Chorea and polycythaemia

Mas, Jean Louis; Guéguen, B.; Bouché, P; Derouesné, Christian; Varet, Bruno; Castaigne, P

doi:10.1007/bf00313895

Cited by 20 publications

(8 citation statements)

References 16 publications

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“…Several mechanisms have been suggested: sluggish cerebral blood flow particularly through the basal ganglia; reduced levels and turnover of cerebral catecholamines and serotonin in older people, resulting in receptor upregulation; oestrogen deficit in postmenopausal women, resulting in dopamine receptor hypersensitivity; and excess dopamine due to platelet congestion in the cerebral vessels [19]. We had one case (patient 19) in whom chorea was associated with polycythaemia and was probably its ultimate cause; the eventual disappearance of the chorea suggests that its proximal cause may have been transitory ischemia.…”

Section: Discussionmentioning

confidence: 99%

Cause and course in a series of patients with sporadic chorea

Piccolo

Defanti

Soliveri³

et al. 2003

Journal of Neurology

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Although a convincing concordance between choreic signs and neuroradiological findings was possible in 4 patients only, it was possible to assign an aetiology in most cases with vascular related causes the most frequent and metabolic factors often participating. Huntington's disease is not unusual as a cause of sporadic choreas. HIV infection is an emerging cause of chorea and AIDS-related disease should be considered in young patients presenting without a family history of movement disorders. We emphasize the importance of follow-up to identify persistent chorea for which genetic testing is mandatory.

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Section: Discussionmentioning

confidence: 99%

Cause and course in a series of patients with sporadic chorea

Piccolo

Defanti

Soliveri³

et al. 2003

Journal of Neurology

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“…20Polycythaemia occurs more often in men (3:2), but polycythaemic chorea is seen predominantly in women (5:2), usually after the age of 50, with an overall prevalence of 1% to 2.5% 1920 As many as two thirds of the patients present with chorea, and on examination are found to have facial erythrosis and splenomegaly consistent with polycythaemia. The chorea may begin insidiously or acutely, is sometimes episodic, and may initially be asymmetric, although it typically becomes generalised, with predominantly facial, lingual, and brachial involvement.…”

Section: Hypoxic-ischaemic Causesmentioning

confidence: 99%

Dystonia and chorea in acquired systemic disorders

Janavs

Aminoff

1998

Journal of Neurology, Neurosurgery & Psychiatry

121

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Dystonia and chorea are uncommon abnormal movements which can be seen in a wide array of disorders. One quarter of dystonias and essentially all choreas are symptomatic or secondary, the underlying cause being an identifiable neurodegenerative disorder, hereditary metabolic defect, or acquired systemic medical disorder. Dystonia and chorea associated with neurodegenerative or heritable metabolic disorders have been reviewed frequently.1 Here we review the underlying pathogenesis of chorea and dystonia in acquired general medical disorders (table 1), and discuss diagnostic and therapeutic approaches. The most common aetiologies are hypoxia-ischaemia and medications. [2][3][4] Infections and autoimmune and metabolic disorders are less frequent causes. Not uncommonly, a given systemic disorder may induce more than one type of dyskinesia by more than one mechanism.The areas of the brain associated with particular movement disorders have been determined by cerebral imaging and necropsies of patients, and by animal lesioning studies. 5-7Based on such data, chorea seems to result from hypofunction of the indirect pathway from the putamen to the internal globus pallidus, and dystonia correlates more strongly with hyperfunction of the direct relative to the indirect pathway between the putamen and internal globus pallidus, both resulting in inappropriate disinhibition of thalamic projections to the premotor and motor cortex (figure). Chorea has been most consistently associated with lesions in the caudate nucleus or putamen, resulting in disinhibition of the external globus pallidus. Lesions of the subthalamointernal pallidal pathway also result in chorea.Associated neurotransmitter abnormalities include deficient striatal GABA-ergic function and striatal cholinergic interneuron activity, and dopaminergic hyperactivity in the nigrostriatal pathway. Dystonia has been correlated with lesions of the contralateral putamen, external globus pallidus, posterior and posterior lateral thalamus, red nucleus, or subthalamic nucleus, or a combination of these structures. The result is decreased activity in the pathways from the medial pallidus to the ventral anterior and ventrolateral thalamus, and from the substantia nigra reticulata to the brainstem, culminating in cortical disinhibition. Altered sensory input from the periphery may also produce cortical motor overactivity and dystonia in some cases. 8 To date, the changes found in striatal neurotransmitter concentrations in dystonia include an increase in noradrenaline and a decrease in dopamine concentrations. Hypoxic-ischaemic causes MECHANISMSChorea and dystonia may result from hypoxiaischaemia due to global cerebral hypoperfusion or cellular hypoxia, such as in toxic mitochondrial dysfunction. Two hypotheses have been put forward to explain hypoxic-ischaemic injury to the basal ganglia: selective hypoperfusion of certain vascular territories and an intrinsic metabolic susceptibility of the striatum to hypoxia-ischaemia due to its high oxidative metabolism. 7 The internal segm...

