of the central nervous system has been recognized as a rare cause of stridor secondary to vocal cord paralysis. This respiratory problem usually occurs in the later stages of the disease. We report the case of a 53-year-old man who presented with bilateral vocal cord paralysis accompanied by stridor and sleep apnea, which were relieved by a tracheostomy. Two years after the onset of stridor, he developed extrapyramidal and cerebellar signs combined with severe autonomic failure. Magnetic resonance imaging of the brain revealed atrophy of the brainstem and cerebellum. This clinical picture is consistent with the diagnosis of MSA. Vocal cord paralysis preceding any other neurologic and autonomic manifestations has been infrequently described. This case should remind clinicians that MSA should be considered in the differential diagnosis of vocal cord palsy of undetermined origin.

Atrophy↗

Idiopathic hypereosinophilic syndrome with eosinophilic myositis, peripheral neuropathy and central nervous system involvement.

Idiopathic hypereosinophilic syndrome (HES) is a rare disorder marked by a sustained overproduction of eosinophils and a predilection for damage to multiple organ systems. Its neurologic involvement ranges from the central to the peripheral nervous system, and can be associated with eosinophilic myositis. We report a 68-year-old woman who had eosinophilia, eosinophilic dermatitis and eosinophilic pneumonia. She also suffered from numbness and weakness of the lower limbs. Because of long-lasting (> 6 mo) eosinophilia (> 1.5 x 10(9)/L) in the peripheral blood and the fact that no other underlying causes of eosinophilia and neurologic involvement could be identified, a diagnosis of idiopathic hypereosinophilic syndrome was made. The muscle biopsy showed infiltration of inflammatory cells, including a few eosinophils (Liu's stain). Magnetic resonance images, motor evoked potentials, somatosensory evoked potentials and nerve conduction velocities also showed abnormalities in the central and peripheral nervous systems. The pathogenesis and treatments of HES are discussed in this report.

Central Nervous System Diseases↗

Brain abscess as a complication of stroke.

BACKGROUND: Systemic infection is a common complication of stroke. However, brain abscess as a complication of stroke has never been reported. CASE DESCRIPTIONS: We report two patients, one with a hypertensive intracerebral hemorrhage and the other with nonseptic cerebral infarction. In both patients, brain abscess developed at the stroke lesion site after an infectious complication. After surgical aspiration and antibiotic treatment, one patient recovered and the other died. CONCLUSIONS: These two cases demonstrate that brain abscess may occur in a previous hemorrhage or infarction area as a complication of systemic infection. Recognition of the risk is important for early diagnosis and proper treatment of this potentially fatal complication of stroke.

Aged↗

Concurrent trigeminal, abducens, and facial nerve palsies presenting as false localizing signs: case report.

Multiple cranial nerve dysfunction presenting as false localizing signs is rare. We report a 20-year-old woman who had concurrent trigeminal sensory disturbance, abducens, and peripheral facial nerve palsies in association with a contralateral acoustic neuroma. After surgery, the trigeminal nerve disturbance and the abducens nerve palsy completely recovered, but the peripheral facial nerve palsy persisted. The nature of tumor, the presence of brain stem distortion, the anatomic variation of posterior fossa, and the relationships of cranial nerves and nearby blood vessels, which are factors influencing the occurrence of false localizing, are briefly discussed.

Abducens Nerve↗

Fabry's disease: report of a case.

Fabry's disease is a rare hereditary disorder of glycosphingolipid metabolism. Its clinical features have not been adequately described in Taiwan. This paper reports on a 32-year-old man who had painful acroparesthesia, disseminated skin angiokeratomas, whorled corneal opacity, mitral valve prolapse and renal insufficiency. There was also involvement of the central motor pathways and the autonomic nervous system. A sural nerve biopsy showed loss of small myelinated and unmyelinated fibers. A reduced serum activity of alpha-galactosidase A and a large amount of urinary globotriaosylceramide confirmed the diagnosis of Fabry's disease.

Adult↗

A morphological study of experimental mononeuropathy in the rat: early ischemic changes.

This paper describes early morphological changes following the placement of four loose ligatures round the rat sciatic nerve, a method used to produce altered pain-related behaviour. The ligatures were tied so as not to constrict the nerve, but there was compression of some epineurial blood vessels. This mild lesion caused behavioural changes and nerve fiber abnormalities within 8.5 h. The distribution of nerve fiber changes was patchy, and initially due to stagnant ischemia, with necrosis of Schwann cells and evidence of axonal stasis. By 48 h, endoneurial oedema increased leading to a compressive lesion and eventually to severe nerve fiber degeneration.

Animals↗

MRI/myelographic localization of fistulous tract in spinal dural arteriovenous malformations prior to arteriography.

OBJECTIVE: Our goal was to use currently available imaging methods to localize the fistulous tract of spinal dural arteriovenous malformations (SDAVMs) prior to arteriography. MATERIALS AND METHODS: Two middle-aged men showed MR findings indicative of a SDAVM. In the first patient coronal T2-weighted (T2W) imaging identified the engorged draining radiculospinal vein and traced it back to the intervertebral foramen site where the "nidus" was located. Then, axial T2W imaging was performed at the level of the involved intervertebral foramen. In the second patient MRI failed to demonstrate the nidus; myelography was carried out and showed the course of the abnormal intraspinal vein. RESULTS: Successful MR/myelographic demonstration of the nidus (Case 1) or the abnormal intraspinal vein (Case 2) simplified and abbreviated the later studies. CONCLUSION: With MRI and/or myelography we were able to localize the site of the fistulous tract in the two cases of SDAVM prior to spinal arteriography.

Angiography↗

Serial MRI studies in pathologically verified Balò's concentric sclerosis.

We report serial MRI studies in a patient with Balò's concentric sclerosis, who presented with a progressive weakness in the right limbs of 10 day duration. Serial MR images revealed several concentric lesions characterized by layer by layer of demyelinated and myelinated appearance and multiple plaques in the white matter. Pathological study of a biopsy of a concentric lesion (which was performed to rule out neoplasm) further confirmed this diagnosis. These MR findings resolved in correspondence with clinical improvement. The patient was almost completely normal 1 year later. In conclusion, in this case, we found that MRI could show the striking feature reminiscent of the histopathologic findings of Balò's concentric sclerosis and played an important role in antemortem diagnosis of this rare demyelinating disease.

Adult↗

Epidemiological and genetic studies of myotonic dystrophy type 1 in Taiwan.

To investigate the prevalence and genetic characteristics of myotonic dystrophy type 1 (DM1) in Taiwan, DM-suspected patients and their families identified during the period of 1990-2001 had their clinical records reevaluated and the CTG repeat sizes at the DM1 locus examined. A total of 96 subjects belonging to 26 families were identified as DM1 patients, which gave a minimal disease prevalence of 0.46/100,000 inhabitants. Clinical anticipation was frequently observed in affected families, even in some parent-child pairs with transmission contraction of the CTG repeat size. The inverse correlation between age at onset and CTG repeat length was significant only in patients with small expansions. In addition, a DM1 carrier with a childhood-onset son was found to have CTG length heterogeneity in the range of 40-50, indicating that premutation alleles could be unstable during gametogenesis as well as in somatic tissues. Our data demonstrated that DM1 is a rare disease in Taiwan and showed that transmission contraction of repeat size is more likely to occur in alleles with large repeats.

Adolescent↗