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Biomedical subjects

V E Drory

Publications and source records attributed to V E Drory.

At least 19 recordsLinked to original sources

Evoked potential studies in the antiphospholipid syndrome: differential diagnosis from multiple sclerosis.

BACKGROUND: The CNS manifestations of the antiphospholipid syndrome (APS) can mimic multiple sclerosis both clinically and radiologically. OBJECTIVE: To compare evoked potential studies in APS patients and patients with multiple sclerosis with similar neurological disability. METHODS: 30 APS patients with CNS manifestations and 33 patients with definite multiple sclerosis and similar neurological disability underwent studies of visual evoked potentials (VEP), somatosensory evoked potentials (SSEP) in the upper and lower limbs (UL, LL), and sympathetic skin responses (SSR) in the upper and lower limbs. RESULTS: The neurological manifestations in the APS patients included stroke (n = 17), transient ischaemic attacks (n = 10), and severe headache with multiple white matter lesions on brain MRI (n = 3). Abnormal SSEP (LL), and SSR (UL; LL) were seen in APS patients (37%, 27%, and 30%, respectively) but VEP and UL SSEP were rarely abnormal (10% and 6%, respectively in APS v 58% and 33% in multiple sclerosis; p = 0.0005, p = 0.008). Mean VEP latencies were more prolonged in multiple sclerosis (116 ms v 101 ms, p<0.001). Only one APS patient had abnormal findings in all three evoked potential studies, compared with seven patients in the multiple sclerosis group (p = 0.04) CONCLUSIONS: Abnormal VEPs are uncommon in APS in contrast to multiple sclerosis. Coexisting abnormalities in all other evoked potentials were similarly rare in APS. In patients with brain MRI findings compatible either with multiple sclerosis or APS, normal evoked potential tests, and especially a normal VEP, may support the diagnosis of APS.

Adult↗

R-R interval variation in Parkinson's disease and multiple system atrophy.

OBJECTIVE: To investigate whether the cardiac R-R interval variation (RRIV) is of value in differentiating patients with Parkinson's disease (PD) from multiple system atrophy (MSA). BACKGROUND: RRIV assessment is a simple procedure, reflecting mainly vagal efferent activity. Reduced RRIV was reported in MSA. METHODS: RRIV at rest and after 120 s of deep breathing was assessed blindly to clinical diagnosis in 22 PD and 20 MSA patients. The results were compared with data from 23 age-matched healthy subjects. RESULTS: RRIV at rest was 7.1 +/- 2.7% in PD and 9.7 +/- 7.2% in MSA, increasing after deep breathing to 11.2 +/- 6.3 and 12.3 +/- 6.6% correspondingly. The frequency of the RRIV abnormalities in the PD group (4/22, 18.2%) and MSA (6/20, 30%) were higher than among controls (P < 0.004). CONCLUSIONS: RRIV, either at rest or after deep breathing, may be abnormal both in PD and MSA, but does not distinguish between these disorders.

Aged↗

The value of muscle exercise in patients with amyotrophic lateral sclerosis.

The role of physical activity for patients with amyotrophic lateral sclerosis (ALS) is controversial. Twenty-five ALS patients were randomized to receive a moderate daily exercise program (n=14) or not to perform any physical activity beyond their usual daily requirements (n=11). At baseline and after 3, 6, 9 and 12 months, patients were assessed by manual muscle strength testing, the Ashworth spasticity scale, ALS functional rating scale (FRS), fatigue severity scale, a visual analogue scale for musculoskeletal pain and the quality-of-life scale (SF-36). At 3 months, patients who performed regular exercise showed less deterioration on FRS and Ashworth scales, but not on other parameters. At 6 months, there was no significant difference between groups, although a trend towards less deterioration in the treated group on most scales was observed. At 9 and 12 months, there were too few patients in each group for statistical evaluation. Our results show that a regular moderate physical exercise program has a short-lived positive effect on disability in ALS patients and should be recommended.

Adult↗

Association of APOE epsilon4 allele with survival in amyotrophic lateral sclerosis.

APOE epsilon4 allele is associated with poorer outcome in degenerative neurological diseases. Its role in amyotrophic lateral sclerosis (ALS) is still unclear. The aim of the present study was to further analyze the association of APOE epsilon4 allele with progression and survival of ALS. One hundred consecutive ALS patients (53 males) and 133 controls were genotyped for the APOE epsilon4 allele. The association of this allele with survival to death or tracheostomy was analyzed by Kaplan-Meier survival analysis. The frequency of the APOE epsilon4 allele in ALS patients was slightly higher (15.1%) than in the control group (10.9%). Patients with or without an APOE epsilon4 allele had a similar age of onset and frequency of bulbar onset. There was a significant shortening of the 50% probability of survival (by 32 months) in patients carrying the APOE epsilon4 allele (p=0.03). In conclusion, carrying an APOE epsilon4 allele is a poor prognostic factor in ALS. This is compatible with a role of apolipoprotein on neuronal survival and repair.

