The differential diagnosis of chorea

@article{Wild2007TheDD,
  title={The differential diagnosis of chorea},
  author={Edward J. Wild and Sarah J. Tabrizi},
  journal={Practical Neurology},
  year={2007},
  volume={7},
  pages={360 - 373}
}
Chorea is a hyperkinetic movement disorder characterised by excessive spontaneous movements that are irregularly timed, randomly distributed and abrupt. In this article, the authors discuss the causes of chorea, particularly Huntington’s disease and the genetic syndromes that may resemble it, including HDL1-3, inherited prion disease, spinocerebellar ataxias 1, 3 and 17, neuroacanthocytosis, dentatorubro-pallidoluysian atrophy (DRPLA), brain iron accumulation disorders, Wilson’s disease, benign… 

Figures and Tables from this paper

Hereditary Chorea Associated With and Aggravated by Systemic Lupus Erythematosus
TLDR
It is recommended to be vigilant and have a low threshold for diagnosing co-existing autoimmune conditions like SLE in patients with hereditary choreiform disorder, as the first case of hereditary chorea associated with and aggravated by SLE is reported.
Update on the Non-Huntington's Disease Choreas with Comments on the Current Nomenclature
  • R. Walker
  • Medicine, Biology
    Tremor and other hyperkinetic movements
  • 2012
TLDR
The nomenclature of these disorders which has become somewhat unwieldy, but may ultimately be refined by association with the causative gene is discussed, which is essential for genetic counseling and, hopefully, ultimately genetic therapies.
Genetic diagnosis of hyperkinetic movement disorders.
  • R. Roos
  • Medicine
    Expert opinion on medical diagnostics
  • 2012
TLDR
This review of the literature presents the most frequently occurring genetically-determined forms of hyperkinesias, mainly chorea and dystonia and tries to give some practical guidelines.
Benign hereditary chorea: an update.
Chorea and related movement disorders of paraneoplastic origin: the PNS EuroNetwork experience
TLDR
In most patients, the movement disorder was classical choreoathetosis with symmetric involvement of the trunk, neck, and limbs, and CV2/CRMP5 was the most frequently associated antibody, followed by Hu.
Idiopathic progressive chorea: misnomer or still reality? A case with neuropathological disconfirmation
TLDR
The classic term of ‘‘idiopathicchorea’’ has been critically put into question and the patient further deteriorated present-ing diffuse spasticity and dystonia.
Polycythemia and chorea.
Missing Huntington's Disease for Tardive Dyskinesia: A Preventable Error
  • Hrishikesh Kumar, M. Jog
  • Medicine, Psychology
    Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques
  • 2011
TLDR
On the basis of available literature and experience of movement disorders clinics with such patients, useful clinical criteria are put forward to help differentiate between abnormal movements of TD and HD.
The neuropsychiatry of neuroacanthocytosis syndromes
...
1
2
3
4
5
...

References

SHOWING 1-10 OF 57 REFERENCES
Clinical features and natural history of neuroferritinopathy caused by the FTL1 460InsA mutation.
TLDR
In conclusion, isolated parkinsonism is unusual in neuroferritinopathy, and unlike Huntington's disease, cognitive changes are absent or subtle in the early stages, and gradient echo brain MRI will identify all symptomatic cases.
Cause and course in a series of patients with sporadic chorea
TLDR
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.
Clinical and genetic heterogeneity in benign hereditary chorea
TLDR
BHC is a clinically and genetically heterogeneous disorder, with one well-defined clinical syndrome mapping to chromosome 14q, and in the unlinked families, symptoms and signs were more heterogeneous as to age at onset and the occurrence of myoclonic jerks or dystonia.
Benign hereditary chorea—Entity or syndrome?
TLDR
Analysis of published reports of families with BHC concluded that BHC is not a diagnosis, but a syndrome that requires further investigation, and whether there is a distinct entity “BHC” with a single gene abnormality remains to be proven.
Huntington's disease
Adult‐onset chorea and mitochondrial cytopathy
  • M. Caer, K. Viala, Y. Agid
  • Medicine, Biology
    Movement disorders : official journal of the Movement Disorder Society
  • 2005
We report on 2 adult patients presenting with choreic movements as the main clinical feature of mitochondrial cytopathy. One patient exhibited a sensory neuronopathy and ophthalmoplegia. The other
Anti–basal ganglia antibodies in acute and persistent Sydenham’s chorea
TLDR
Western immunoblotting and immunofluorescence are the best methods for detecting anti–basal ganglia antibodies, and reactivity to basal ganglia antigens of 40, 45, and 60 kDa were commonly seen in both acute and persistent cases of SC.
A systematic review of the treatment studies in Huntington’s disease since 1990
TLDR
A systematic review of pharmacological therapy in HD using the available papers that were published between 1990 and 2006 found that a surprising amount of current drug therapy of HD in clinical practice is based on studies published before 1990.
Autosomal dominant cerebellar ataxias: clinical features, genetics, and pathogenesis
Movement disorders and AIDS: a review.
...
1
2
3
4
5
...