CLINICAL AND PARACLINICAL FEATURES OF CORTICOBASAL DEGENERATION
DOI:
https://doi.org/10.11603/1811-2471.2025.v.i4.15813Keywords:
cortico-basal degeneration, cortico-basal syndrome, atypical parkinsonism, dystonia, "alien limb" syndrome, dementiaAbstract
SUMMARY. Cortico-basal degeneration (CBD) is a rare neurodegenerative disease. The leading syndrome of CBD is asymmetric rigidity and akinesia, so the differential diagnosis with Parkinson’s disease (PD), especially in the early stages of the disease, is challenging.
The aim – to evaluate the clinical and paraclinical features of сortico-basal syndrome (CBS), as one of the most common CBD phenotypes, to improve the differential diagnosis with PD.
Material and Methods. 6 patients with a diagnosis of CBS aged 52 to 64 years were examined. All patients underwent clinical-neurological and neuropsychological examination: cognitive functions (MOCA test, frontal assessment battery (FAB)), HADS scale to determine the level of anxiety and depression;brain MRI.
Results. The initial manifestations of the disease were as follows: unilateral rigidity and oligobradykinesia, symptom of "alien hand", dystonia, myoclonus, gait disturbance, limb apraxia, static-kinetic tremor, speech problems. Asymmetric akinetic-rigid Parkinsonism developed in all patients 3–4 years after the first signs of the disease. The most common manifestations were oligobradykinesia – in 83.3 %, the syndrome of "alien limb" – in 66.7 %, ideomotor apraxia – in 66.7 %, postural instability – in 50.0 %, unilateral limb dystonia – in 50.0 % of patients. Cognitive impairment reached the level of demencia according to neuropsychological scales. The average score on the MOCA scale was 18.2±0.4, and FAB scale – 10.1±0.3 points. MRI of the brain did not show specific changes in the early stages of the disease in 66.7% of patients. In the later stages, MRI showed moderate asymmetric atrophy in the parietal area or in the posterior frontal and parietal areas, a decrease signal from the basal ganglia and dilation of the lateral ventricles in the fronto-parietal areas.
Conclusions. The most significant differential clinical and paraclinical features of CBS from PD are: levodoresistance, "alien hand" syndrome, dystonia and myoclonus of the limb, apraxia, presence of disorders of complex types of sense, atrophy of the fronto-parietal brain cortex on MRI. Difficulties in diagnosing of CBD in the early stages of the disease are often due to: monosymptomatic nature at the beginning of the disease, underestimation of other neurological symptoms, hyperdiagnosis of "Vascular Parkinsonism", absence of typical atrophic changes in MRI.
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