Short report: Paroxysmal choreoathetosis as a presenting symptom in idiopathic hypoparathyroidism

Soffer, Dov; Licht, Abraham; Yaar, Israel; Abramsky, Oded

doi:10.1136/jnnp.40.7.692

Cited by 40 publications

(15 citation statements)

References 16 publications

(19 reference statements)

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“…The [ 18 F]-FDG PET data also suggest that hypocalcaemia leads to PKC by determining neural hypofunction; this is by contrast with what was previously hypothesised by some authors,18who considered PKC the consequence of a neural hyperexcitability due to an increased membrane permeability. Of note is that the reduction of glucose metabolism in the striatum has also been reported in other movement disorders, such as different types of dystonias 19.…”

Section: Discussioncontrasting

confidence: 56%

Regression of ventral striatum hypometabolism after calcium/calcitriol therapy in paroxysmal kinesigenic choreoathetosis due to idiopathic primary hypoparathyroidism

Volontè

2001

Journal of Neurology, Neurosurgery &Amp; Psychiatry

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Section: Discussioncontrasting

confidence: 56%

Regression of ventral striatum hypometabolism after calcium/calcitriol therapy in paroxysmal kinesigenic choreoathetosis due to idiopathic primary hypoparathyroidism

Volontè

2001

Journal of Neurology, Neurosurgery &Amp; Psychiatry

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“…The differential diagnosis is wide and includes metabolic, infectious, toxin-induced and degenerative causes 53. Among the metabolic disorders, idiopathic or surgical hypoparathyroidism is probably the most common cause of symmetric basal ganglia calcification, and dystonia as presenting feature may occur 54. Infections (including congenital forms) by toxplasmosis, rubella, cytmomegaly, herpes and HIV may result in basal ganglia damage with calcifications and secondary dystonia 55,56.…”

Section: Resultsmentioning

confidence: 99%

Secondary Dystonia-Clinical Clues and Syndromic Associations

Schneider¹,

Bhatia

2009

JMD

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Background:Dystonia is a hyperkinetic movement disorder defined by involuntary sustained muscle spasms and unusual postures. Etiologically, dystonic syndromes can be broadly divided into primary and secondary forms, dystonia-plus syndromes and heredodegenerative forms. In particular, diagnosis of secondary dystonic syndromes can be challenging in view of the variety of causes.Purpose:The purpose of this article is to highlight some clinical clues and syndromic associations as well as investigational findings which may be helpful in the approach to a patient with suspected secondary dystonia.Methods:We outline characteristic clinical and neuroimaging findings which may be directive in the diagnostic process of dystonia patients and facilitate making the correct diagnosis, thus allowing initiating the best treatment.Results:Secondary causes of dystonia include, among others, strategic brain lesions of various origins, metabolic disease, neurodegenerative conditions, and previous exposure to drugs or toxins. Presence of clinical signs including prominent oromandibular involvement, eye movement disorders, retinitis pigmentosa, deafness, peripheral neuropathy, parkinsonism or progressive dementia should alert the clinician to consider a secondary cause. Strategic lesions within the basal ganglia, but also within the brainstem, cerebellum or cortical areas may underlie dystonia and should thus be excluded.Conclusions:When thorough clinical examination reveals features atypical of primary dystonia, syndromic associations may help the clinician to narrow down the list of differential diagnosis. Directive investigations like neuroimaging may confirm the clinical suspicion.

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“…As with PKC, the most commonly reported association is with multiple sclerosis (MS) [3,9,39]. There have been numerous other reported causes including encephalitis [36], head trauma [41], endocrine disorders including hypoparathyroidism [42], thyrotoxicosis [14], basal ganglia calcification [43] and a case with AIDS [44]. Cases with vascular lesions involving the basal ganglia, particularly the thalamus, have been associated with paroxysmal dystonia [40].…”

Section: Symptomatic Pdcmentioning

confidence: 99%

The paroxysmal dyskinesias

Bhatia

1999

Journal of Neurology

111

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The clinical, pathophysiological and genetic features of some of the paroxysmal movement disorders are reviewed. Paroxysmal kinesigenic choreoathetosis/dyskinesias (PKC/PKD) is a condition in which brief and frequent dyskinetic attacks are provoked by sudden movement. PKC is more common in men and can be idiopathic (commonly familial) or due to a variety of causes. The pathophysiology of PKC is uncertain but it could be an ion-channel disorder. Antiepileptic drugs particularly carbamazepine are very helpful in a large proportion of cases. Paroxysmal exercise induced dystonia (PED) is a rare disorder manifesting as episodes of dystonia mostly affecting the feet induced by continuous exercise like walking or running. Although the initial cases were familial, there is a higher proportion of sporadic cases. The pathophysiology of PED is unknown and antiepileptic drugs are generally unhelpful. In paroxysmal dystonic choreoathetosis/non-kinesigenic dyskinesias (PDC/PNKD) the attacks are of long duration and induced by variety of factors including coffee, tea, alcohol and fatigue but not by sudden movement. PDC can be idiopathic (familial or sporadic) or symptomatic due to a variety of causes. The gene for familial PDC has been linked in 2 families to chromosome 2 q close to a cluster of ion channel genes again suggesting that this disorder may also be a channelopathy. Other paroxysmal disorders include paroxysmal nocturnal dyskinesia, a form of frontal lobe epilepsy in some cases which may be familial with autosomal dominant inheritance (ADNFLE). The gene for ADNFLE in one family has been found to be a mutation in the neuronal acetylcholine receptor gene (CHRNA4) on chromosome 20q. Tonic spasms in multiple sclerosis and Sandiffers syndrome producing intermittent torticollis in infants and children are other paroxysmal movement disorders.

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Short report: Paroxysmal choreoathetosis as a presenting symptom in idiopathic hypoparathyroidism

Cited by 40 publications

References 16 publications

Regression of ventral striatum hypometabolism after calcium/calcitriol therapy in paroxysmal kinesigenic choreoathetosis due to idiopathic primary hypoparathyroidism

Regression of ventral striatum hypometabolism after calcium/calcitriol therapy in paroxysmal kinesigenic choreoathetosis due to idiopathic primary hypoparathyroidism

Secondary Dystonia-Clinical Clues and Syndromic Associations

The paroxysmal dyskinesias

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