Sibel Özekmekçi

Clinical features of 35 patients with Parkinson's disease displaying REM behavior disorder

OBJECTIVE To assess and compare the disease severity, the treatment properties and the frequency of motor complications in the patients with Parkinsons disease (PD) having and not having REM sleep behavior disorder (RBD). PATIENTS AND METHODS Based on chart review, patients with Parkinsons disease whose bed partners have reported prominent motor activity while dreaming were identified. Standard questionnaires assessing the presence of RBD have been addressed to these patients and their informants. Obtained data fulfilled clinical diagnostic criteria of probable RBD in 35 patients (RBD group) with the mean age at symptom onset was 61.8 years. Of them 77% were men. Clinical features of these patients concerning Hoehn-Yahr stage of PD, the severity of PD according to the Unified Parkinsons disease rating scale (UPDRS), the mean dose and duration of levodopa (LD) therapy, the presence of motor complications were compared with those of gender and age at PD-onset matched 35 PD patients without RBD (NRBD group). RESULTS The mean values of PD duration, Hoehn-Yahr stage and UPDRS scores did not differ between groups. The duration of LD therapy was significantly longer in RBD group in comparison to NRBD group (6.2 years versus 3.05 years, respectively, P<0.005) and also mean actual dose of LD was higher (460.3 mg/day versus 320.3 mg/day respectively, P<0.02). The dose and duration of dopamine agonists did not differ between groups. In RBD group, wearing-off phenomenon was significantly common (P<0.01), its duration was longer (P<0.005), and LD-related dyskinesias were more frequent (P<0.01). CONCLUSION In the current study, when compared with NRBD group, the patients with RBD required higher doses of LD treatment at an earlier stage of PD which eventually led to motor complications. In these patients, dopaminergic treatment restored UPDRS scores, but did not prevent the occurrence of RBD.

Relationship between age and subtypes of psychotic symptoms in Parkinson's disease

ObjectivePsychotic symptoms (PS) in Parkinsons disease (PD) usually develop as a side effect of the dopaminergic therapy and consist of hallucinations and delusions. We observed that PD patients who developed delusions tend to be younger than those with hallucinations and we aimed to investigate the validity of this observation.MethodsThe medical records of 127 PD patients with PS were reviewed and 76 patients who were on treatment with dopamine agonists with or without levodopa at the time of developing PS were included. Patients were stratified into 3 groups according to the subtypes of PS: patients with solely hallucinations (n = 46), solely delusions (n = 18), and both types (n = 12). The groups were compared with respect to the age-at-onset of PD and PS, duration of PD, Activities of Daily Living (ADL) and motor subscale scores of Unified PD Rating Scale (UPDRS), and levodopa equivalent dose of the dopaminergic agents administered at the time of PS onset.ResultsThe mean age-atonset of PD and PS was significantly younger (p = 0.0001) in patients with delusions (49 and 55.9 years) than those with hallucinations (61.9 and 68.9 years). The same parameters were also significantly different (p = 0.002 and p = 0.001, respectively) between the groups of patients with concurrent delusions and hallucinations (51.7 and 57.2 years) and those with only hallucinations. ADL and motor subscale scores were higher in patients with hallucinations (p = 0.016 and p = 0.013) compared with those noted in patients with delusions despite similar disease duration. The mean levodopa equivalent doses of the dopaminergic agents administered at the time of onset of PS did not differ between the groups.ConclusionThis study supported an association of delusions with younger onset of both PD and psychosis as compared with hallucinations. However, additional factors related to this association remain to be elucidated.

Features associated with the development of hallucinations in Parkinson's disease

Objective –  To identify features related to the development of hallucinations in Parkinsons disease (PD).

Long term follow-up of Parkinson's disease patients with impulse control disorders ☆

OBJECTIVE Impulse control disorders (ICDs) are mainly triggered by dopaminergic therapy in Parkinsons disease (PD). Previously, we failed to identify a relationship between the types of dopaminergic therapy and occurrence of ICDs in 33 PD patients. In this study, we aim to evaluate the outcome of ICD behaviors in the same patient group. PATIENTS AND METHODS Among 33 patients with ICDs, 22 patients were included. Demographics, dopaminergic therapy and disease severity were compared between two time points (Time 1: time of diagnosis of ICD, Time 2: last visit) and between patients who recovered from ICDs and with ongoing ICDs. Types of antipsychotic and antidepressant medication were noted. RESULTS Mean follow-up period was 43.2 months. At Time 2 mean dopamine agonist (DA) dose was significantly lower, levodopa dose and total UPDRS score was significantly higher. ICDs were dissolved in 16 patients (73%), but persisted in six (27%). In ICD(+) subgroup, DA doses in Time 1 was found significantly higher than ICD(-). However, age, PD severity, disease duration and levodopa dosage were similar. Fourteen patients were prescribed atypical antipsychotics and 13 antidepressants. In ICD(+) group, symptoms of ICDs were mainly increased libido and compulsive eating. CONCLUSIONS Although we studied a small number of patients the recovery from compulsive behaviors may be associated with the decrease in DA dosage and increase in levodopa. Furthermore, majority were given antipsychotic and/or antidepressant drugs. It is difficult to speculate which strategy could be more effective on the improvement of ICDs in such a small group. In patients who were on high doses of DA, ICDs could be persistent.

