Abstract
Recognition of multiple sclerosis (MS) attacks relies mostly on clinical assessment. However, their definition based on McDonald criteria refers mostly to timing and when dealing with clinical features is rather ambiguous: “…of the kind seen in multiple sclerosis.” This is heightened in clinically isolated syndromes of the brainstem/cerebellum (CISB), where clinical manifestations can be manifold. This study aimed to describe the clinical features of patients with CISB to improve clinical recognition of patients with brainstem manifestations at the onset of their MS. To this end, we conducted a retrospective analysis of case notes of consecutively recruited patients with CISB assessed within 3 months of symptoms onset. Seventy-five patients were included. Most common brainstem-specific symptoms were: diplopia (68%), facial sensory symptoms (32%) and gait disturbance (31%). Adjusting for follow-up times, total number of symptoms and presence of other brainstem-specific symptoms, only the presence of facial sensory symptoms was predictive of (a lower risk of) conversion to clinically definite (CD) MS (Odds ratio: 0.086; p = 0.007). Neither the total number of brainstem-specific, non brainstem-specific nor the sum of both predicted conversion to CDMS. Results indicate that diplopia, facial sensory symptoms and gait disturbance occur in more than 30% of patients with CISB. Facial sensory symptoms are less associated with conversion to CDMS.
Similar content being viewed by others
References
Comi G, Filippi M, Barkhof F, Durelli L, Edan G, Fernandez O, Hartung HP, Seeldrayers P, Soelberg-Sørensen P, Rovaris M, Martinelli V, Hommes OR, the Early Treatment of Multiple Sclerosis Study Group (2001) Effect of early interferon treatment on conversion to definite multiple sclerosis: a randomized study. Lancet 357:1576–1582
McDonald I, Compston A (2006) The symptoms and signs of multiple sclerosis. In: Compston A, Confavreux C, Lassmann, McDonald I, Miller D, Noseworthy J, Smith K, Wekerle H (eds) McAlpine’s multiple sclerosis, 4th edn. Churchill Livingston – Elsevier, London, pp 287–346
McDonald WI, Compston DAS, Edan G, Goodkin D, Hartung HP, Lublin FD, McFarland HF, Paty DW, Polman CH, Reingold SC, Sandberg-Wollheim M, Sibley W, Thompson A, van den Noort S, Weinshenker BY, Wolinsky JS (2001) Recommended diagnostic criteria for MS. Ann Neurol 50:121–127
Miller DH, Weinshenker BG, Filippi M, Banwell BL, Cohen JA, Freedman MS, Galetta SL, Hutchinson M, Johnson RT, Kappos L, Kira J, Lublin FD, McFarland HF, Montalban X, Panitch H, Richert JR, Reingold SC, Polman CH (2008) Differential diagnosis of suspected multiple sclerosis: a consensus approach. Mult Scler 14:1157–1174
Pelayo R, Tintoré M, Rovira A, Rio J, Nos C, Grivé E, Téllez N, Comabella M, Montalban X (2007) Polyregional and hemispheric syndromes: a study of these uncommon first attacks in a CIS cohort. Mult Scler 13:731–736
Polman C, Kappos L, Freedman MS, Edan G, Hartung HP, Miller DH, Montalbán X, Barkhof F, Selmaj K, Uitdehaag BM, Dahms S, Bauer L, Pohl C, Sandbrink R, BENEFIT investigators (2008) Subgroups of the BENEFIT study: risk of developing MS and treatment effect of interferon beta-1b. J Neurol 255:480–487
Poser CM, Paty DW, Scheinberg L, McDonald WI, Davis FA, Ebers GC, Johnson KP, Sibley WA, Silberberg DH, Tourtellotte WW (1983) New diagnostic criteria for multiple sclerosis: guidelines for research protocols. Ann Neurol 13:227–231
Poser CM (1995) Onset symptoms of multiple sclerosis. J Neurol Neurosurg Psychiatry 58:253–254
Sastre-Garriga J, Tintoré M, Rovira A, Grivé E, Pericot I, Comabella M, Thompson AJ, Montalban X (2003) Conversion to multiple sclerosis after a clinically isolated syndrome of the brainstem. Cranial resonance imaging, cerebrospinal fluid and neurophysiological findings. Mult Scler 9:39–43
Sastre-Garriga J, Tintoré M, Rovira A, Nos C, Río J, Thompson AJ, Montalban X (2004) Specificity of Barkhof criteria in predicting conversion to multiple sclerosis when applied to clinically isolated brainstem síndromes. Arch Neurol 61:222–224
Tintoré M, Rovira A, Martínez MJ, Rio J, Díaz-Villoslada P, Brieva L, Borrás C, Grivé E, Capellades J, Montalban X (2000) Isolated demyelinating syndromes: comparison of different MR imaging criteria to predict conversion to clinically definite multiple sclerosis. Am J Neuroradiol 21:702–706
Uitdehaag BM, Kappos L, Bauer L, Freedman MS, Miller D, Sandbrink R, Polman CH (2005) Discrepancies in the interpretation of clinical symptoms and signs in the diagnosis of multiple sclerosis. A proposal for standardization. Mult Scler 11:227–231
Weinshenker BG, Bass B, Rice GP, Noseworthy J, Carriere W, Baskerville J, Ebers GC (1989) The natural history of multiple sclerosis: a geographically based study. 2. Predictive value of the early clinical course. Brain 112:1419–1428
Conflict of interest statement
The authors report no conflicts of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Sastre-Garriga, J., Tintoré, M., Nos, C. et al. Clinical features of CIS of the brainstem/cerebellum of the kind seen in MS. J Neurol 257, 742–746 (2010). https://doi.org/10.1007/s00415-009-5403-0
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00415-009-5403-0