Sural nerve and epidermal vascular abnormalities in a case of POEMS syndrome.
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Collagen Type IV↗
Biomedical subjects
V Provitera
Publications and source records attributed to V Provitera.
Distal degeneration of sensory and autonomic cutaneous nerve fibres in systemic sclerosis.
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Adult↗
Generalised anhidrosis: different lesion sites demonstrated by microneurography and skin biopsy.
Generalised anhidrosis (GA) shows a uniform clinical picture whether the pathogenesis involves intrinsic abnormalities of sweat glands or postganglionic sympathetic cholinergic nerve dysfunction. We describe two patients who presented intolerance to heat and anhidrosis. In the first patient, symptoms started at 33 years of age, and were associated with absent tendon reflexes and a mydriatic right pupil unreactive to light. The other patient had been unable to sweat since birth. GA was diagnosed on the basis of clinical findings and thermoregulatory tests. Microneurography and morphological analysis of the skin and its innervation disclosed a different lesion site underlying GA in the two patients, and distinguished between a postganglionic autonomic nerve fibre lesion and sweat gland dysfunction.
Adult↗
Is Ross syndrome a dysautonomic disorder only? An electrophysiologic and histologic study.
OBJECTIVE: To define the involvement of peripheral nerve fibers in Ross syndrome. METHODS: Mechanical pain perception, tactile and thermal thresholds on hand, foot dorsum, thigh, median nerve orthodromic sensory conduction velocity (SCV) and motor conduction velocity (MCV), sural nerve antidromic SCV, peroneal nerve MCV, H-reflex, F-wave, median, tibial nerve somatosensory evoked potentials (SSEPs), perioral, hand CO(2) laser late (LEPs) and ultralate evoked potentials, sympathetic skin response (SSRs), cardiovascular, Minor sweat, silastic imprint, histamine, photopletysmographic and pupil pilocarpine tests, cutaneous innervation immunohistochemical techniques were studied in 3 patients with Ross syndrome. RESULTS: Quantitative sensory testing showed altered results in patients 1 and 2, and patient 3 had a slight impairment of mechanical pain perception. Nerve conduction, except for a median nerve distal reduction of sensory conduction in patient 1, F-wave and SSEP findings were normal; H-reflex was absent at rest in all patients. Hand LEPs were absent in patient 2, ultralate potentials were absent in patients 1 and 2. Skin biopsy showed a disease duration related reduction of unmyelinated and myelinated sensory fibers and a lack of unmyelinated autonomic fibers in all patients. CONCLUSIONS: Our data suggest that Ross syndrome is a degenerative disorder involving progressive sudomotor fibers, and then epidermal sensory unmyelinated and myelinated fibers.
Adult↗
Small fibers involvement in Friedreich's ataxia.
Although the involvement of large myelinated sensory fibers in Friedreich's ataxia (FA) is well documented, an impairment of unmyelinated fibers has not been described. We demonstrate an involvement of cutaneous unmyelinated sensory and autonomic nerve fibers in FA patients. We performed a morphological and functional study of cutaneous nerve fibers in 14 FA patients and in a population of control subjects. We used immunohistochemical techniques and confocal microscopy applied to punch skin biopsies from thigh, distal leg, and fingertip, and compared the density of epidermal nerve fibers (ENFs) with the results of mechanical pain sensation and thermal and tactile thresholds performed on hand dorsum, thigh, distal leg, and foot dorsum. We observed in our patients a statistically significant loss of ENFs, a reduced innervation of sweat glands, arrector pilorum muscles and arterioles, and an impairment of thermal and tactile thresholds and mechanical pain detection.
Adolescent↗
Absent innervation of skin and sweat glands in congenital insensitivity to pain with anhidrosis.
OBJECTIVES: A case of a 10-year-old girl with congenital insensitivity to pain with anhidrosis (CIPA) is reported. METHODS AND RESULTS: Parents referred several hyperpyretic episodes without sweating occurring since birth, and insensitivity to pain, noticed when the child was 2 years old. Her body had many bruises and scars, bone fractures and signs of self-mutilation. Neurological examination was normal except for insensitivity to pain. Her IQ was 52. Electrical and tactile sensory nerve conduction velocities were normal. The patient was unable to detect thermal stimuli. Histamine injection evoked a wheal but not a flare; pilocarpine by iontophoresis did not induce sweat. Microneurography showed neural activity from A-beta sensory fibers while nociceptive and skin sympathetic C fiber nerve activity was absent. No small myelinated fibers and very rare unmyelinated fibers were found in the sural nerve. Immunohistochemistry showed a lack of nerve fibers in the epidermis and only few hypotrophic and uninnervated sweat glands in the dermis. CONCLUSIONS: The lack of innervation of the skin (C and A-delta fibers) appears to be the morphological basis of insensitivity to pain and anhidrosis, and is consistent with the loss of unmyelinated and small myelinated fibers in the sural nerve biopsy.
Child↗
Response of thalamic pain syndrome to lamotrigine.
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Journal Article↗
Profile of cerebrospinal fluid and serum cytokines in patients with relapsing-remitting multiple sclerosis: a correlation with clinical activity.