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“…2 Among the latter, chorea has been reported most frequently 34 The pathogenesis of polycythaemic chorea still is a subject of speculation 5…”

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confidence: 99%

Chorea disclosing deterioration of polycythaemia vera

Nazabal

Marey-López

Perez

et al. 2000

Postgraduate Medical Journal

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Kikuchi's disease is usually a self limiting illness characterised by pyrexia, neutropenia, and cervical lymphadenopathy particularly in young women of Asian descent. This often leads to an initial misdiagnosis of lymphoma. A case of a young Asian woman who presented with pyrexia of unknown origin is described. (Postgrad Med J 2000;76:655-656) Keywords: pyrexia of unknown origin; cervical lymphadenopathy Case reportA 19 year old south Asian women was admitted with a six week history of fever, malaise, anorexia, and cervical lymphadenopathy. She had been well until onset of her current illness. She was on no medication and had no contacts with tuberculosis.She was born in India but had moved to the UK at the age of 9 years. Foreign travel included a brief return to her native country 10 years previously and a trip to the United States two years before presentation.Clinical examination revealed a young women who appeared well; her temperature was 39ºC. The main findings were several tender mobile posterior cervical lymph nodes of which one was large. There were no lymph nodes elsewhere and she had no splenomegaly. The rest of the clinical examination was unremarkable.Investigations revealed a low white cell count at 2.7 × 10 9 /l with a neutrophil count of 1.3 × 10 9 /l, a raised plasma viscosity of 1.99 cp (normal range 1.5-1.72). A biochemical profile, chest radiography, and abdominal ultrasound gave normal results.The clinical impression was that this was likely to be tuberculous as she was Asian, or it could possibly be a lymphoma. Her clinical course was of a swinging intermittent pyrexia of up to 40.5ºC. Blood and urine cultures, thin and thick films for malaria parasites, cytomegalovirus, Epstein-Barr virus, and HIV titres were all negative.Tests for antinuclear factor, double stranded DNA, and antineutrophil cytoplasmic antibodies were negative. Thyroid microsomal antibody titres were positive at 1/400 and T cell analysis showed severe T lymphopenia of CD4, CD8, and NK populations with T cell activation.Later in her illness she had computed tomography of her chest, abdomen, and pelvis which was unremarkable, although minimal splenic enlargement was noted. Bone marrow aspiration and a trephine biopsy did not show any evidence of lymphoma or non-caseating granulomas.The large posterior cervical lymph node was excised. Microscopic examination using an antibody against CD 68 showed numerous darkly staining histiocytes, many of them containing cell fragments. There was necrosis and apoptosis and immature lymphoid cells in keeping with a diagnosis of Kikuchi's disease. Kikuchi's disease was originally described in the Far East in 1972 and over the last decade has become increasingly recognised in the Western world.Antibiotic treatment was withdrawn. Her temperature settled after three weeks, but she continued to remain neutropenic for six weeks from the beginning of the illness. When reviewed in outpatients four weeks after discharge from hospital she was well and all her lymph nodes had disappeared. ...

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Chorea and polycythaemia

Abstract: Two patients with chorea and polycythaemia vera are described. The literature on this rare association is reviewed and its pathophysiology discussed.

Cited by 20 publications

References 16 publications

Cause and course in a series of patients with sporadic chorea

Cause and course in a series of patients with sporadic chorea

Dystonia and chorea in acquired systemic disorders

Chorea disclosing deterioration of polycythaemia vera

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