Despite the consensus criteria for multiple system atrophy (MSA), the diagnosis of MSA of cerebellar type (MSA-C) may be difficult in the early stage of the disease. There are several differential diagnoses including idiopathic late-onset cerebellar ataxias (ILOCA) and it is often necessary to wait for clinical worsening so that the criteria can be met. Our aim was to assess the efficacy of clonidine growth hormone test (CGH test) to distinguish MSA-C from ILOCA in the early stage of the disease.
Within our cohort of late-onset sporadic, progressive cerebellar ataxia, the group of patients meeting the criteria for possible or probable MSA was compared to the ILOCA group. Clinical and paraclinical examination including CGH test were repeated during the prospective follow-up.
Eighty-six patients were recruited, including 42 patients in the MSA group and 44 ILOCA patients with a mean follow-up of 33 months. At the inclusion visit, CHG test was pathological for 31% MSA of patients and 18.2% of ILOCA patients (p = 0.35). During the follow-up, 52.4% of MSA-C had a pathological CGH test, while only 20.5% of ILOCA (p < 0.01). CGH test had a sensitivity of 69.1% and a specificity of 68.2%, (p < 0.001) for MSA-C patients; CGH test allows in three quarters of cases, if negative, to rule out a probable MSA-C (negative predictive value of 75%, p = 0.0014).
This prospective, controlled study showed that CGH test could be helpful in clinical practice to differentiate MSA-C from ILOCA in the early stage of the disease.
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Conflicts of interest
Mathieu Anheim declares honoraria and/or grants from AbbVie, Teva, Merz, Orkyn, Aguettant, Actelion Pharmaceuticals, Johnson and Johnson. Christine Tranchant declares honoraria from Merz and Allergan. Thomas Wirth received research grants from the Revue Neurologique, the Fondation Planiol and the APTES association and travel funding from LVL medical.
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Bonnard, C., Wirth, T., Gebus, O. et al. Clonidine GH stimulation test to differentiate MSA from idiopathic late onset cerebellar ataxia: a prospective, controlled study. J Neurol (2020). https://doi.org/10.1007/s00415-020-09737-z
- Multiple system atrophy
- Cerebellar ataxia
- Clonidine growth hormone test
- Idiopathic late-onset cerebellar ataxia