Cerebrospinal fluid homovanillic acid levels in rapid-onset dystonia- parkinsonism

Allison Brashear, Ian J. Butler, Keith Hyland, Martin R. Farlow, William B. Dobyns

Research output: Contribution to journalArticlepeer-review

37 Scopus citations


Rapid-onset dystonia-parkinsonism (RDP) is characterized by sudden onset over hours to days of dystonia, dysphagia, dysarthria, and parkinsonism. RDP has been reported by our group in two apparently unrelated families. We now report analysis of cerebrospinal fluid metabolites of dopamine, norepinephrine, and serotonin for mild and severely affected individuals, known asymptomatic gene carriers, and at-risk individuals from both families with RDP. Levels of the dopamine metabolite homovanillic acid (HVA) were decreased in severely affected patients and in some asymptomatic gene carriers. HVA levels increased with treatment in some affected individuals, but this increase did not predict clinical response to carbidopa/levodopa. We suggest that a low HVA level is a biological marker with modest association to the diagnosis of RDP.

Original languageEnglish (US)
Pages (from-to)521-526
Number of pages6
JournalAnnals of Neurology
Issue number4
StatePublished - Apr 1 1998
Externally publishedYes

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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