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Biomedical subjects

L S Ro

Publications and source records attributed to L S Ro.

At least 19 recordsLinked to original sources
PubMed2000

Transient global amnesia and amaurosis fugax in a patient with common carotid artery occlusion--a case report.

The etiology of transient global amnesia (TGA) is debatable. The hypothesis of a thromboembolic cause of TGA has been questioned by recent case control studies. Occlusion of the common carotid artery (CCA) is rare. Although amaurosis fugax (AF) is a hallmark of ipsilateral internal carotid artery disease, its occurrence in CCA occlusion is less known. Association of these three conditions in a patient may imply pathophysiologic significance. Here, the authors report a 76-year-old man who suffered from a spell of TGA and then several attacks of AF of the right eye. Progressive occlusion of the right CCA was documented by repeat carotid duplex scans and was finally confirmed by cerebral angiography. In this patient, the occurrence of CCA occlusion and AF implicates a vascular etiology for the event of TGA. A noninvasive carotid ultrasonographic screen may, therefore, be worthwhile for patients with TGA.

Aged↗
PubMed2000

gamma-sarcoglycan deficiency muscular dystrophy in two adults.

All dystrophin-associated proteins contain sarcoglycan complex. Different forms of muscular dystrophy are caused by defective expression of different proteins of this structure. gamma-Sarcoglycan deficiency muscular dystrophy, so-called severe childhood autosomal recessive muscular dystrophy (SCARMD), is a rare disease that has not been previously reported in Taiwan. This paper describes two Taiwanese adults with this disease: a 26-year-old man with calf pseudohypertrophy who had weakness in both legs for 1 year; and a 43-year-old woman who had progressive weakness in all four limbs, with the initial symptom of gait disturbance at the age of 32 years. Analysis of muscle biopsy specimens, which showed total deficiency of gamma-sarcoglycan protein on immunostaining, confirmed the diagnosis of SCARMD in both cases. However, the clinical manifestations in these two patients, including lower proximal limb weakness initially developing in adulthood with a slow progressive course, are different from previously reported cases of SCARMD. The literature on this disease is reviewed and possible mechanisms of these distinct clinical presentations are discussed.

Adult↗
PubMed1999

Current perception threshold testing in Fabry's disease.

We investigated 16 patients with Fabry's disease (eight hemizygous men and eight heterozygous women) in one family. We used constant current perception threshold (CPT) testing, which evaluated three major sensory nerve fiber populations, to assess subjective complaints of pain and paresthesias. We also examined clinical and biochemical features and compared the values of CPTs and nerve conduction studies (NCS) in detecting the sensory neuropathy. Our results showed that CPT testing at low frequencies (5 and 250 Hz) was significantly more sensitive than at a higher frequency (2 kHz) and NCS in detecting sensory neuropathy in patients with Fabry's disease. However, there was no correlation between CPT testing and clinical symptom scores, duration of disease, creatinine clearance (Ccr) values or alpha-galactosidase A (AGA) activities in either hemizygous or heterozygous patients. Hemizygous patients clinically demonstrated more severe symptom scores, poorer renal function, and higher prevalence of hypohidrosis and corpora angiokeratomas than did heterozygous patients, which indicates that detailed clinical examinations can differentiate the clinical status of hemizygous men from heterozygous women. There were no associations between the biochemical levels of serum AGA activity and renal function (Ccr values) or the symptom scores (grading of acroparesthesia), indicating that biochemical parameters do not predict clinical severity.

Adolescent↗
PubMed1999

Effect of NGF and anti-NGF on neuropathic pain in rats following chronic constriction injury of the sciatic nerve.

