Limb ataxia originating from peri-central sulcus demyelinating lesion in multiple sclerosis

• Published in: Journal of the neurological sciences Vol. 320; no. 1; pp. 136 - 140
• Main Authors: Karmon, Yuval, Morrow, Sarah Anne, Weinstock, Arie, Hojnacki, David, Weinstock-Guttman, Bianca
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier B.V 15.09.2012; Elsevier
• Subjects: Adult; Age; Ataxia; Ataxia - complications; Ataxia - pathology; Ataxia - physiopathology; Atrophy; Atrophy - pathology; Biological and medical sciences; Cerebral Cortex - pathology; Cerebral Cortex - physiopathology; Demography; Demyelination; Disease Progression; Evoked Potentials, Somatosensory - physiology; Evolution; Female; Functional Laterality - physiology; Humans; Inflammation; Limbs; Magnetic resonance imaging; Magnetic Resonance Imaging - methods; Magnetic Resonance Imaging - statistics & numerical data; Male; median nerve; Median Nerve - physiology; Medical sciences; Middle Aged; Motivation; MRI; Multiple Sclerosis; Multiple Sclerosis - complications; Multiple Sclerosis - pathology; Multiple Sclerosis - physiopathology; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Nerve Fibers, Myelinated - pathology; Nerve Fibers, Myelinated - physiology; Neuroimaging - methods; Neuroimaging - statistics & numerical data; Neurology; Somatosensory evoked potentials; Substantia alba; Therapeutic applications; Tibial Nerve - physiology; Tremor; Tremor - complications; Tremor - pathology; Tremor - physiopathology; Multiple Sclerosis; Ataxia; Tremor; MRI; Nervous system diseases; Multiple sclerosis; Central nervous system disease; Neurological disorder; Nuclear magnetic resonance imaging; Inflammatory disease; Involuntary movement; Cerebral disorder
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/j.jns.2012.05.039

Abstract 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 3.1 years (range 0–8 years) from disease onset. The contralateral cortical MRI lesion became visible on average 23.2 months 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. Conclusion 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.