Posterior fossa syndrome with a large inflammatory ponto-mesencephalic lesion

Breit, S; Keserü, B; Nyffeler, T; Sturzenegger, Matthias; Krestel, Heinz Eric (2017). Posterior fossa syndrome with a large inflammatory ponto-mesencephalic lesion. Brain and cognition, 111, pp. 107-111. Elsevier Science 10.1016/j.bandc.2016.09.013

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Demonstration of a posterior fossa syndrome (PFS) in a 32-year-old male patient with clinically isolated syndrome which subsequently developed into relapsing-remitting Multiple Sclerosis. The patient suffered from double vision, coordination problems including unsteady gait and atactic dysarthria, concentration difficulties, as well as adynamia and impaired decision making. The patient clinically presented a cerebellar and dysexecutive syndrome. Cerebral magnetic resonance imaging (MRI) revealed a contrast enhancing ponto-mesencephalic lesion with a volume of 4.8cm(3). Neuropsychological tests showed pronounced executive dysfunctions, reduced visuoconstructive skills, attentional deficits, echolalia, and non-fluent speech production. After cortisone and plasmapheresis, the cerebellar syndrome improved but manual fine motor skills and executive dysfunctions persisted. After three months, symptoms remitted except for a slight gait imbalance. After six months, neuropsychological tests were normal except for a moderate attention deficit. MRI revealed a clear regression of the ponto-mesencephalic lesion to a volume of 2.4cm(3) without contrast enhancement. This case report intends to provide an overview of the symptomatology and etiology of PFS and offers new insights into its pathomechanism demonstrating a pontine disconnection syndrome caused by a large demyelinating plaque.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Head Organs and Neurology (DKNS) > Clinic of Neurology
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > DCR Unit Sahli Building > Forschungsgruppe Neurologie

UniBE Contributor:

Sturzenegger, Matthias and Krestel, Heinz Eric

Subjects:

600 Technology > 610 Medicine & health

ISSN:

0278-2626

Publisher:

Elsevier Science

Language:

English

Submitter:

Stefanie Hetzenecker

Date Deposited:

07 Feb 2017 16:05

Last Modified:

07 Feb 2017 16:05

Publisher DOI:

10.1016/j.bandc.2016.09.013

PubMed ID:

27842285

Uncontrolled Keywords:

Cerebellar cognitive affective syndrome; Clinically isolated syndrome; Contrast enhancing ponto-mesencephalic lesion; Disconnection syndrome; Posterior fossa syndrome

BORIS DOI:

10.7892/boris.90717

URI:

https://boris.unibe.ch/id/eprint/90717

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