The systemic administration of anti-nerve growth factor (NGF) antibodies can prevent local sensory hypersensitivity and block nociceptive fibers from sprouting into denervated adult rat skin. However, in the case of chronic constriction injury (CCI) in a rat, there is evidence that NGF reverses some effects of axotomy and alleviates thermal hyperalgesia. It is with this in mind that we investigated the influence of local anti-NGF and NGF on neuropathic pain and collateral sprouting caused by CCI. In our study, we looked at the effects to the ligated nerves after 30 consecutive days of local injections of anti-NGF and NGF. A high-dose of anti-NGF (1800 ng) was found to eradicate heat and cold hyperalgesia during postoperative days 16-28 and from days 8 to 34 after CCI, respectively. Our results show that a low-dose anti-NGF (18 ng) only mildly alleviates heat hyperalgesia but not cold hyperalgesia. There is evidence that a rebound phenomenon occurs for a short period of time after the anti-NGF injections cease. Results show that anti-NGF injections, whether in a high or low dose, significantly reduces the severity of autotomy or prevents the spread of collateral sprouting from the saphenous nerve into the sciatic innervation territory. In contrast, when a NGF (0.75 ng/g body weight) was applied to the ligated nerve immediately after the ligation, heat and cold hyperalgesia were eradicated during postoperative days 4-68 and from days 4 to 28, respectively. The results show that the effect of anti-NGF is delayed at the onset, is short in duration, and is dependent on the dosage. However, anti-NGF but not NGF blocked collateral sprouting and decreased the severity of autotomy, suggesting that anti-NGF may be a better potential alternative analgesic for the treatment of neuropathic pain in humans. The different initiation times to abolish thermal hyperalgesia by anti-NGF (delayed onset) and NGF (early onset) suggests that alterations in neurotrophic factors contribute to the development of behavioral hyperalgesia via a complex mechanism in CCI rats.

Animals↗
PubMed1999

Identification of five spinocerebellar ataxia type 2 pedigrees in patients with autosomal dominant cerebellar ataxia in Taiwan.

OBJECTIVES: The autosomal dominant cerebellar ataxias (ADCAs) are a group of genetically diverse neurological conditions linked by progressive deterioration in balance and coordination. Spinocerebellar Ataxia Type 2 (SCA2) is one of the ADCAs and also belongs to a special group caused by the expansion of an unstable CAG repeat encoding a polyglutamine tract. We aimed to investigate the frequency of SCA2 mutation in the ataxia patients referred to the clinic. MATERIALS AND METHODS: We screened 58 families with inherent cerebellar ataxia and 57 normal individuals by the use of radioactive genomic polymerase chain reaction (PCR) method. A simple non-radioactive PCR for rapid detection of the expanded SCA2 alleles via agarose gel electrophoresis was also employed. RESULTS: Eight SCA2 affected patients and 1 at-risk individual in 5 unrelated SCA2 families were identified. The CAG repeats of normal alleles in the sample studied range in size from 16 to 30 repeat units, while those of SCA2 chromosomes are expanded to 34 to 49 repeat units. Our results also showed that unlike SCA 1 and SCA3/MJD, the size distribution of the normal alleles showed few polymorphisms, with the 22 repeat allele accounting for 90.1%. Homozygosity in normal individuals was 80.2%. No overlap in ataxin-2 allele size between normal and expanded chromosomes was observed. CONCLUSION: This is the first report of the SCA2 gene distributions in the population of Taiwan. The SCA2 mutation accounts for 8.6% of ADCA type I families referred to us, intermediate between SCA1(1.7%) and SCA3/MJD (24%) of the ADCA type I families in our collection.

Adolescent↗
PubMed1998

Hirayama disease: MR diagnosis.

We report the MR findings in two cases of Hirayama disease, a kind of cervical myelopathy related to flexion movements of the neck. In flexion MR studies, we can see the striking and pathognomonic picture of anterior shifting of posterior dura at the lower cervical spinal canal. In nonflexion studies, we find that asymmetric cord atrophy, especially at the lower cervical cord, though subtle, is highly suggestive of Hirayama disease. When it is seen, a flexion MR study is warranted to prove this diagnosis.

Adolescent↗
PubMed1998

Extent of collateral sprouting of intact nerve fibers in rats depends on the local availability of nerve growth factor.