Progressive motor syndrome in a welder with pallidal T1 hyperintensity on MRI: A two-year follow-up.

Chronic exposure to manganese (Mn) fume during welding may lead to mainly extrapyramidal syndrome that is resistant to treatment. We present a 32‐year‐old patient who developed severe postural instability, Parkinsonism, dystonia, and pyramidal signs in the 10th year of welding. The neurological condition of the patient worsened markedly in the following 3 years, resulting in severe disability rendering him to be assisted in all his daily activities and he did not benefit from any dopaminergic agent. T1 sequences of the MRI of the brain showed pallidal hyperintensity symmetrically. Welders in our country often protect their eyes but ignore to use tools that protect them from inhalation of the fume. Since chronic Mn toxicity may cause serious disability and irreversible neurological disturbances, we strongly believe that it is necessary to inform welders and their employers about this potential hazard.

Clinical characteristics of 49 patients with psychogenic movement disorders in a tertiary clinic in Turkey.

Patients admitted to movement disorders outpatient unit at a university hospital between January 2002 and June 2007 were screened for psychogenic movement disorders (PMDs). Out of 1,743 patients, 49 patients (2.8%), including four children, were diagnosed to have PMDs. Women to men ratio was 34/15. The mean age and the age‐at‐onset were 41 ± 17 years and 36 ± 15 years in the adult group, and 10 ± 2 and 9 ± 2 years in children. Among the whole group, 44% had tremor, 24% dystonia, 12% pure gait disorders, 8% parkinsonism, 6% chorea‐ballism, and 4% tic disorder. PMD developed acutely in 85% of patients, and distractibility was observed in 83%. Of the patients, 81% met the criteria for clinically established PMD, whereas 16% for documented and 2% for probable PMD. Although our data was obtained from a different culture, our results showed that hospital‐based frequency and phenomenological features between our PMD group and previously reported ones are similar.

A consanguineous Turkish family with early-onset Parkinson's disease and an exon 4 parkin deletion

The importance of parkin in early‐onset Parkinsons disease in Japan, Europe, and the United States is well established. The contribution of this gene to the risk of Parkinsons disease in other populations is less well known. To explore the importance of parkin in those of Turkish ancestry, we studied familial cases from that country, and identified a consanguineous family with early‐onset Parkinsons disease due to a homozygous mutation in parkin.

A hospital-based study: risk factors in development of motor complications in 555 Parkinson's patients on levodopa therapy.

OBJECTIVES Although levodopa (LD) is the gold standard therapy for symptomatic treatment of Parkinsons disease (PD), the chronic use of LD leads to the development of motor complications in almost all patients. PATIENTS AND METHODS We assessed the presence and risk factors for motor complications in PD patients on LD therapy. We examined 555 PD patients on LD for the presence or absence of wearing-off (WO+/-) and dyskinesia (DK+/-). RESULTS WO was present in 46.3%, and DK in 30.1% of patients. The mean age at onset of symptoms were earlier in WO(+)/DK(+) groups (p<0.001). The duration of PD was longer in WO(+)/DK(+) groups (p<0.001). The time between the first symptom and the occurrence of WO/DK, or LD initiation were not significantly different. The initial LD dose was significantly higher in WO(+) compared to WO(-) (300.1mg/d versus 232.5mg/d, p<0.001), and DK(+) compared to DK(-) groups (291.4 mg/d versus 251.9 mg/d, p=0.001). The time until dopamine agonist (DA) initiation was longer in WO(+)/DK(+) groups (p<0.001). WO (p<0.001) and DK (p=0.002) were more common in patients with H&Y stages 3+4. UPDRS scores were higher in WO(+) and DK(+) patients (p<0.001 and p=0.027). CONCLUSION Our study showed that the development of motor complications was associated with early onset PD, longer disease duration, advanced disease, higher initial LD dose, longer LD use, and late DA initiation, but not with the timing of LD initiation.

Apraxia in Parkinson’s disease and multiple system atrophy

Objective:  To determine praxis function in patients with Parkinson’s disease (PD) and multiple system atrophy (MSA).

Two siblings with homocystinuria presenting with dystonia and parkinsonism

Movement disorders such as dystonia, chorea or tremor are rarely encountered in patients with homocystinuria. We present 2 siblings with laboratory‐confirmed homocystinuria, one with severe generalized dystonia and the other with mild parkinsonism. The movement disorders in our patients appeared in the second and first decades, respectively.