Adult↗

Electrophysiologic evaluation of upper motor neuron involvement in amyotrophic lateral sclerosis.

BACKGROUND: The demonstration of upper motor neuron (UMN) dysfunction in patients with amyotrophic lateral sclerosis (ALS) with predominantly lower motor neuron (LMN) signs is clinically sometimes difficult. METHODS: We analyzed the possible role of F and H waves in the diagnosis of UMN dysfunction in 36 patients with different clinical forms of ALS and 20 controls. In both lower limbs we evaluated F-wave persistence and the F/M ratio of tibial nerves, Hmax/Mmax ratio, vibratory and recurrent (paired) inhibition of the H-reflex. RESULTS: The persistence of the F-response was decreased significantly in the LMN group only. The F/M amplitude ratio, vibrated-H/rest-H amplitude ratio and conditioned H/rest H amplitude ratio were significantly increased in patients with predominantly UMN, as well as in those with predominantly LMN signs. H/M amplitude ratios did not differ between the various patient groups. CONCLUSION: These findings show that ALS patients, including those without clinical evidence of UMN involvement, have a marked disinhibition of anterior horn motor neurons. The simple tests described could support an UMN abnormality when clinical signs are lacking, and help to establish a diagnosis sooner and more accurately.

Adult↗

Anal sphincter EMG does not distinguish between multiple system atrophy and Parkinson's disease.

Clinical distinction of multiple system atrophy (MSA) from Parkinson's disease (PD) is often difficult. Several recent reports indicate that objective classification may be accomplished using electromyographic (EMG) testing of the anal or urethral sphincters, but some authors have found that these tests are not reliable for this purpose. We studied 13 patients with PD and 10 with probable MSA, as diagnosed by consensus of four movement disorders specialists, according to accepted clinical criteria. Anal sphincter EMG was performed blind to the clinical diagnosis. We found no significant differences in the mean duration of motor unit potentials (MUPs), mean MUP amplitude, or prevalence of polyphasic potentials, satellite potentials, very long duration MUPs, or spontaneous activity between the two groups. Thus, anal sphincter EMG does not differentiate between PD and MSA.

Aged↗

Neurotoxicity of isolated limb perfusion with tumor necrosis factor.

Hyperthermic isolated limb perfusion (HILP) with tumor necrosis factor alpha (TNFalpha) is effective for advanced melanoma and sarcoma of the limbs. Ten patients undergoing HILP with TNFalpha were evaluated by neurological examinations, nerve conduction studies (NCS), sympathetic skin responses (SSR) and conventional and quantitative electromyography (EMG), performed before, 7 days and 6 weeks following HILP. Seven patients showed minimal clinical signs of peripheral nerve damage following HILP; in two the injury was evident electrophysiologically: 7 days following HILP five patients had paresthesias and/or hypoesthesia, one had a mild foot drop and one had autonomic disturbances in the affected limb. SSR was low in two patients in the affected limb, sensory nerve action potentials were not elicited in one, with normal motor NCS and EMG. At 6 weeks, four patients continued to have mild paresthesias and one had dysautonomia of the perfused limb. Sensory responses and SSR did not change, motor abnormalities were not found. These findings show that HILP with TNFalpha induces a mild, mainly sensory neuropathy in perfused limbs, not disturbing functionality and improving over time.

Adult↗

N30 somatosensory evoked potentials in patients with unilateral Parkinson's disease.

The N30 component of the somatosensory evoked responses (SEP) was reported as absent or abnormally low in various basal ganglia disorders, including Parkinson's disease (PD), but its relationship to the more affected side in asymmetric disease has not been assessed systematically. In 14 patients with unilateral PD and 10 controls, SEP were performed by stimulating each upper limb and recording from the parietal and frontal contralateral cortex. N30 was symmetric in 4 patients and 4 controls; it was asymmetric in 2 controls and in 8 patients (in 4 the responses were of lower amplitude over the affected hemisphere); no response was elicited in 2 patients and 4 controls. In all patients SEP were recorded off medication; in 9 of them the test was repeated following administration of L-dopa and did not show any significant changes. In conclusion, the N30 was asymmetric in a similar proportion of PD patients and controls. No correlation was found between the affected side and N30 latencies or amplitudes; no change was seen following L-dopa administration as well. The motor deficits and SEP abnormalities in PD probably reflect pathologies in different anatomical structures or functional circuits.

Adult↗

Antineurofilament antibodies in postpolio syndrome.

