1/21. Cardiac sympathetic denervation in Ross syndrome demonstrated by MIBG-SPECT.We investigated cardiac sympathetic innervation by metaiodobenzylguanidine (MIBG) imaging in a patient with tonic pupils, loss of tendon reflexes, and segmental anhidrosis (Ross syndrome). Despite normal cardiovascular reflex tests, we observed a reduced global myocardial MIBG uptake as well as a regional uptake defect over the posterolateral cardiac territory indicating left ventricular peripheral sympathetic denervation. MIBG imaging seems to be a useful noninvasive diagnostic method for detection of early--possibly subclinical--cardiac autonomic impairment in Ross syndrome and provides further evidence of injury to postganglionic autonomic neurons as the underlying pathological mechanism of the disease.- - - - - - - - - - ranking = 1keywords = reflex (Clic here for more details about this article) |
2/21. Ross syndrome plus: beyond horner, Holmes-Adie, and harlequin.BACKGROUND: Ross syndrome is an uncommon disorder characterized by the triad of segmental anhidrosis, hyporeflexia, and tonic pupils. methods: The authors describe the clinical findings of five patients with Ross syndrome and detail the results of their pharmacologic and autonomic testing. RESULTS: In four patients, the classic findings of Ross syndrome were accompanied by Horner's syndrome. Other symptoms of dysautonomia were also common. CONCLUSIONS: These findings suggest that Ross syndrome is a dysautonomic condition of varying expression resulting from a generalized injury to ganglion cells or their projections.- - - - - - - - - - ranking = 1366.9619407728keywords = hyporeflexia, reflex (Clic here for more details about this article) |
3/21. Ross' syndrome (tonic pupil plus).Two cases of tonic pupil, hyporeflexia, and segmental hypohidrosis (Ross' syndrome) are reported. The relationship of this syndrome to other autonomic dysfunction is discussed. Those symptoms (emotional instability, loss of sweating, orthostatic hypotensive symptoms, and signs of bilaterality of the tonic pupil) which should alert the clinician to more extensive disease states are noted. It is suggested that these conditions may represent a continuum or spectrum of disorders with a widespread degree of severity and progression.- - - - - - - - - - ranking = 1366.9619407728keywords = hyporeflexia, reflex (Clic here for more details about this article) |
4/21. Indolent corneal ulcers in a patient with congenital insensitivity to pain with anhidrosis: a case report and literature review.PURPOSE: To report a case of bilateral corneal neurotrophic ulcer in patient with congenital insensitivity to pain with anhidrosis (CIPA) and review the literature. CASE REPORT: A 6 year-old boy presented with bilateral central corneal sterile ulcer, decreased corneal sensitivity, moderately altered corneal reflex and normal tearing response. history taken, systemic evaluation and medical chart review were undertaken. DISCUSSION: Fifty-two cases of CIPA have been reported worldwide. Fourteen cases had corneal involvement. The clinical picture of our patient is characteristic of CIPA. CONCLUSIONS: Congenital insensitivity to pain with anhidrosis may present as neurotrophic corneal ulcer. We report herewith, this vision threatening corneal congenital abnormality. early diagnosis and prompt treatment are mandatory to prevent corneal complications such as scarring and perforation.- - - - - - - - - - ranking = 0.5keywords = reflex (Clic here for more details about this article) |
5/21. Selective loss of cholinergic sudomotor fibers causes anhidrosis in Ross syndrome.Ross syndrome consists of segmental hyperhidrosis with widespread anhidrosis, adie syndrome, and areflexia. The cause of this disorder is unknown. Selective degeneration of cholinergic fibers or of neural crest-derived structures has been suggested. We present clinical and skin biopsy data of 4 patients, providing evidence of reduced cholinergic sweat gland innervation in hypohidrotic skin by morphometric analysis. These findings indicate a selective degenerative process of the cholinergic sudomotor neurons.- - - - - - - - - - ranking = 0.5keywords = reflex (Clic here for more details about this article) |
6/21. The circadian rhythm of body core temperature (CRT) is normal in patient with congenital generalized anhidrosis.The temperature of the human body is not constant during the day, and is related to a double modulation of both homeostatic and circadian processes. The circadian rhythm of body core temperature (CRT) is known to depend on the central mechanism involved in thermoregulatory variations. The role of sweating in the nocturnal fall of body core temperature (BcT) is not clear. We evaluated the CRT in a 45-year-old female with a lack of sweating since birth because of congenital generalized anhidrosis. She referred episodes of heat intolerance when ambient temperature was around 35 degrees C. skin biopsies of both forearms and left axilla revealed atrophy and morphologic changes of eccrine glands. Neurological examination, nerve conduction studies, sympathetic skin response and cardiovascular reflex tests were normal. The study of CRT was performed by monitoring rectal temperature continuously in controlled conditions (ambient temperature 24 /- 1 degrees C and humidity 40-50% in a light-dark schedule). The rhythmometric analysis showed normal 24-hour fluctuations. This case represents an "experiment of nature"demonstrating that the physiological nocturnal fall of BcT is independent of sweating.- - - - - - - - - - ranking = 0.5keywords = reflex (Clic here for more details about this article) |
7/21. 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.- - - - - - - - - - ranking = 1keywords = reflex (Clic here for more details about this article) |
8/21. Segmental facial anhidrosis and tonic pupils with preserved deep tendon reflexes: a novel autonomic neuropathy.A 31-year-old woman had exertional right-sided hemifacial flushing and sweating. Examination demonstrated slightly dilated pupils with absent constriction to light and a tonic near response and redilatation, features consistent with adie syndrome. Neurological examination was otherwise normal, including preservation of deep tendon reflexes. magnetic resonance imaging of brain and spine were normal. The combination of unilateral loss of sudomotor and vasomotor activity without loss of ocular sympathetic innervation fulfills the diagnosis of Harlequin syndrome. The combination of Harlequin and Adie syndromes has been called Ross syndrome, but the preservation of deep tendon reflexes precludes a diagnosis of Ross syndrome in our patient. This previously undescribed variant adds further complexity to the spectrum of autonomic neuropathies.- - - - - - - - - - ranking = 3keywords = reflex (Clic here for more details about this article) |
9/21. 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.- - - - - - - - - - ranking = 0.5keywords = reflex (Clic here for more details about this article) |
10/21. tonic pupil, areflexia, and segmental anhidrosis: two additional cases of Ross syndrome and review of the literature.Two patients are described with the triad of tonic pupil, hyporeflexia and segmental anhidrosis (Ross syndrome). Only 18 cases of this syndrome have been reported in the literature so far. While tonic pupil and reduced sweating can be attributed to the affection of postganglionic cholinergic parasympathetic and sympathetic fibres projecting to the iris and sweat glands, respectively, the pathogenesis of diminished or lost tendon jerks remains obscure. To identify the characteristic clinical features, the previous cases of Ross syndrome are reviewed. Recent evidence of subclinical disturbances of sweating in most patients with Adie's syndrome, i.e. tonic pupil and areflexia, casts doubt on the nosological concept of Ross syndrome as a distinct clinical entity.- - - - - - - - - - ranking = 1369.4619407728keywords = hyporeflexia, reflex (Clic here for more details about this article) |
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