1/12. 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 = ganglion (Clic here for more details about this article) |
2/12. Painful sweating.OBJECTIVE: The authors report a case of spontaneous and gustatory facial pain and sweating. methods: The patient had frequent episodes of pain, sweating, and flushing bilaterally in the hairless skin of the ophthalmic and maxillary distributions of the trigeminal nerve. Gustatory stimuli (e.g., orange juice, pickled onions) reliably evoked episodes, but episodes also frequently came on spontaneously. The problem had begun during adolescence, about the time of topical treatment and then electrocauteries for facial warts. The patient reported benefit from tricyclic antidepressants, guanethidine, and trospium chloride (an anti-cholinergic quaternary amine used in europe for urinary urgency). There was no pain or excessive sweating in other body areas, nor pain with exercise. RESULTS: Administration of edrophonium IV evoked pain and sweating, and ganglion blockade by IV trimethaphan eliminated pain and sweating and markedly attenuated responses to edrophonium. Trospium chloride also prevented edrophonium-induced pain and sweating. Bicycle exercise produced the same increment in forehead humidity as in a spontaneous episode but did not evoke pain. tyramine infusion did not bring on pain or sweating, whereas iontophoretic acetylcholine administration to one cheek evoked pain and sweating bilaterally. Topical glycopyrrolate cream eliminated spontaneous, gustatory, and edrophonium-induced episodes. CONCLUSIONS: The findings indicate that facial pain and sweating can result from occupation of muscarinic cholinergic receptors after acetylcholine release from local nerves. The authors propose that after destruction of cutaneous nerves, aberrant regenerant sprouting innervates sweat glands, producing gustatory sweating as in auriculotemporal syndrome (Frey syndrome), and innervates nociceptors, producing pain.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
3/12. 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 = 1keywords = ganglion (Clic here for more details about this article) |
4/12. Loss of reflex tearing: an expected consequence of juvenile nasopharyngeal angiofibroma.OBJECTIVE: To report the loss of reflex tearing after surgical treatment of juvenile nasopharyngeal angiofibroma (JNA). STUDY DESIGN AND SETTING: A retrospective case series of 8 patients with surgical extirpation of JNA from 1995 to 2003 in a major teaching hospital setting was studied for symptomatic xerophthalmia. RESULTS: One patient was lost to follow-up. Four of the remaining 7 patients reported a dry ipsilateral eye after surgical treatment of JNA. CONCLUSION: The location of the pterygopalatine ganglion and its associated fibers in the pterygopalatine fossa is directly adjacent to the location of origin of JNA. Lacrimal innervation passes through the pterygopalatine ganglion. Given the extensive nature of advanced JNA and mandate for complete surgical excision, lacrimal dysfunction should be considered an expected consequence of surgery. SIGNIFICANCE: The loss of reflex tearing has not been reported as a consequence of JNA or its surgical treatment. EBM Rating: C.- - - - - - - - - - ranking = 2keywords = ganglion (Clic here for more details about this article) |
5/12. miller fisher syndrome: etiological significance of serial blink reflexes and MRI study.We studied a patient with miller fisher syndrome using a serial MRI and electrophysiological procedures. There were peripheral nerve pathology and CNS dysfunction. In particular, MRI showed a lesion at the level of the 3rd nerve nucleus.- - - - - - - - - - ranking = 0.12691997118222keywords = nucleus (Clic here for more details about this article) |
6/12. Acute dysautonomia associated with Hodgkin's disease.A patient is described with acute dysautonomia associated with Hodgkin's disease. Testing of cardiovascular reflex control showed that this patient had a rare manifestation of autonomic cardiovascular neuropathy, namely intact parasympathetic heart rate control in combination with a sympathetic postganglionic lesion affecting the control of the vascular tree.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
7/12. Tonic pupils with acute ophthalmoplegic polyneuritis.Two patients with the symmetrical ophthalmoplegic variant of acute idiopathic polyneuritis (Fisher's syndrome) developed bilateral dilated pupils with cholinergic supersensitivity and light-near dissociation (tonic pupils), indicating involvement of the postganglionic parasympathetic neuron within the orbit. Supranuclear eye movement dysfunction also was present, indicated by relative levator sparing in both patients and by preservation of Bell's phenomenon in 1 patient despite paralysis of voluntary upward gaze. The lesions that produce the eye signs of ophthalmoplegic polyneuritis are not necessarily restricted to the cranial nerve trunks. The present cases support a relationship between the ophthalmoplegic variant of the guillain-barre syndrome and acute postinfectious encephalomyelitis (brainstem encephalitis) on the one hand, and idiopathic autonomic neuropathies such as the Holmes-adie syndrome and pandysautonomia on the other.- - - - - - - - - - ranking = 1keywords = ganglion (Clic here for more details about this article) |
8/12. Hyperekplexia exacerbated by occlusion of posterior thalamic arteries.A 65-year-old man with the onset of hyperekplexia at 37 years of age experienced resolution of the illness at the age of 45 years. Twenty years later after a posterior thalamoperforate artery occlusion that produced a "rubral tremor," severe hyperekplexia redeveloped. The patient's symptoms were controlled with clonazepam, except for brief periods. Interruption of the rubrothalamic pathways or neuronal aggregates at the level of the red nucleus seemed to disinhibit the startle reflex.- - - - - - - - - - ranking = 0.12691997118222keywords = nucleus (Clic here for more details about this article) |
9/12. The manometric evaluation of the rectosphincteric reflex in total colonic aganglionosis.The manometric assessment of rectosphincteric function in 33 patients with histologically proven aganglionosis is reviewed. The age of the patient at first assessment and the presence of total colonic aganglionosis influenced the results obtained. overall, a 67% accuracy rate was achieved at first assessment. This rate fell to 43% when the test was carried out during the first 7 days of life. Six cases had total colonic aganglionosis. In only a single patient of this group was the first test result positive for aganglionosis. attention is drawn to this finding.- - - - - - - - - - ranking = 8keywords = ganglion (Clic here for more details about this article) |
10/12. Utilization behavior after right thalamic infarction.We report a patient who showed exaggerated responses to external cues (utilization behavior), motor impersistence, and a right-hand-predominant instinctive grasp reaction after right thalamic infarction. High-resolution computed tomography with stereotaxic lesion localization revealed almost complete destruction of the ventroanterior nucleus and intralaminar nuclei of the right thalamus; the dorsomedial nucleus was only partially involved. Single photon emission computed tomography revealed hypoperfusion in the right thalamus and over the entire right cerebral cortex with some prominence in the frontal area. From these observations, we believe that the utilization behavior in our case was caused by the disturbance in maintaining cortical tone of the right hemisphere as well as by the dysfunction of the right frontal lobe, both secondary to the damage to the right ventroanterior nucleus and intralaminar nuclei.- - - - - - - - - - ranking = 0.38075991354665keywords = nucleus (Clic here for more details about this article) |
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