We investigated the influence of local administration of anti-nerve growth factor (NGF) serum on collateral nerve fiber sprouting caused by chronic constriction injury (CCI) of the sciatic nerve in rats. CCI was induced in adult Sprague-Dawley rats (six per group) by constriction of the sciatic nerve of the right hind limb. Control rats received no further manipulation. In four other groups, rabbit preimmune (control) serum or low (18 ng) or high (1800 ng) doses of anti-NGF serum were injected into the operated or unoperated hind limb each day for 1 month. We used an Evans dye-labeled plasma extravasation technique to visualize the extent of collateral sprouting. Local injection of anti-NGF serum at both high and low doses, but not control serum, significantly reduced the spread of collateral sprouting from the saphenous nerve into the sciatic nerve innervation territory. High-dose anti-NGF serum did not block collateral sprouting when injected into the contralateral (unoperated) hind limb, indicating that the inhibition of sprouting was not caused by a systemic effect. In conclusion, local administration of anti-NGF serum can block sprouting of collateral fibers after nerve injury. Our findings suggest that collateral sprouting is dependent on the local availability of NGF to nearby intact cutaneous nerve fibers.

Animals↗
PubMed1998

Lhermitte-Duclos disease: first report in Taiwan.

We report a case of Lhermitte-Duclos disease (dysplastic gangliocytoma of the cerebellum), an uncommon disorder of uncertain pathogenesis characterized by disarrangement of the normal cerebellar laminar cytoarchitecture. A 40-year-old man was admitted because of vomiting and syncope of a few days' duration, and a 2-month history of intermittent headaches and unsteady gait. A computed tomographic scan of the patient's head showed obstructive hydrocephalus due to displacement of the fourth ventricle by a large, nonenhancing cerebellar mass. The magnetic resonance images of the brain also revealed a space-occupying lesion within the right cerebellum with unusual septation. After surgery, the histologic examination confirmed the diagnosis of Lhermitte-Duclos disease. This is the first report of Lhermitte-Duclos disease in Taiwan.

Adult↗
PubMed1997

The MRI signs of spinal arachnoid diverticula.

Our goal was to find MRI signs of use for identifying a spinal arachnoid diverticulum. Three cases of spinal arachnoid diverticula, one extradural and two intradural, were examined on a 1.5 T imager. There was obvious mass effect on the adjacent structures in one case and increased signal intensity in the diverticulum on proton density- and T2-weighted images in two cases. Signal changes due to turbulent movement of the spinal fluid inside the diverticula were seen in all cases on sagittal fast spin-echo (FSE) proton density- and T2-weighted images; it was difficult to tell whether these signal changes imply a communication or are simply FSE artefacts. On contrast-enhanced studies, all cases showed partial enhancement inside the diverticula. There thus are four signs of diverticula: mass effect, the increased signal, signal void sign and partial enhancement; the last of these, the most reliable, has never been reported before.

Adult↗
PubMed1996

Local application of anti-NGF blocks the collateral sprouting in rats following chronic constriction injury of the sciatic nerve.

Systemic administration of anti-nerve growth factor (NGF) antibodies can block nociceptive fiber sprouting into denervated adult rat skin. However, the effect of local application of anti-NGF on collateral sprouting in rats caused by chronic constriction injury (CCI) has not been well studied. We investigated the influence of local anti-NGF on collateral sprouting caused by CCI of the rat's sciatic nerve. Using a dye-labeled plasma extravasation technique, we can easily evaluate the extent of collateral sprouting by the clearly defined blue coloration area. Our results showed that local application of anti-NGF either in a high or low dose significantly prevented the spread of collateral sprouting from the saphenous nerve into the sciatic innervation territory. In contrast, distilled water did not show a significant block of the saphenous nerve collateral sprouting. Our study suggests that collateral sprouting is dependent on the local availability of NGF by the nearby intact cutaneous nerve fibers.

Animals↗
PubMed1996

Balò's concentric sclerosis: MRI.

We report a case of Balò's concentric sclerosis. In the past, the diagnosis was usually made post mortem. In this case, we found that CT demonstrated only low-density lesions, but not their concentric pattern. However, MRI showed a striking feature, reminiscent of histopathology of Balò's concentric sclerosis, and played an important role in antemortem diagnosis of this rare demyelinating disease.

Basal Ganglia↗
PubMed1996

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↗
PubMed1996

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↗
PubMed1996

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↗
PubMed1996

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↗
PubMed1995

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↗
PubMed1995

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↗