Association of Lance Adams syndrome and Post-Traumatic Stress Disorder in the context of cerebellar injury: a case report

Abstract Background: only 150 cases of LANCE-ADAMS syndrome (LAS) has been reported. In this paper, we present the case of a subject suffering from LANCE-ADAMS syndrome and post-traumatic stress disorder (PTSD) in the context of cerebellar anoxic lesions confirmed by Magnetic Resonance Imaging (MRI) and Positron Emission Tomography (PET-FDG). Case presentation: LANCE-ADAMS syndrome is a myoclonic post-anoxic syndrome and the involvement of cerebellum in pathogenesis of cortical myoclonic syndromes has been hypothesized. Prominent motor signs of our patient were adiadococinaesia in the upper limbs and ataxic dysarthria without alteration of linguistic functions. Right cerebellar hemisphere, dominant for speech production, connects with lower precentral gyrus in the left frontal lobe, and contributes to language production. This network also appears to be engaged in cognitive functions, such as verbal working memory. The main cognitive problems of our patient were difficulties in verbal episodic memory tasks and dysexecutive syndrome. Recent work has shown important functional interactions between the cerebellum and the hippocampal formation. The predominant psychiatric feature of the patient was post-traumatic stress disorder, which appeared before motor signs and persisted after improvement. It could be related to a decrease of functional connectivity between the posterior cerebellum and prefrontal regions. Conclusions: These findings suggest the potential role of the cerebellar region in the psychopathology of PTSD. Our clinical case suggests the potential link between post-traumatic stress disorder and the affective component of the entity known as the « Cerebellar Cognitive and Affective Syndrome » (CCAS) described in 1998 by Schmahmann & Sherman.