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At least 487 records · Page 27Linked to original sources
PubMed1996

Phase II trial of vinorelbine in metastatic squamous cell esophageal carcinoma. European Organization for Research and Treatment of Cancer Gastrointestinal Treat Cancer Cooperative Group.

PURPOSE: To evaluate the response rate and toxic effects of vinorelbine (VNB) administered as a single agent in metastatic squamous cell esophageal carcinoma. PATIENTS AND METHODS: Forty-six eligible patients with measurable lesions were included and were stratified according to previous chemotherapy. Thirty patients without prior chemotherapy and 16 pretreated with cisplatin-based chemotherapy were assessable for toxicity and response. VNB was administered weekly as a 25-mg/m2 short intravenous (i.v.) infusion. RESULTS: Six of 30 patients (20%) without prior chemotherapy achieved a partial response (PR) (95% confidence interval [CI], 8% to 39%). The median duration of response was 21 weeks (range, 17 to 28). One of 16 patients (6%) with prior chemotherapy had a complete response (CR) of 31 weeks' duration (95% CI, 0% to 30%). The overall response rate (World Health Organization [WHO] criteria) was 15% (CR, 2%; PR 13%; 95% CI, 6% to 29%). The median dose-intensity (DI) was 20 mg/m2/wk. VNB was well tolerated and zero instances of WHO grade 4 nonhematologic toxicity occurred. At least one episode of grade 3 or 4 granulocytopenia was seen in 59% of patients. A grade 2 or 3 infection occurred in 16% of patients, but no toxic deaths occurred. Other side effects were rare, and peripheral neurotoxicity has been minor (26% grade 1). CONCLUSION: These data indicate that VNB is an active agent in metastatic esophageal squamous cell carcinoma. Given its excellent tolerance profile and low toxicity, further evaluation of VNB in combination therapy is warranted.

Abdominal Pain↗
PubMed2003

Primary lateral sclerosis: A heterogeneous disorder composed of different subtypes?

OBJECTIVE: To determine identifiable subgroups of patients with primary lateral sclerosis (PLS) with distinct clinical features as a first step in identifying patients likely to have the same disorder. METHODS: Twenty-five patients meeting previously proposed diagnostic criteria for PLS were seen for examination, measurement of gait and finger tapping speed, and physiologic tests to assess motor pathways. Motor cortex excitability and central motor conduction time were assessed with transcranial magnetic stimulation. Brainstem motor pathways were assessed by the acoustic startle reflex. MRS was performed in a subgroup of patients to assess metabolites in the motor cortex. RESULTS: Fifty-six percent of the patients with PLS had a similar pattern of symptom progression, which the authors termed ascending. In these patients spasticity began in the legs and progressed slowly and steadily. Spasticity in the arms developed 3.6 years after the legs, on average, and speech impairment followed 1.5 years later. Motor evoked potentials were absent. MRS showed a mean reduction of N-acetylaspartate/creatinine in the motor cortex. The remaining patients with PLS had heterogeneous patterns of symptom progression and physiology. CONCLUSIONS: Patients with PLS with an ascending progression of symptoms form a distinct clinical subgroup that may be amenable to investigations of etiology and treatment.

Adult↗
PubMed2003

Vestibular perception in patients with acquired ophthalmoplegia.

Using a perceptual technique it is shown that patients with chronic external ophthalmoplegia have shortened vestibular responses. It is postulated that this is secondary to the retinal image slip experienced by these patients during head movements and a useful compensatory mechanism to suppress motion-induced sickness and spatial disorientation.

Adult↗
PubMed2004

Preserved visual-vestibular interaction in patients with bilateral vestibular failure.

BACKGROUND: During caloric vestibular stimulation, subjects showed bilateral activation of the vestibular cortex in the posterior insula and retroinsular region as well as concurrent deactivation of visual cortex areas bilaterally. This finding was the basis for the concept of a reciprocal inhibitory interaction between the vestibular and the visual systems. OBJECTIVE: To analyze the modulations of this activation and deactivation pattern in patients with loss of vestibular input, that is, in patients with bilateral vestibular failure (BVF). METHODS: Modulations of regional cerebral blood flow (rCBF) in PET were measured in nine patients with BVF and compared with those in healthy volunteers using statistical group as well as single-subject analyses (Statistical Parametric Mapping 96b). RESULTS: The group analysis of the BVF patients showed only one small region of activation in the posterior insula contralateral to the stimulated ear, whereas the other areas correlating with vestibular, autonomic, and ocular motor function were not activated. Furthermore, the concurrent rCBF decreases of the primary visual cortex seen in healthy volunteers were not found in the patients. These decreases seem to be dependent on an intact vestibular input with concurrent vestibular nystagmus. CONCLUSIONS: The results are compatible with the concept of a reciprocal inhibitory sensorisensory interaction between the vestibular and visual systems that normally act together for orientation in space and perception of motion. This interaction appears to be preserved in the patients at a significantly lower level, that is, with less activation and less deactivation.

