HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Figures Only Full Text Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Related articles in Neurology Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Dichgans, M. Articles by Auer, D. P. Search for Related Content PubMed PubMed Citation Articles by Dichgans, M. Articles by Auer, D. P. Related Collections MRS Gait disorders/ataxia All Cerebrovascular disease/Stroke Infarction Migraine

¹H-MRS alterations in the cerebellum of patients with familial hemiplegic migraine type 1

From the Department of Neurology (Drs. Dichgans, Herzog, and Freilinger), Klinikum Grosshadern, Ludwig Maximilians University, and NMR Group (Drs. Wilke and Auer), Max Planck Institute for Psychiatry, Munich, Germany.

Address correspondence and reprint requests to Dr. M. Dichgans, Marchioninistrasse 15, 81377 Muenchen, Germany; e-mail: mdichgans{at}nefo.med.uni-muenchen.de

Background: About 20% of patients with familial hemiplegic migraine (FHM) develop progressive cerebellar signs. Genetic studies have established an association with mutations in the CACNA1A gene. However, the mechanisms underlying cerebellar involvement are largely unknown.

Objective: To use proton MR spectroscopy (¹H-MRS) to investigate metabolic alterations in the cerebellum as well as cortical regions known to be involved in the propagation of migraine aura.

Methods: Fifteen CACNA1A mutation carriers from three FHM families and 17 healthy control subjects were studied. Eleven patients had clinical signs of cerebellar involvement. LCModel fits were used to estimate absolute concentrations of N-acetyl aspartate (NAA), myo-inositol (mI), glutamate (Glu), choline-containing compounds, total creatine, and lactate in the superior cerebellar vermis (SCV), parietal cortex, and occipital cortex. To control for atrophy effects, automated image segmentation was performed using SPM99. The brain parenchyma fraction (BPF) was determined for all three regions.

Results: Compared with controls, the brain parenchyma fraction (BPF), NAA, and Glu were significantly reduced and mI was significantly elevated in the SCV of patients with FHM. In contrast, no metabolite alterations were found in supratentorial regions. BPF and NAA in the SCV significantly correlated with cerebellar scores, in particular, gait ataxia.

Conclusions: The findings suggest that there is a regionally distinct neuronal impairment in the superior cerebellar vermis that exceeds macroscopic tissue loss. Correlations with clinical scores emphasize the functional relevance of localized atrophy (brain parenchyma fraction) and N-acetyl aspartate levels. These measures may be useful to monitor disease progression. The observed reduction in glutamate may in part reflect impaired glutamatergic neurotransmission.

Related articles in Neurology:

February 22 Highlights

Neurology 2005 64: 586-587. [Full Text]  

Familial hemiplegic migraine: More than just a headache

Michael Benatar and Corey M. Ford
Neurology 2005 64: 592-593. [Full Text]  

This article has been cited by other articles:

				U. G. Schulz, A. M. Blamire, R. G. Corkill, P. Davies, P. Styles, and P. M. Rothwell Association between cortical metabolite levels and clinical manifestations of migrainous aura: an MR-spectroscopy study Brain, December 1, 2007; 130(12): 3102 - 3110. [Abstract] [Full Text] [PDF]

				M. Benatar and C. M. Ford Familial hemiplegic migraine: More than just a headache Neurology, February 22, 2005; 64(4): 592 - 593. [Full Text] [PDF]