1/26. Neuroaxonal dystrophy with dystonia and pallidal involvement.Infantile neuroaxonal dystrophy (INAD) is an autosomal recessive disease of infantile onset, characterised by progressive clinical course, multi-systemic involvement and widespread presence of dystrophic axons in both the central and peripheral nervous system. Clinical, neurophysiological and neuroradiological criteria of the disease are established, but the occurrence of atypical cases is known. Since the availability of molecular markers is still lacking, diagnostic evidence in vivo is provided by the presence of specific axonal lesions distally in the peripheral nerve fibres. In two children who had a protracted course of the disease with dystonic postures of the upper limbs and showed dystrophic axons following sural nerve biopsy, bilateral pallidal hypointensity was observed after T2-weighted MRI scans. These findings are consistent with iron deposition, and are usually observed in Hallervorden-Spatz syndrome (HSS), a condition which is also characterised by dystrophic axons diffusely present in the central nervous system, but without peripheral nervous system involvement. These observations raise the issue of different phenotypes of INAD, and are consistent with the existence of intermediate forms between INAD and HSS. Altered mechanisms of iron storage and transport to and from the cellular compartments may play a role in the pathogenesis of the disease.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/26. Focal limb dystonia in a patient with a cerebellar mass.BACKGROUND: Focal dystonia of acute onset is indicative of a structural lesion in the nervous system. Cerebellar lesions have rarely been associated with dystonia. CASE DESCRIPTION: A 42-year-old woman was admitted to the neurology ward because of fever, confusion, and gait unsteadiness. She was diagnosed as having tuberculous meningitis, and, after a few days of antituberculous treatment, she developed prominent dystonia of the left upper limb. Cranial nuclear magnetic resonance imaging showed an isolated lesion compatible with a tuberculoma in the left cerebellar hemisphere. Both the limb dystonia and the tuberculoma resolved with maintained antituberculous treatment. CONCLUSIONS: In the patient described, the presence of upper-limb dystonia ipsilateral to a focal cerebellar lesion and the resolution of the dystonia and the mass lesion following treatment suggest that the cerebellum or its connections to the thalamus and/or basal ganglia could be involved in the pathophysiology of the dystonia.- - - - - - - - - - ranking = 0.21777129568387keywords = nervous system (Clic here for more details about this article) |
3/26. movement disorders possibly induced by traditional chinese herbs.The authors describe the neurological presentation and CT/MRI findings in 4 patients exposed to overdoses of decoctions of two different Chinese herbs. Case 1, a 15-year-old boy, ingested herba serissae along with the safe-dosage salvia miltiorrhiza for treating a left renal stone. sophora subprostrata root (SSR) was primarily used for treating three other diseases: viral B hepatitis in case 2, a 9-year-old boy; infection of the throat and a low fever in case 3, a 11-year-old girl, and a minor facial infection in case 4, a 12-year-old boy. All patients showed complex neurological manifestations primarily including convulsions, mental changes and dystonia syndromes. Their CT and/or MRI revealed abnormal density lesions in the striatum and globus pallidus bilaterally. They excluded the possibility of Wilson's disease in each of the 4 patients and suggested that overdosage of SSR and herba serissae could cause intoxications of the central nervous system, particularly damage to the basal ganglia. Chemically, coumarin (case 1) and matrine and oxymatrine (cases 2-4) in the two medicinal herbs are suggested to be possibly responsible for the morbidity.- - - - - - - - - - ranking = 0.56445740863227keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/26. Probable topical permethrin-induced neck dystonia.A 28-year-old Caucasian man who applied topical 5% permethrin cream once for the treatment of scabies infestation experienced a severe dystonic reaction on the left side of his neck. Assessment of causality using the Naranjo algorithm revealed a probable relationship between this adverse drug event and application of topical permethrin. Dystonic or musculoskeletal adverse reactions, including muscle spasms, have been reported with pyrethroid insecticides only after inappropriate occupational dermal exposure. These reported reactions occurred 4-48 hours after exposure and lasted from a few days to a few weeks. The mechanism behind these adverse effects may be related to permethrin's ability to delay sodium channel closure within nerve cells. This may result in a lowered threshold of nerve fibers to activation of further action potentials, leading to repetitive firing and hyperexcitation of the nervous system. Clinicians should be aware of this rare but potential adverse effect of permethrin.- - - - - - - - - - ranking = 0.21777129568387keywords = nervous system (Clic here for more details about this article) |