Levels of tumor necrosis factor (TNF)-alpha, granulocyte macrophage-colony stimulating factor (GM-CSF), interleukin (IL)-10 and transforming growth factor (TGF)-beta in cerebrospinal fluid (CSF) and serum of 29 patients with multiple sclerosis (MS) of the relapsing-remitting type and of 20 controls with other non inflammatory neurological diseases were studied. Sixteen patients were in the active phase of disease and 13 in remission. In CSF, higher IL-10 and TGF-beta concentrations were found in patients with a stable phase of MS, while in the active phase there were elevated levels of TNF-alpha and GM-CSF. These results suggest that a different cytokine pattern could be probably involved in the pathogenesis of relapsing-remitting MS.
Adolescent↗
Possible role of transforming growth factor-beta in relapsing-remitting multiple sclerosis.
Cerebrospinal fluid (CSF) and serum levels of transforming growth factor (TGF)-beta and soluble intercellular adhesion molecule-1 (sICAM-1) were evaluated in ten patients with definite multiple sclerosis (MS) of the relapsing-remitting type. CSF TGF-beta levels of MS patients in remission were significantly (p < 0.01) higher than of MS patients in active phase, and there was a significant inverse correlation (p < 0.05) between TGF-beta and slCAM-1 levels in the CSF of patients in both remitting and relapsing type. This is consistent with a possible down-regulation of TGF-beta on ICAM-1 expression and suggests a possible synthesis in the central nervous system of TGF-beta.
Adult↗
Age-related risk factors for ischemic stroke in Italian men.
We evaluated the age-related stroke risk factors in 164 Italian male patients with a diagnosis of first-ever ischemic stroke. Based on the age, we divided the patients into two groups: 42 patients ranging in age from 40 to 55 years, and 122 patients ranging in age from 56 to 75 years. For each case, an age-matched control without a history or symptoms indicating vascular disease was randomly selected from hospital patients. Information were obtained on the various risk factors. Univariate analysis showed that for the younger patients high systolic blood pressure, diabetes mellitus, hypertriglyceridemia, smoking and family history of ischemic stroke were significantly related to stroke. In the older patients, high diastolic blood pressure and smoking had a strong association with stroke. Multivariate analysis showed that high systolic blood pressure, hypertriglyceridemia, smoking and family history of stroke remained significantly and independently associated with stroke in patients up to the age of 55 years. Among patients 56 years or older, only high systolic and diastolic blood pressure, and smoking were significant predictors of stroke. In conclusion, the sets of factors associated with the risk of stroke among young and old male patients appear to be different.
Adult↗
Mood disorders in epilepsy.
Depression is a common psychiatric problem associated with epilepsy. Interictal depressive symptoms are more frequent and severe in epileptic patients than in subjects with comparable chronic neurologic diseases or physical handicaps. Epileptic depression was characterized as major or dysthymic: bipolar depression is rarely described. Several Authors are of opinion that interictal depression is more frequent in epileptics with temporal lobe foci and, in particular, with temporal left hemisphere lesions. The pathogenetic significance of depression in epileptics is unclear. Some suggest the hypothesis that depression represents behavioral effects of neurochemical responses to brain injury for asymmetrical hemispheric distribution of neural substrate for mood. We think that depression in epileptic patients does not represent a psychological reaction to a particular cognitive or physical impairment, but it is in some way related to the type of epilepsy. In addition, some antiepileptic drugs may have psychotropic effects: the most positive findings were associated with carbamazepine.
Anticonvulsants↗
Cytokines in the pathogenesis of multiple sclerosis.
The authors evaluate the involvement of various cytokines (interleukin-1, interleukin-2, interleukin-4, interleukin-6, tumor necrosis factor alpha and gamma-interferon) in the pathogenesis of multiple sclerosis. The cytokines might participate in nervous tissue damage by promoting demyelination and oligodendrocyte injury or by enhancing local immune response. In addition, several authors reported increased levels of some cytokines in serum and cerebrospinal fluid of patients with multiple sclerosis. These findings suggest that cytokines can play a significant role in the immunopathogenesis of the disease.
Cytokines↗
Soluble interleukin-2 receptor and soluble CD8 molecules in cerebrospinal fluid and serum of patients with multiple sclerosis.
The purpose of this study was to analyse soluble interleukin-2 receptor (sIL-2R) and soluble CD8 (sCD8) molecules in the cerebrospinal fluid (CSF) and serum of 18 patients with definite multiple sclerosis (MS) and of 16 with noninflammatory neurologic diseases (NIND). All MS patients suffered from an exacerbation of the relapsing-remitting form of the disease within one month before examination. The mean serum levels of sIL-2R and sCD8 in the MS patients were not significantly different from those of NIND patients. Only one patient with MS had detectable sIL-2R in the CSF. CSF sCD8 was detectable in 10 of 18 MS patients and in 1 of 16 NIND patients. Our data indicate that the CSF and serum sIL-2R concentrations do not correlate with the disease activity. Conversely, increased levels of sCD8 only in the CSF of MS patients support the hypothesis of an intrathecal activation of CD8+ cells in MS. We think that CSF sCD8 can be a useful marker for the presence of activated T cells in the central nervous system.
Adult↗