Iraqi Journal of Medical Sciences

Vol. 13 Issue 1 January - March / 2015
Published on website | Date : 2016-03-22 23:23:08


Shaymaa J. Mohammed, Mazin M. Hammady


Background:Spinal muscular atrophies are a group of degenerative diseases primarily affecting the anterior horn cells of the spinal cord and motor cells of cranial nerve nuclei. Even if the clinical picture is mainly dominated by the diffuse muscular atrophy, in some cases, patients may show associated, atypical clinical features. There have been a few reported cases of spinal muscular atrophy with central nervous system involvement; in particular, the association with progressive myoclonic epilepsy has been rarely described.
Objectives:To clarify the impact of this degenerative disease on central nervous system and specifically on cerebral activity by using electroencephalographic exam.
Methods: Thirty two patients with spinal muscular atrophy and 20 control subjects were included in this study. Their ages were between two months and one year. Brain CT and MRI, electroencephalography was done for all of them and cerebral activity was precisely assessed with emphasis on normal brain waves frequency and distribution.
Results:The frequency of brain waves recorded from patients with spinal muscular atrophy who show abnormal electroencephalography were 1.49±0.4 and it is significantly lower than that of control subjects (4.11±1.1). The mean frequency of brain waves recorded from patients with spinal muscular atrophy with normal electroencephalography were 4.13±1.2 which is higher than the mean frequency of brain waves recorded form patients with abnormal electroencephalography (1.49± 0.4).
Conclusion:Central nervous system could be affected in patients with spinal muscular atrophy specifically cerebral activity, which might show diffuse slowing in brain waves as revealed in this study.
Keywords :Spinal muscular atrophy, cerebral activity, electroencephalography