gms | German Medical Science

Objective: Cerebral cavernous malformations (CCM) most frequently become manifest by seizures, focal neurologic deficits or headaches. Movement disorders (MDs) secondary to CCM have only rarely been described in thalamic, basal ganglia and brainstem CCM. Owing to their rarity, awareness of this association is limited, while optimal management remains uncertain.

Methods: In this study, we included all patients with MDs associated with a CCM who presented in our department over a period of 10 years. MDs secondary to other cerebrovascular lesions or spinal cavernous malformations were excluded. In addition to our case series clinical and radiological findings of relevant previous reports were reviewed and treatment approaches are discussed.

Results: We encountered a total of 6 patients (5 women and 1 man). Mean age was 38,5 years (range: 16-77 years). In all cases radiographic sign of previous haemorrhage was evident. MDs secondary to CCM located in various locations included: hemiparkinsonism – anterolateral pontomesencephalon (1); chorea – subthalamic region (1); tremor – subthalamic region/dentatothalamic pathways (2); dystonia – putamen/thalamomesencephalic region (2). Two patients underwent surgical removal of the CCM with subsequent resolution of MDs. One patient had radiosurgery with disappearance of tremor but occurrence of spasticity. In three patients with moderate severity of MDs a conservative approach was adopted. A review of the relevant literature revealed a total of 37 cases of CCM associated MD.

Conclusion: Movement disorders secondary to CCM may include chorea/ballism, tremor, parkinsonism and dystonia. They usually present unilaterally on the contralateral side to the CCM. The onset of MDs is variable with dystonic syndromes and hypokinetic syndromes mostly showing a subacute or chronic course, whereas chorea/ballism and tremor have a sudden occurrence. CCM are dynamic lesions, which can exhibit enlargement or regression and therefore the natural course of associated MDs is difficult to predict. Although spontaneous resolution is possible, it appears that CCM-related MDs may persist or worsen within several years. The treatment strategy should be chosen on an individual basis.