Ganglion Cell Complex Thinning in Young Gaucher Patients: Relation to Prodromal Parkinsonian Markers
Autor: | Mai Seif El Din Abdeen, Nouran Yousef Salah, Noureldin Hussein Hashem, Weam Mohamed Ebeid, Azza A.G. Tantawy, Amira Abdel Moneam Adly |
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Rok vydání: | 2020 |
Předmět: |
0301 basic medicine
medicine.medical_specialty Medication history Adolescent REM Sleep Behavior Disorder Gastroenterology Retina 03 medical and health sciences Young Adult 0302 clinical medicine Hyposmia Internal medicine medicine Humans Child Wechsler Intelligence Scale for Children Gaucher Disease Intelligence quotient business.industry Parkinsonism Beck Depression Inventory Wechsler Adult Intelligence Scale Parasomnia medicine.disease 030104 developmental biology Neurology Neurology (clinical) medicine.symptom business 030217 neurology & neurosurgery Biomarkers Tomography Optical Coherence |
Zdroj: | Movement disorders : official journal of the Movement Disorder SocietyReferences. 35(12) |
ISSN: | 1531-8257 |
Popis: | BACKGROUND Patients with Gaucher disease (GD) have an increased risk for parkinsonism. Retinal thinning has been described in parkinsonism as an early nonmotor feature. Scarce reports have addressed retinal thickness changes in GD. OBJECTIVES The objectives of this study were to compare ganglion cell complex (GCC) thickness in adolescents and young adults (AYAs) with GD with healthy control subjects, and to correlate it with the presence of parkinsonian features (PFs), clinical prodromal markers of parkinsonism, severity score index (SSI), and glucosylsphingosine (Lyso-GL-1). METHODS This study included 48 AYAs with GD (11-29 years), 11 with manifest PFs (Group 1) and 37 with no PFs (Group 2), and 48 matched healthy control subjects (Group 3). Age of GD onset, disease duration, medication history, history of constipation, SSI, and hematological assessment were done. Neurocognitive evaluation included Parts I, II, and III of the Unified Parkinson's Disease Rating Scale (UPDRS), Wechsler Adult and Intelligence Scale and Wechsler Intelligence Scale for Children, Beck Depression Inventory (BDI), rapid eye movement sleep behavior disorder (RBD) scale, Munich Parasomnia Screening scale, and the olfactory dysfunction scale. Molecular analyses of the acid GBA gene and Lyso-GL-1 were done. Participants underwent full ophthalmological examination and optical coherence tomography with GCC thickness measurement. RESULTS GCC was significantly thinner in Group 1 than in Groups 2 and 3 (P |
Databáze: | OpenAIRE |
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