Adult↗
PubMed2004

Vertical vestibular responses to head impulses are symmetric in downbeat nystagmus.

BACKGROUND: Downbeat nystagmus (DBN), a frequent ocular motor sign often caused by cerebellar lesions, is a fixational nystagmus with fast phases directed downward. Its precise etiology is not known. One hypothesis is that it is caused by a central imbalance of pathways of the vertical vestibulo-ocular reflex (VOR). Such an imbalance would cause not only ocular drift but also deficient and asymmetric vertical VOR responses. OBJECTIVE: To test this hypothesis, the authors analyzed the functionally relevant VOR responses to head impulses in the roll, pitch, and yaw planes. METHODS: Head and eye movements were measured with the search-coil method in 10 patients with DBN and 10 age-matched control subjects. RESULTS: Analysis revealed no gain difference in patients with DBN compared with control subjects. Specifically, upward and downward VOR responses in patients with DBN in the pitch plane were symmetric. CONCLUSIONS: These findings do not support the hypothesis of a vertical VOR imbalance and put into question the view that DBN is a central vestibular syndrome in the sense of vestibular dysfunction. Although DBN possibly involves vestibulocerebellar pathways, in the patients that we studied, DBN did not affect the immediate VOR responses in the high-frequency range that corresponds to natural head movements.

Aged↗
PubMed2004

Two novel mutations of the glycine receptor gene in a Taiwanese hyperekplexia family.

The authors report a Taiwanese family with autosomal recessive hyperekplexia. Two novel mutations, W96C (from the paternal allele) and R344X (from the maternal allele), which are located in exon 4 and exon 7 of the GLRA1 gene, were identified in this family. A series of electrophysiologic investigations were conducted in one of the probands, and the results suggest that the "startle center" is located subcortically.

Acoustic Stimulation↗
PubMed1979

Peripheral neuropathy in cerebrotendinous xanthomatosis.

Four patients with cerebrotendinous xanthomatosis (CTX) underwent electrophysiologic investigations, which demonstrated impairment in the functioning of the peripheral nerves in all four cases. The changes consisted of slow motor and sensory conduction. The changes were most marked in the older subjects, in whom the disease was more advanced, and who also had clinical manifestations of mild peripheral neuropathy. We conclude that the peripheral nerves are damaged in CTX.

Adolescent↗
PubMed1986

Autonomic dysfunction in Parkinson's disease.

We studied autonomic functions in 31 chronically treated patients with Parkinson's disease. They were tested twice: before a dose of medication and after medication. Before a dose of medication, when motor disability was maximal ("off"), patients had higher resting pulse rate, greater orthostatic fall in blood pressure, and decreased responses to Valsalva and cold pressor stimuli than their spouse-controls. To a heat stimulus, sweating was increased in the head and neck, and skin temperatures were cooler. After medication when function was optimal ("on"), the cardiovascular reflex abnormalities remained but were no worse. Skin temperature alterations and sweating abnormalities resolved.

Adult↗
PubMed1990

Autosomal dominant cerebellar ataxia: clinical analysis of 263 patients from a homogeneous population in Holguín, Cuba.

We describe 263 patients with autosomal dominant cerebellar ataxia from the Holguín province, Cuba. There is evidence of a common ancestry and the population represents the largest homogeneous group of patients yet described. Primary features include gait ataxia, dysarthria, dysmetria, adiadochokinesia, cramps, tremor, hypotonia, abnormal reflexes, and slowed/limited eye movements. Age at onset ranged from 2 to 65 years. There was considerable clinical variability within the families. No patients had optic atrophy, spasticity, pigmentary retinal degeneration, or cogwheel rigidity, and only 1 had dementia.

Adolescent↗