et al. 1 reported postural tremor in one patient with polycythaemia vera which was tentatively attributed to manganese deposits in the basal ganglia and improved after therapy with a chelating agent. Herein we report a patient with polycythaemia vera who developed persistent parkinsonism and in whom MRI showed features consistent with manganism.In 1998 a 47-year-old man employee began to complain of headache and arterial hypertension that led to the diagnosis of polycythaemia vera, which were treated with repeated blood-lettings. The next year he began to complain of dysesthesia to the feet, nocturnal leg cramps, and unsteady gait. His wife reported that he was slightly confused. The neurological examination showed masked face, monotonous voice, rigidity of the neck and of the right upper arm, slightly ataxic gait with forward bent of the trunk and diffusely brisk tendon reflexes. The blood examination revealed an increase of the hematocrit (50.2%), and transferrin (504 mg/dL; NV 290-390 mg/dL) and a decrease of the sideremia (33 lg/dL; NV 50-150 lg/dL). The serum magnesium, copper and ammonium were within normal range. Serum or urine manganese was not assessed. Hepatic biopsy was negative for increased hepatic copper content. Cranial CT was negative. T1 weighted MR imaging revealed a symmetric hyperintensity of the basal ganglia, cerebral peduncles, red nucleus, quadrigeminal bodies, superior cerebellar peduncles, and dentate nuclei. He started levodopa therapy (100 mg/die) which was unsuccessful and interrupted few months later. Seven years later the clinical condition was substantially stable and the score of the Unified Parkinson's Disease Rating Scale subscale 3 was 16. A control MRI showed progression of the T1 symmetric hyperintensity in the cerebral peduncels and evidence of the same signal change also in occipital white matter (Fig. 1). There was only a mild diffuse cerebellar atrophy. SPECT examination, obtained by injection of 111-185 MBq of [ 123 I]FP-CIT, showed only a mild decrease of dopamine transporter (DAT) density in the right putamen. Manganese level was finally assessed in blood and urine and it was within normal limits.The diagnosis of manganism in our patient was based on the clinical features, exclusion of Parkinson's disease and Wilson's disease, and on presence of the typical MRI appear-ance of symmetric hyperintensity in T1-weighted images of the basal ganglia and other brain structures caused by the paramagnetic properties of manganese. 1 Although a direct demonstration of increased manganese in the serum of our patient was not available, the normal manganese levels observed at follow-up do not exclude the diagnosis since it is well known that, serum or urine levels correlate with actual, but not previous, levels of exposure. 2 Manganese intoxication is usually caused by professional exposure as it typically occurs in miners, welders, and workers in steel industry, 3 but it can also be observed in acute or chronic hepatic diseases and anemia. 4,5 In particular, increased level of se...
Recent studies suggest that impulse control disorders (ICD) in Parkinson disease are not uncommon, and antiparkinsonian therapy, mainly the use of dopaminergic agonists, plays a causal role in the development of these symptoms. Pramipexole has been mainly related to the occurrence of ICDs, although these disorders may occur when any dopaminergic agonist-based therapy is administered.In this paper, we describe 4 patients with Parkinson disease and 1 with multisystem atrophy who presented a history (several months or years) of pathological gambling, hypersexuality, punding, and pathological use of the Internet secondary to piribedil. This previously undescribed association suggests that the development of these disorders might be also related to piribedil administration. It is also the first report of a patient with multisystem atrophy developing such adverse effects.
An 81-year-old female patient with a 12-year history of lingual pain associated to tongue and jaw involuntary movements secondary to long-term neuroleptic intake was observed. She received several pharmacological therapies without major improvement or side effects. Treatment with botulinum toxin type A markedly benefited both pain and lingual dyskinesia.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.