5/26. Tonic dystonia: an uncommon complication of reflex sympathetic dystrophy syndrome. A review of five cases.Tonic dystonia is an underrecognized complication of reflex sympathetic dystrophy syndrome (RSDS) characterized by an increase in muscle tone at the site of injury. Case-reports.- We describe five cases of tonic dystonia complicating RSDS of the lower extremity. There were four women and one man, with a mean age of 52 years. In addition to the typical features of RSDS, the patients had fixed equinovarus of the foot with hyperextension or hyperflexion of the great toe. In two patients, examination after spinal anesthesia showed that the deformity was reducible. Spontaneous resolution of the dystonia occurred in one patient. Another patient failed to experience meaningful improvement after a motor block followed by botulinic toxin injections. In two patients, the same treatment was followed by a slight improvement. Treatment options are still being evaluated in the last patient. Discussion.- Tonic dystonia is an underrecognized complication of RSDS that often develops after a minor injury yet causes prolonged pain and disability. Spread of the dystonia to other sites is not infrequent. The underlying mechanisms remain unclear but may involve dysfunction of the central or peripheral nervous system or psychogenic factors. Suggested treatments include motor block, intrathecal baclofen, sympathetic block, and sympathectomy. However, none of these treatments has been proved effective. Conclusion.- The five cases described here provide useful information on RSDS-associated tonic dystonia, a condition that runs a protracted course and remains difficult to manage.- - - - - - - - - - ranking = 0.21777129568387keywords = nervous system (Clic here for more details about this article) |
6/26. acupuncture-induced cerebral blood flow responses in dystonia.OBJECTIVE: The effect of acupuncture (ACP) on regional cerebral blood flow (rCBF) is unclear. Single-photon emission computed tomography studies on three patients with dystonia were performed before and after ACP treatment to test the contention that ACP affects rCBF. methods: Pre-ACP and post-ACP CBF study were performed on the same day; 99mTc ethyl cysteinate dimer was injected for each study. rCBF images were analyzed using a three-dimensional stereotaxic ROI template (3DSRT) to objectively measure rCBF. We evaluated rCBF bilaterally in five segments related to the pathophysiology of dystonia (1, superior frontal; 2, middle and inferior frontal; 3, primary sensorimotor; 4, lenticular nucleus; and 5, thalamus). More than 10% left-right asymmetry in rCBF over three continuous slices was defined as significant laterality. Post-ACP rCBF and laterality were evaluated with the pre-ACP rCBF study acting as a control in each subject. RESULTS: The clinical effect of ACP was remarkable in all patients and rCBF increased in most segments. Pre-ACP rCBF exhibited significant laterality in eight segments of the three patients. Laterality reversed in seven of these segments and resolved in the remaining segment after ACP. Pre-ACP rCBF laterality was not preserved in any segment after ACP. The remaining five segments exhibited laterality only after ACP. In total, after ACP, 13 of 15 segments demonstrated a change in CBF that was greater unilaterally. CONCLUSIONS: ACP results in an increase in CBF that is greater unilaterally. We think that unilateral change in CBF may be correlated with the action of ACP on the central nervous system in patients with dystonia.- - - - - - - - - - ranking = 0.56445740863227keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/26. A case of chronic GM1 gangliosidosis presenting as dystonia: clinical and biochemical studies.Clinical and biochemical studies are reported on a 32-year-old man with GM1 gangliosidosis who presented with a slowly progressive dystonia that began when he was aged 7 years and eventually became almost totally incapacitating at the age of 35. There was only mild intellectual deterioration, but myoclonus, seizures and macular cherry-red spots were never observed. Proton-density and T2-weighted MRI scans showed symmetrical hyperintense lesions of both putamina. No increase of GM1 ganglioside was found in plasma or cerebrospinal fluid, and the metabolism of GM1 ganglioside in cultured skin fibroblasts from the patient was also almost normal, although the residual activity of GM1 ganglioside beta-galactosidase activity was only 10% of normal. These findings suggest that impaired GM1 ganglioside metabolism is not present systemically as it is in the infantile and juvenile types of the disorder, but is mainly confined to the central nervous system in chronic GM1 gangliosidosis.- - - - - - - - - - ranking = 0.56445740863227keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/26. Generalized dystonia with bilateral striatal computed-tomographic lucencies in a patient with human immunodeficiency virus infection.We report the case of a 36-year-old, drug-addicted woman with laboratory evidence of human immunodeficiency virus infection in the central nervous system. The patient presented with generalized dystonia involving both axial and segmental muscles, mainly in the legs, and with minimal additional neurological abnormalities. A computed-tomographic scan revealed bilateral and symmetrical lucencies in the putaminal region. The possibility of an acquired immunodeficiency syndrome-related manifestation is discussed.- - - - - - - - - - ranking = 0.56445740863227keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/26. chorea and dystonia: a remote effect of carcinoma.A 45-year-old woman with an acquired multifocal neurologic syndrome, including chorea, dystonia, cerebellar dysfunction, multiple cranial neuropathies, and pure sensory neuropathy, was found at autopsy to have oat cell carcinoma. Neuropathologic examination revealed several features typically associated with remote effects of malignancy on the nervous system. We believe that this is the first described case of chorea as a remote effect of malignancy.- - - - - - - - - - ranking = 0.21777129568387keywords = nervous system (Clic here for more details about this article) |
10/26. Peripheral dystonia.We studied four patients with distal, action-induced involuntary postures of the hand that could be considered focal dystonia. All four patients had electrophysiologic findings consistent with peripheral nervous system lesions (pronator teres syndrome, radial nerve palsy, lower brachial plexus lesion, or median nerve lesion). With varying success, patients were treated with carbamazepine, trihexyphenidyl, methocarbamol, and wrist splinting. We wish to emphasize that peripheral entrapment and brachial plexopathy should be added to the causes of secondary dystonias.- - - - - - - - - - ranking = 0.21777129568387keywords = nervous system (Clic here for more details about this article) |
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