1/14. Emergency department presentations of patients with Parkinson's disease.Parkinson's disease (PD) is a chronic progressive neurological disorder characterized by tremor, muscle rigidity, slowness of movement (bradykinesia), and gait instability. In early disease, PD is well managed in an office setting, however, as the disease progresses, a variety of syndromes may result in emergency department visits. The scenarios most likely to require an emergent evaluation are severe motor "off" periods with immobility, involuntary movements (dyskinesia), psychosis, acute confusion, panic disorder, and pain. Other less frequent presentations are also discussed. This article uses illustrative cases to provide a framework to discuss emergency department diagnosis and management issues in caring for these patients.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
2/14. clozapine-associated extrapyramidal reaction.OBJECTIVE: To report a case of extrapyramidal reaction associated with a dosage increase of clozapine. CASE SUMMARY: A 44-year-old white man with a 20-year history of chronic paranoid schizophrenia was admitted to an inpatient psychiatric facility. His prior medications restarted on admission were clozapine 650 mg at bedtime, haloperidol 10 mg at bedtime, clonazepam 2 mg/d, and aspirin 325 mg/d. Two days after admission (hospital day 3), clozapine and clonazepam were discontinued, and he was prescribed haloperidol 5 mg every morning and 10 mg every evening. Stabilization occurred over the following 24 days, with progressively lower dosages of haloperidol and increasing dosages of clozapine. haloperidol was discontinued on day 24. On day 47, the patient was agitated and making bizarre statements; thus, the morning dose of clozapine was increased by 50 mg (total 450 mg/d). On day 48 at 2200, a dystonic reaction was diagnosed; he received intramuscular diphenhydramine 50 mg, which caused the reaction to subside. At the time of the adverse reaction, he was prescribed clozapine 450 mg/d, vitamin e 400 IU three times daily, aspirin 325 mg/d, and acetaminophen, milk of magnesia, and Maalox as needed. DISCUSSION: Although the risk of extrapyramidal symptoms (EPS) is significantly lower with clozapine than with conventional agents, elevated clozapine blood concentrations have been reported to cause EPS; other reports have cited severe dystonias and dyskinesias on abrupt clozapine withdrawal. Considering the medications prescribed at the time and the discontinuation of haloperidol 24 days before the event, clozapine was the most likely cause of the extrapyramidal reaction. CONCLUSIONS: Regardless of anticipated safety associated with novel antipsychotics such as clozapine, reports of dystonic reactions must be taken into account and patients monitored appropriately.- - - - - - - - - - ranking = 13807.015973439keywords = extrapyramidal (Clic here for more details about this article) |
3/14. Clinicopathological study of atypical motor neuron disease with vertical gaze palsy and ballism.The case of a 38-year-old patient with rapidly progressing motor neuron disease, complicated by major dysfunction of the extrapyramidal system and of vertical gaze is described. Neuropathological examination revealed a degenerative process that severely affected the lower motor neurons, as well as the neurons of the pars compacta of the substantia nigra, the nucleus of Darkschewitsch, the nucleus interstitialis of Cajal, the colliculi superiores, and the pallidum. The long tracts were unaffected at all levels of the brain stem and spinal cord. There was no convincing evidence for the presence of a multiple system atrophy or progressive supranuclear palsy; the results rather revealed a pattern of vulnerability characteristic of a variant of motor neuron disease.- - - - - - - - - - ranking = 1972.4308533484keywords = extrapyramidal (Clic here for more details about this article) |
4/14. fatal outcome in a case of pontocerebellar hypoplasia type 2.Pontocerebellar hypoplasia (PCH) is a very rare congenital (autosomal recessive) condition with fetal onset. Only a few cases have been published on the basis of both clinical data (symptoms/neuroradiological imaging) and autopsy results. This paper reports on such a case involving a 1.5-year-old male infant. The child suffered from severe psychomotor delay, extrapyramidal dyskinesia and epileptic seizures, but did not exhibit signs of spinal muscular atrophy as related to PCH type 1. magnetic resonance imaging (MRI) at the age of 6 months demonstrated olivo-pontine and bilateral cerebellar hypoplasia. The boy was unexpectedly found dead. autopsy disclosed a severe aspiration of gastric contents as the final cause of death. The neuropathological examination confirmed PCH type 2 (according to Barth [Brain Dev., 15 (1993) 411-422]) with marked microcephaly and olivopontocerebellar hypoplasia. Histologically, decreased density of olivo-pontine neurons, reduction of granular and Purkinje's cell layers of the cerebellum, slight astroglial proliferation and fragmented appearance of the dentate nuclei were observed. The immunohistochemical expression pattern was determined using antibodies against glial fibrillary acidic protein, synaptophysin and neurofilament protein. Summarizing, typical features of PCH type 2 were present and proved by clinical course, MRI and autopsy. Despite severe symptoms due to a natural disease this rare neurogenetic entity can become of forensic interest, when sudden unexpected death occurs.- - - - - - - - - - ranking = 1972.4308533484keywords = extrapyramidal (Clic here for more details about this article) |
