Objective: Recall clinical situations of hyperkinetic movement disorders (HMD) in a Tunisian cohort.

Background: HMD result from a dysfunction of the basal ganglia(BG) and are characterized by an uncontrollable excess of movement. With heterogenous presentations and underlying conditions, they can range from mildly to severely debilitating.

Method: We conducted an observational study on patients who were hospitalized for management of HMD from 2016 to 2022. Family history, clinical and paraclinical data were collected.

Results: The median age of 25 patients was 51 years old (IQR=[32.5,62]). Acute and progressive time course were reported in 13 and 12 cases respectively. In progressive onset, we noted chorea, a HMD association, dystonia and tremor in 7, 3, 1 and 1 cases respectively. The majority (9 cases) had a positive family history and 10 patients had associated symptoms (cognitive impairment, peripheral neuropathy, gait disorders). Diagnoses were Huntington Disease (6 cases), paraneoplastic and genetic HMD (2 cases respectively), drug induced cause and neuroacanthocytosis (one case respectively). As for patients having an acute onset, we identified chorea (11 cases), dystonia and ballism (one case respectively). Distribution was mainly hemicorporeal (9 cases) and generalized (2 cases). Cerebral MRI revealed BG T1 hyperintensities (7 cases), lacunar infracts (4 cases) and a medullary demyelinating lesion (one patient). Etiologies were metabolic causes (7 cases: nonketotic hyperglycinemia in 5 patients, Gayet Wernicke encephalopathy sequalae and iron accumulation in 1 case respectively), stroke (4 cases), a Multiple Sclerosis relapse and a drug-induced chorea in one case respectively.

Conclusion: HMD are characterized by excessive movements, which can be isolated or associated with other neurological or non-neurological signs. With various possible causes, mode of onset and distribution are very important in helping to identify it. In our cohort as in literature, progressive onset was mostly seen with genetic etiologies, suspected especially with a positive family history and associated specific neuropsychiatric symptoms. For acute onset, it can be associated with potentially curable situations. In our cohort, metabolic etiology was more frequent then in literature. It is mainly hemicorporeal and represents a differential diagnosis with a vascular origin, which is the second most common cause in our series and literature.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/spectrum-of-hyperkinetic-movement-disorders-in-a-tunisian-cohort-an-observational-study/