Search PubMed⌕ Search

Biomedical subjects

C S Lu

Publications and source records attributed to C S Lu.

At least 73 records · Page 4Linked to original sources

Delayed-onset dystonia following recovery from central pontine myelinolysis.

We report on a 47-year-old female patient with Sheehan's syndrome who developed delayed-onset dystonia following recovery from central pontine myelinolysis. The dystonia was observed in the head, neck and limbs during active movement. MRI scan demonstrated a discrete lesion in the central pons without involving the basal ganglia. The action dystonia was markedly improved by giving trihexyphenidyl. The patient showed that generalized dystonia can be induced by an isolated pontine myelinolysis. There have been four other cases reported in the literature. On reviewing all five cases, we conclude that delayed-onset dystonia following central pontine myelinolysis usually presents with generalized action dystonia and has a beneficial response to trihexyphenidyl.

Demyelinating Diseases↗

Hemiparkinsonism in a patient with frontal meningioma.

We report on a 44-year-old woman with a right frontal meningioma, who presented with resting tremor, rigidity and bradykinesia in the left limbs. There were no other neurologic manifestations. A computed tomography scan demonstrated a huge high-density mass in the right frontal lobe and marked surrounding edema causing compression of the basal ganglia. Cerebral angiography showed a typical sunburst tumor stain and three feeding vessels from the bilateral middle meningeal arteries and the right callosomarginal artery. The pathologic diagnosis was transitional type meningioma. Before surgery, treatment with levodopa and bromocriptine was significantly effective in controlling hemiparkinsonism, which completely disappeared after surgical removal of the tumor. This outcome supports the notion that local compression due to edema may cause a functional disorder in the basal ganglia producing reversible contralateral parkinsonism.

Adult↗

Idiopathic palatal myoclonus: report of two cases.

We report two patients with idiopathic palatal myoclonus presenting as a synchronous up-and-down beating of the soft palate, to-and-fro thrusting of the tongue, and contraction of pharyngeal and laryngeal muscles. The obvious pulsatile movements were visible on the submental surface and a loud clicking noise was audible from a distance. The involuntary jerkings predominantly appeared when the mouth was kept shut, but could be interrupted by some oro-lingual maneuvers such as phonation, swallowing, and sticking the tongue out. Although the palatal myoclonus did not affect the function of the mouth, the patients were socially embarrassed by the loud clicking sound and pulsatile movements. On clinical examination and laboratory investigations, no evidence of lesion in the brainstem or cerebellum was obtained. A therapeutic trial with trihexyphenidyl did not bring any benefit. To conclude, because the idiopathic palatal myoclonus is a rare movement disorder which has never been documented in Taiwan, we describe its clinical characters in two Chinese patients and discuss the differential diagnosis with other involuntary lingual movements.

Adult↗

Effects of alcohol on myoclonus and somatosensory evoked potentials in dyssynergia cerebellaris myoclonica.

Three brothers with dyssynergia cerebellaris myoclonica received alcohol to study the correlation between improvement of myoclonus and alteration in somatosensory evoked potentials (SEPs). Alcohol considerably improved myoclonus for about six hours in two patients (cases 1 and 2) but had only a mild effect in one (case 3). All three patients had giant cortical SEPs. The amplitudes of median N20-P25 and P25-N35 components and tibial N30-P40 and P40-N50 components were considerably decreased after alcohol ingestion in two patients (cases 1 and 2) but unchanged or slightly decreased in one (case 3). The peak latencies of those components were not affected by alcohol. There was thus a good correlation between the suppression of myoclonus and the decrease in giant SEP amplitude.

Adult↗

Congenital mirror movement: report of a case.

We report the case of a 27-year-old woman who has had mirror movements in both hands since childhood. These synkinetic involuntary movements impair her performance in bimanual activities such as buttoning clothes, tying shoes, and washing dishes. Cranial magnetic resonance imaging disclosed only mild diffuse cortical atrophy. Although congenital mirror movements have been well-documented in the English literature, we herein report the first case of a Chinese patient in Taiwan.

Adult↗

Early onset parkinsonism in Chinese.

