Quantitative sensory testing: effect of site and pressure on vibration thresholds.

OBJECTIVE: To determine the effect of different sites and locally applied pressure on vibration thresholds. METHODS: Vibration thresholds were compared in 47 normal volunteers at 3 sites of the index finger (pulp, dorsum of the middle phalanx and nail) and at two sites of the great toe (dorsum of the proximal phalanx and nail). The effect of local pressure (30, 50 and 100 g/1.22 cm(2)) were compared in 41 subjects at the dorsum of the middle phalanx of the index finger and the proximal phalanx of the great toe. RESULTS: The hand was more sensitive than the foot for vibration. There were no significant differences in vibration thresholds at different sites of the index finger and different sites of the great toe. The pulp of the index finger yielded the least inter-individual variation. Testing under 30 and 50 g/1.22 cm(2) of pressure yielded equal vibration thresholds. Vibration threshold was higher when tested under 100 g/1.22 cm(2) at the index finger but not the great toe. This difference was small and clinically negligible. CONCLUSION: Testing of vibration thresholds in normal subjects can be adequately conducted at several sites of the index finger and the great toe. The test can be adequately done under low pressure of 30-50 g/1.22 cm(2).

Quantitative sensory testing: effect of site and skin temperature on thermal thresholds.

OBJECTIVE: To determine the effect of different sites and local skin temperature on thermal thresholds. METHODS: Cool and warm detection and cold and heat pain thresholds were compared in 46 normal volunteers at the thenar eminence (TE), dorsum of the hand (DH), volar surface of the wrist (VW) and dorsum of the foot (DF). RESULTS: The hand is more sensitive than the foot for cool and warm. TE is more sensitive for warm than DH and VW but the difference is clinically negligible. DH and VW are equally sensitive to warm. TE, DH, and VW are equally sensitive to cool. Inter-individual variance is smallest at TE. Warm and cool thresholds are independent of local skin temperature (range of 27-37 degrees C). TE is less sensitive for cold pain but otherwise the hand and the foot are equally sensitive to thermal pain. CONCLUSION: Testing of thermal thresholds in normal subjects can be adequately conducted at several sites at the hand, however, TE is preferred given the small inter-individual variability. TE may be preferred for evaluating hyperalgesia to cold given its higher threshold. Warming or cooling of the skin is unnecessary within the range normally encountered in routine clinical evaluation.

Neuropathy in erythropoietic protoporphyrias.

Peripheral neuropathy (PN) has rarely been described as a complication of erythropoietic protoporphyria (EPP). We describe three episodes of PN and the electrophysiologic findings in two patients with EPP. PN is seen in patients with EPP and hepatic failure and raised free erythrocyte protoporphyrin or serum protoporphyrin levels and is identical to that seen in acute intermittent porphyria. Recognition is important because of the good eventual prognosis.

MRI in juvenile ALS: a patient report.

We describe the MR images of a patient with juvenile ALS. MRI of the brain showed bilateral hyperintensities along the corticospinal tracts extending from the corona radiata to the brainstem on T2-weighted images. These findings should be differentiated from the slight hyperintensities seen in the posterior limbs of the internal capsules in normal subjects.

Clinical heterogeneity of familial spastic paraplegia linked to chromosome 2p21.

The clinical features of four families with autosomal dominant spastic paraparesis (FSP) are described, along with the results of linkage analysis to markers from the regions of chromosomes 2, 14, and 15 which are known to contain spastic paraplegia genes. All families had 'pure' spastic paraparesis (FSP), but the severity of symptoms varied widely among families, and other mild neurologic signs were observed in some subjects. Although no family individually yielded a lod score >3.0, all families yielded positive lod scores with chromosome 2 markers, and a maximal lod score of 5.7 was obtained for the families combined using marker D2S352. There was no evidence of linkage to chromosome 14 or 15 in any of the families.

Evaluation of the relative dose response test for vitamin A nutriture in cirrhotics.

The rise in serum vitamin A 5 h after a 450 microgram oral dose of the vitamin (retinyl palmitate) was used to assess vitamin A nutriture in patients with alcoholic cirrhosis. The test was carried out on 21 hospitalized male patients and 12 normal age and sex-matched control subjects. The relative dose response (RDR), expressed as percentage, was calculated as A5 - A0/A5 X 100 where A0 = the fasting serum retinol level and A5 = the serum retinol 5 h postdosing. Vitamin A-deficient patients (those with serum retinol levels less than 30 microgram/dl and an abnormal dark adaptation test or RDR greater than or equal to 14%) were treated with 4 wk of oral vitamin A (10,000 microgram/day), then repeat RDR and dark adaptation tests were carried out. Among eight cirrhotics with abnormal dark adaptation, the mean +/- SEM RDR was 21 +/- 9 versus 3 +/- 3% in patients with normal dark adaptation (p less than 0.01). RDR tests of patients with normal dark adaptation did not differ from those of 12 normal age and sex-matched control subjects (normal RDR response 0 to 14%). Among patients found to be vitamin A-deficient, treatment with vitamin A resulted in the mean +/- SEM RDR declining from 21 +/- 9 to 5 +/- 2%. However, this fall failed to reach statistical significance (p = 0.06). The RDR test appears to be useful as a predictor of vitamin A deficiency, even among patients with far advanced hepatic disease.