Limb ataxia originating from peri-central sulcus demyelinating lesion in multiple sclerosis.
BACKGROUND
To report the development of limb ataxia/intention tremor (LA/IT) associated with inflammatory demyelinating cortical peri-central sulcus lesions.


METHODS
We describe a case series of five multiple sclerosis (MS) patients followed at the Jacobs Neurological Institute who developed LA/IT associated with contralateral cortical lesions without visible subtentorial MRI pathology. Demographics, MRI findings and tremor evolution in relation to specific therapies were recorded.


RESULTS
Five patients (M=1/F=4), age range 29-51 that developed LA/IT associated with a contralateral cortical MRI lesion were identified. LA/IT developed after an average of 3.1years (range 0-8years) from disease onset. The contralateral cortical MRI lesion became visible on average 23.2months before the development of limb ataxia/intention tremor. Central sulcus widening was noted suggesting local atrophy. Median nerve somatosensory evoked potentials revealed asymmetric reductions in N20 amplitudes. Abnormalities in latencies and amplitudes were also noted in the posterior tibial somatosensory evoked potentials, ipsilateral to the lesion. Symptomatic therapeutic interventions were only partially beneficial.


CONCLUSIONS
Limb ataxia/intention tremor can be associated with a demyelinating lesion involving cortical and adjacent subcortical white matter, in the absence of rubro-cerebellar lesions. Aggressive therapeutic intervention to control the cortical inflammatory process is recommended.
Baird MS Center, Jacobs Neurological Institute, SUNY Buffalo School of Medicine and Biomedical Sciences, United States. md.yuval@gmail.com
Yuval
Y
Karmon
Sarah Anne
SA
Morrow
Arie
A
Weinstock
David
D
Hojnacki
Bianca
B
Weinstock-Guttman
D000328:Adult; D001259:Ataxia / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D001284:Atrophy / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology* / Q000503:physiopathology; D018450:Disease Progression; D005073:Evoked Potentials, Somatosensory / Q000502:physiology; D005260:Female; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods / Q000706:statistics & numerical data; D008297:Male; D008475:Median Nerve / Q000502:physiology; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D009413:Nerve Fibers, Myelinated / Q000473:pathology* / Q000502:physiology; D059906:Neuroimaging / Q000379:methods* / Q000706:statistics & numerical data; D013979:Tibial Nerve / Q000502:physiology; D014202:Tremor / Q000150:complications / Q000473:pathology / Q000503:physiopathology