5/14. Alternating paroxysmal hemiballism-hemichorea in bilateral internal carotid artery stenosis.A 72-year-old man presented with paroxysmal and transient involuntary movements, or "limb shaking". The attacks occurred alternately from one side of the body to the other and ceased spontaneously. Surface EMG study showed synchronous grouping discharges in multiple limb muscles, being compatible with hemiballism-hemichorea. cerebral angiography demonstrated marked stenosis of the bilateral internal carotid arteries. Bilateral carotid endarterectomy led to complete disappearance of the involuntary movements. Alternating paroxysmal hemiballismhemichorea might be a transient ischemic attack, and alternating striatal dysfunction induced by cerebral hemodynamic or microembolic ischemia probably plays a central role in the occurrence of such involuntary movements.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
6/14. Rabbit syndrome secondary to risperidone.After taking risperidone for 4 months, a 38-year-old man with a major depressive disorder experienced rabbit syndrome. This uncommon extrapyramidal side effect is characterized by rhythmic movements of the mouth and may be caused by typical antipsychotics and risperidone secondary to blockade of dopamine D2 receptors. The syndrome, whose exact mechanism is unknown, responded to anticholinergic treatment in this patient. A literature review indicated that most recent cases have occurred as reactions to the atypical antipsychotic risperidone. Changing treatment to an atypical antipsychotic other than risperidone, such as olanzapine 10 mg/day, may lead to suppression or elimination of the syndrome.- - - - - - - - - - ranking = 1972.4308533484keywords = extrapyramidal (Clic here for more details about this article) |
7/14. Neuromotor dysfunction in early psychosis.Neuromotor dysfunction, particularly extrapyramidal signs and symptoms (EPSS), plays an important role in the assessment and treatment of patients in the early stages of psychotic disorders such as schizophrenia. By blocking dopamine D2 receptors, antipsychotic medications can produce EPSS, including tardive dyskinesia. EPSS is also observed in a third or more of patients first presenting with a psychotic disorder, prior to initiation of antipsychotic pharmacotherapy. This suggests that abnormalities in neuromotor control may be an integral component of the brain mechanisms associated with psychosis. Atypical antipsychotic agents can alleviate psychosis without inducing EPSS. Preexisting EPSS may be corrected.- - - - - - - - - - ranking = 1972.4308533484keywords = extrapyramidal (Clic here for more details about this article) |
8/14. "On the other hand": a case of hereditary, congenital mirror movement.We describe a 20-year-old male Singaporean army recruit with hereditary, congenital mirror movements who presented with difficulties in military training because releasing the grip of one hand resulted in a similar release by the other hand. His father has mirror movements with a significant decrease in symptoms with time, a phenomenon that has not been previously described. Both father and son have no associated neurological abnormalities. Bilateral cortical representation for hand muscles and the presence of active ipsilateral corticospinal projections have been postulated as the mechanisms responsible for this condition.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
9/14. Functional MR imaging of hand motor cortex in a case of persistent mirror movement.Persistent mirror movements are unwanted movements restricted to muscles homologous to those moved intentionally on the opposite body half. It is rarely observed and the functional MRI findings in a case of persistent mirror movement are described.- - - - - - - - - - ranking = 1keywords = muscle (Clic here for more details about this article) |
10/14. Familial pediatric rapidly progressive extrapyramidal syndrome: is it Hallervorden-Spatz disease?The clinical features of two children of a family with rapidly progressive extrapyramidal-pyramidal-dementia complex have been described. Inheritance seems most likely to be autosomal recessive. magnetic resonance imaging results of brain were negative. Even so, the authors argued in favor of a diagnosis of Hallervorden-Spatz disease because the cases fulfilled the clinical criteria for diagnosis of this disease. Apart from the negative magnetic resonance findings, the other unusual feature was the early development of levodopa-induced dyskinesia.Few conditions need to be considered in the differential diagnosis of a childhood-onset rapidly progressive extrapyramidal syndrome. Such conditions include Wilson's disease, Hallervorden-Spatz disease (HSD), juvenile form of Huntington's disease, juvenile neuronal ceroid lipofuscinosis, early-onset machado-joseph disease neuroacanthocytosis, storage disorders, and variant form of dopa-response dystonias (DRD). Rarer conditions are Leigh's disease, Lafora body disease, and dentato-rubro-pallido-luysian atrophy. HSD is a rare disorder characterized by progressive extrapyramidal dysfunction and dementia. Onset is most commonly in late childhood or early adolescence. The disease can be familial or sporadic. When familial, it is inherited recessively and has been linked to chromosome 20. Recently, a mutation in the pantothenate kinase (PANK2) gene on band 20pl3 has been described in patients with typical HSD. HSD produces typical magnetic resonance imaging (MRI) changes in brain, aiding in antemortem diagnosis. The typical finding is of bilaterally symmetrical hyperintense signal changes in the external segment of globus pallidus, with surrounding hypointensity on T(2)-weighted image. These imaging features are fairly diagnostic and have been termed the "eye-of-the tiger sign". The hyperintensity represents pathologic changes, including gliosis, demyelination, neuronal loss, and axonal swelling, and the surrounding hypointensity is caused by loss of signal secondary to iron deposition. Described herein are the clinical aspects of a family with autosomal recessive inheritance with rapidly progressive extrapyramidal-pyramidal-dementia complex but with negative brain MRI results. The diagnosis should be considered a variant form of HSD.- - - - - - - - - - ranking = 15779.446826788keywords = extrapyramidal (Clic here for more details about this article) |
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