Category: Ataxia
Objective: This study aimed to evaluate oculomotor disturbances in paraneoplastic cerebellar syndromes.
Background: Paraneoplastic cerebellar syndrome is an immune-mediated disorder triggered by neoplastic lesion in which immune system attacks Purkinje cells in the cerebellum typically with subacute onset. Most prominent paraneoplastic oculomotor disturbance is opsoclonus within opsoclonus-myoclonus-ataxia syndrome.
Method: The study included 8 patients with sporadic subacute cerebellar ataxia in which symptomatic and most frequent hereditary ataxias (SCA1, 2, 3, 6, 7, 17 and Friedreich’s ataxia) were excluded. All patients were neurologically examined with screening for neoplastic lesion. Antibodies directed towards intracellular antigens detection included immunohistochemistry/immunofluorescence accompanied with immunoblot. Definite diagnosis of paraneoplastic neurological disorder was done by Updated diagnostic criteria for paraneoplastic neurological syndromes [1]. For scoring oculomotor disturbances Scale for Ocular motor disorders in Ataxia (SODA) was used.
Results: All patients had subacute cerebellar ataxia in the form of gait and limb ataxia with dysarthria. Mean age of onset of the symptoms was 63±12.72 years. All patients had oculomotor disturbances. Most frequent finding was slow saccades in 38% of patients. Patients also had opsoclonus, downbeat nystagmus, gaze evoked nystagmus and vertical ophtalmoparesis in the frequency of 25% each. One patient had oculomotor apraxia and skew deviation. All patients fulfilled criteria for paraneoplastic syndrome. Small lung cancer was present in 38%, adnexal tumors 25%, and in one patient each lymphoma, ovarian and colon cancer. Antibodies directed towards intracellular antigens were positive in 50% of patients, 38% had anti-Yo antibody while one patient had anti-Ri antibody. Mean score on SODA scale was 2.25±1.98.
Conclusion: The most frequent oculomotor disturbance in our group of patients with paraneoplastic cerebellar syndromes was slow saccades, while other types such as opsoclonus, downbeat, gaze evoked nystagmus, ophtalmoparesis, oculomotor apraxia and skew deviation were seen.
References: [1] Graus F, Vogrig A, Muñiz-Castrillo S et al. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. Neurol Neuroimmunol Neuroinflamm. 2021 May 18;8(4):e1014. doi: 10.1212/NXI.0000000000001014.
To cite this abstract in AMA style:
A. Milovanović, I. Basta, N. Kresojević, O. Tamaš, A. Tomić Pešić, I. Stanković Tutuš, M. Svetel, VS. Kostić, NT. Dragašević Mišković. Oculomotor findings in paraneoplastic neurological syndromes with cerebellar ataxia [abstract]. Mov Disord. 2023; 38 (suppl 1). https://www.mdsabstracts.org/abstract/oculomotor-findings-in-paraneoplastic-neurological-syndromes-with-cerebellar-ataxia/. Accessed November 22, 2024.« Back to 2023 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/oculomotor-findings-in-paraneoplastic-neurological-syndromes-with-cerebellar-ataxia/