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Clonidine GH stimulation test to differentiate MSA from idiopathic late onset cerebellar ataxia: a prospective,
C Bonnard1,2,3, T Wirth1,2,3, O Gebus1,2,3
1Service de Neurologie, Département de Neurologie, Hôpitaux Universitaires de Strasbourg, Hôpital de Hautepierre, 1, Avenue Molière, 67098, Strasbourg Cedex, France.
Introduction:
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.
Methods:
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.
Results:
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).
Conclusion:
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.
Insights
The clonidine growth hormone (CGH) test can help differentiate early-stage multiple system atrophy of cerebellar type (MSA-C) from idiopathic late-onset cerebellar ataxias (ILOCA). This diagnostic tool shows promise in clinical practice for accurate early disease identification.
Area of Science:
- Neurology
- Clinical Diagnostics
Background:
- Diagnosing early-stage multiple system atrophy of cerebellar type (MSA-C) is challenging due to overlapping symptoms with idiopathic late-onset cerebellar ataxias (ILOCA).
- Current diagnostic approaches often require disease progression to meet consensus criteria.
Purpose of the Study:
- To evaluate the effectiveness of the clonidine growth hormone (CGH) test in distinguishing early-stage MSA-C from ILOCA.
- To assess the CGH test's sensitivity and specificity in this differential diagnosis.
Main Methods:
- A prospective, controlled study comparing patients with possible or probable MSA-C to those with ILOCA.
- Inclusion of clinical and paraclinical examinations, including serial CGH tests during follow-up.
- Analysis of CGH test results for pathological findings, sensitivity, specificity, and predictive values.
Main Results:
- The study included 86 patients (42 MSA-C, 44 ILOCA) with a mean follow-up of 33 months.
- Pathological CGH tests increased in MSA-C patients during follow-up (52.4%) compared to ILOCA patients (20.5%).
- The CGH test demonstrated a sensitivity of 69.1% and specificity of 68.2% for MSA-C, with a negative predictive value of 75% for ruling out probable MSA-C.
Conclusions:
- The CGH test is a valuable tool for differentiating early-stage MSA-C from ILOCA.
- This diagnostic method can aid clinicians in making earlier and more accurate diagnoses.
- The study supports the integration of the CGH test into clinical practice for suspected early MSA-C.