We determined the levels of antineurofilament antibodies in 29 patients with postpolio syndrome (PPS), 26 stable postpolio (PP) patients, 22 patients with ALS, and 20 normal controls (NCs). Patients with PPS had higher antibody levels to cholinergic neurofilaments than did all other groups. PP patients and those with ALS had antibody levels similar to those of NCs. The antibody binding level showed no relation to the age of the patients, duration of disease, or motor score.

Adult↗

The sympathetic skin response in reflex sympathetic dystrophy.

The sympathetic skin response (SSR) was examined in 12 consecutive patients with reflex sympathetic dystrophy (RSD). It was normal in the involved limbs of five patients, of lower amplitude than on the contralateral side in three and absent only on the involved side in four. The response abnormality was more prominent in patients examined during the first year following the injury. These abnormalities could reflect either the peripheral injury underlying RSD or secondary central nervous system disturbances.

Adult↗

Tests of autonomic dysfunction in patients with multiple sclerosis.

Autonomic dysfunction is frequent in patients with multiple sclerosis (MS). The sympathetic skin response (SSR) and the R-R interval variation (RRIV) are simple electrophysiologic tests for the assessment of central and peripheral autonomic disturbances. Both tests were performed in 60 patients with clinically definite MS and 30 controls. The SSR was recorded simultaneously from both upper and both lower limbs. In all volunteers normal responses were recorded from the four limbs, but 39 patients (65%) showed abnormal responses in at least one limb. The reduction in amplitude of the response was correlated with patients' EDSS. In individual limbs, the SSR amplitude correlated with weakness, spasticity and cerebellar dysfunction, but was not sufficiently related to the deep sensory loss. The RRIV was abnormal in 48 MS patients (80%), as compared to the controls, but showed no significant relationship either to the EDSS or to the SSR. The sensitivity of SSR and RRIV is high and compatible with that of visual and somatosensory evoked potentials.

Adult↗

Hypersensitivity vasculitis and systemic lupus erythematosus induced by anticonvulsants.

Vasculitis can be a systemic manifestation of hypersensitivity to many drugs, among them anticonvulsants. The clinical manifestations include rash and renal, hepatic, and pulmonary involvement. Diagnosis is based upon clinical findings and a characteristic biopsy showing granulocytic and sometimes eosinophilic infiltrates around small blood vessels, especially venules. A severe form of hypersensitivity vasculitis, with extensive visceral involvement and poor prognosis, has been encountered very rarely following phenytoin and in isolated cases following carbamazepine and trimethadione administration. Drug-induced systemic lupus erythematosus is much more frequent, with distinct clinical and laboratory abnormalities. The syndrome was described following treatment with most anticonvulsants in clinical use--phenytoin, carbamazepine, ethosuximide, trimethadione, primidone, and valproate, but not phenobarbital or benzodiazepines. The early recognition of these syndromes as being related to drugs is important, because they usually remit upon withdrawal of the offending agent.

Anticonvulsants↗

Sympathetic skin response: age effect.

We examined the sympathetic skin response in 100 normal subjects aged 20 to 91 years. The response was present in all younger subjects, but, in the elderly, was present in the lower limbs of only 50% and in the upper limbs of 73%. The amplitude of the response showed a marked decline with age in both the upper and lower limbs.

Adult↗

Electrophysiologic features in patients with chronic neurolathyrism.

Neurolathyrism is a toxic nutritional disorder induced by the ingestion of the chick-pea "lathyrus sativus" and characterized by a pure motor spastic paraparesis. Eight patients with long-standing disease underwent nerve conduction and electromyographic studies. Two of them (25%) showed electrophysiological signs of lower motor neuron disease in their lower limbs. Subclinical affection of the anterior horn cells occurs probably more frequently than expected in chronic neurolathyrism.

Aged↗

Carpal tunnel syndrome: a complication of idiopathic torsion dystonia.

Carpal tunnel syndrome (CTS) is usually an idiopathic disorder. Certain occupations that require frequent flexion movements of the hand at the wrist are recognized as precipitating the development of CTS. Dystonia can cause similar excessive movements at the wrists. We report the clinical and electromyographic findings of two patients with idiopathic torsion dystonia (ITD), with frequent flexion postures of the wrists, in both of whom typical CTS developed in the more involved hand. It is concluded that nerve entrapment such as CTS has to be considered a possible complication in patients with ITD and other motor control disorders.

Adult↗

EEG recordings following intrathecal iohexol administration.

Electroencephalogram (EEG) recordings were performed before and after intrathecal administration of iohexal (Omnipaque) in 30 patients who underwent cervical myelography or cisternography. Tracings were recorded before, several hours after, and 24 h after iohexol administration. No abnormalities were recorded. It can be concluded that iohexol is a safe contrast medium, having less encephalopathic effects than more commonly used substances such as metrizamide.

Adult↗