We describe the clinical features of parkinsonism in 25 patients whose age of onset was under 40 years. Among them, 17 patients, whose age of onset was after their 21st birthday, were classified as young onset Parkinson's disease (YOPD), and the remaining 8 whose age of onset was before their 21st birthday were classified as juvenile parkinsonism (JP). Rigidity and akinesia were revealed in all 25 patients. Resting tremor was observed in only 5 patients; 3 in the YOPD group and 2 in the JP group. There were 8 of the 25 patients (32%) who experienced an aching sensation in the leg before or at the onset of the parkinsonian features. In 6 of these 8 cases, the sensory symptoms were on the same side where the clinical manifestations of parkinsonism later developed. In the JP group, 2 patients had right foot dystonia, which improved with levodopa. Diurnal fluctuations in parkinsonian symptoms were found in 9 of the 25 cases. The familial incidence of parkinsonism was higher in the JP group. The parkinsonian disabilities in all 25 cases responded dramatically to levodopa therapy. Unfortunately, 10 cases, 5 in the YOPD group and 5 in the JP group, developed dyskinesia. The longer they took levodopa, the greater the chance of developing dyskinesia. The cumulative percentage of dyskinesia was 100% in the YOPD group and 83% in the JP group by the seventh and fourth year of treatment, respectively. A positive correlation was found between the prevalence of dyskinesia and the duration of treatment in both groups.

Adolescent↗

Progressive myoclonic ataxia (the Ramsay Hunt syndrome).

It has been suggested from studies of patients with progressive myoclonus epilepsy that the term Ramsay Hunt syndrome should be abandoned, as its use has led to nosologic confusion, and because, in the light of modern diagnostic techniques, the majority of cases can be allocated to specific disease categories, chiefly, Unverricht-Lundborg disease (Baltic myoclonus) and mitochondrial encephalomyopathy. Review of 30 cases of this syndrome, defined as progressive ataxia and myoclonus and infrequent seizures in the absence of dementia, showed that a clinical or biochemically supported diagnosis could not be made in 43%. This low diagnostic yield probably reflects differences in ascertainment of patients; those described here were referred with a syndrome of progressive myoclonic ataxia (the Ramsay Hunt syndrome) rather than progressive myoclonus epilepsy. These two syndromes share common causes, but a smaller proportion of patients with progressive myoclonic ataxia can currently be diagnosed precisely during life.

Adolescent↗

Parkinsonism with neuroacanthocytosis.

Two patients with neuroacanthocytosis are described. One presented with parkinsonism and the other resembled diurnal dystonia of the Segawa type. Both patients responded well to dopaminomimetic therapy. A PET scan with fluorodopa revealed a nigrostriatal deficit in the first patient.

Acanthocytes↗

Oculopalatal myoclonus: report of a case.

We report a patient who developed a delayed onset of oculopalatal myoclonus, characterized by synchronous oscillatory movements of the eyeballs, soft palate and the orbicularis oculi muscle, 5 months after a pontine hemorrhage. The involuntary oscillations did not affect the speech or swallowing but continued in sleep with decreased amplitudes. These oscillations also failed to respond to clonazepam therapy and the patient was unable to voluntarily suppress them for several minutes. A temporary association in synchronous titubation of the head was also observed. A characteristic vertical ocular oscillation was demonstrated in an oculogram. This is in accordance with the midline form of oculopalatal myoclonus.

Cerebral Hemorrhage↗

Chronic manganese intoxication.

We report six cases of chronic manganese intoxication in workers at a ferromanganese factory in Taiwan. Diagnosis was confirmed by assessing increased manganese concentrations in the blood, scalp, and pubic hair. In addition, increased manganese levels in the environmental air were established. The patients showed a bradykinetic-rigid syndrome indistinguishable from Parkinson's disease that responded to treatment with levodopa.

Adult↗

Ramsay Hunt syndrome and coeliac disease: a new association?

Two patients with the syndrome of Ramsay Hunt (dyssynergia cerebellaris myoclonica, DCM), associated with malabsorption due to adult coeliac disease, are reported. Both presented with progressive cerebellar ataxia, action myoclonus, and epilepsy. One had gastrointestinal symptoms (recurrent diarrhea and weight loss which responded satisfactorily to a gluten-free diet), but the other did not. In both patients, jejunal biopsy revealed subtotal villous atrophy; serum folate and vitamin E level were also reduced. Neither a gluten-free diet nor vitamin supplements improved the neurological picture. However, some symptomatic relief was afforded by treatment with clonazepam, sodium valproate, carbamazepine, and piracetam. It could be argued that the association between these two disorders is coincidental. However, since we have found this combination in 2 of 14 consecutive cases with DCM, a causal relationship seems likely, although the underlying mechanism remains unknown. Patients with the Ramsay Hunt syndrome should be investigated for malabsorption, and also undergo small intestinal biopsy.

Adult↗