Maja Relja

EFNS/MDS‐ES recommendations for the diagnosis of Parkinson's disease

A Task Force was convened by the EFNS/MDS‐ES Scientist Panel on Parkinsons disease (PD) and other movement disorders to systemically review relevant publications on the diagnosis of PD.

Clinical rating scales

In Parkinsons disease (PD), rating scales are used to assess the degree of disease-related disability and to titrate long-term treatment to each phase of the disease. Recognition of non-motor symptoms required modification of existing widely used scales to integrate non-motor elements. In addition, new scales have been developed for the assessment of non-motor symptoms. In this article, assessment of PD patients will be discussed, particularly for non-motor symptoms such as pain and fatigue.

Restless Legs Syndrome in Hemodialysis Patients: Association with Calcium Antagonists

Uremia-related restless legs syndrome (RLS) is a known form of secondary RLS. This cross-sectional survey included patients (n = 82) on stable hemodialysis (HD; >3 months, Kt/V >1.2) who were iron-replete, free of neurodegenerative or psychiatric disorders, severe polyneuropathy and radiculopathy, not exposed to antipsychotics/antidepressants, and not severely anemic. Forty-nine (60%) were RLS ‘positive’, and 25 (31%) had severe/very severe symptoms (International Restless Legs Syndrome Study Group criteria). None had been diagnosed previously. In a multivariate analysis, the prevalence of RLS was higher in diabetic patients [vs. nondiabetics; prevalence ratio (PR) 2.32, 95% CI 1.50–3.60, p < 0.001] and those exposed to Ca2+ antagonists (vs. nonexposed; PR 2.02, CI 1.47–2.76, p < 0.001), and also increased with dialysis duration (PR 1.05, 95% CI 1.02–1.09, p < 0.001). Association of Ca2+ antagonists and RLS in uremic patients has not been reported previously and deserves further research.

Nondemented Parkinson disease patients : is cognitive performance associated with depressive difficulties?

BackgroundPoorer cognitive performance in depressed versus nondepressed nondemented Parkinson disease (PD) patients has been suggested. ObjectiveInvestigate the relationship between level of depression assessed on a depression-measuring scale and cognitive performance in nondemented PD patients. MethodsNondemented idiopathic PD patients (n=110) were evaluated for the level of depression [cognitive-affective items of the Becks Depression Inventory (BDI)] and performance in a set of tests evaluating cognitive domains typically affected in PD (memory, visuospatial, and executive functions). ResultsMultiple regression analysis of BDI scores demonstrated independent association of poorer cognitive performance, more severe parkinsonism, and poorer education with higher BDI scores. The association between poorer cognition and higher BDI scores was conditional on education, that is, was apparent only in less educated patients (<12u2009y of formal education). ConclusionsPoorer cognitive performance in nondemented idiopathic PD patients is associated with more severe depressive difficulties. Poorer education is also associated with more severe depression. Education modifies the cognition-depression relationship.

Acute Pisa Syndrome as a Neurological Emergency

To the Editor: Pisa syndrome is a rare clinical entity usually associated with underlying neurodegenerative diseases such as Parkinson’s disease and multiple system atrophy. However, it can also occur as an adverse effect of treatment with antipsychotics, especially in patients with predefined risk factors. Pisa syndrome is defined as sustained lateral flexion of the trunk.When observed in practice, it denotes an incapacitating symptom of underlying neurodegenerative diseases such as Parkinson’s disease and multiple system atrophy. Pisa syndrome can also occur as an adverse effect of prolonged treatment with antipsychotics, especially typical agents. However, reports describing acute drug-induced Pisa syndrome with possible treatment modalities are lacking. Here we report a patient with Alzheimer’s disease with severe risperidoneinduced acute Pisa syndrome in the neurological emergency room,whowas successfully treatedwith low-dose quetiapine.

Parkinsonian syndrome and ataxia as a presenting finding of acquired hepatocerebral degeneration

The term “acquired hepatocerebral degeneration” (AHD) was coined to describe clinical entity distinct from genetically defined Wilson’s disease. AHD is chronic neurological disorder, characterized by extrapyramidal and neuropsychiatric symptoms accompanied with advanced liver disease with portosystemic shunts. In majority of AHD cases, extrapyramidal symptoms appear in the presence of known liver disease. Here we present a patient with subacute onset of bilateral, asymmetric, hypokinetic-rigid syndrome and ataxia as initial presentation of liver cirrhosis. Manganese toxicity have major role in AHD pathogenesis. Failure of liver detoxification and presence of portosystemic shunting enables neurotoxic substance of manganese to avoid hepatic metabolism and to enter and accumulate in central nervous system. Predilection brain regions for manganese deposits are globus pallidum (GP) and substantia nigra (SN). Characteristic MRI findings of bilateral, symmetrical hyperintensities of GP and SN on T1-weighted sequences supported the diagnosis of AHD in our patient. In addition, increased T2 signal in dendate nuclei seen in our patient is rare radiological finding. So far, no consensus guidelines regarding medical treatment of AHD exist. We initiated low-dose levodopa treatment, but failed to provide beneficial effect. In conclusion, AHD is distinct clinical entity that should be included in differential diagnosis of both typical and atypical parkinsonian syndromes. Furthermore, our case highlights the importance of performing MRI in patients with atypical parkinsonism.

Brainstem nuclei changes in migraine detected by transcranial sonography

The aim of this study was to estimate the role of transcranial sonography in detecting basal ganglia changes as structural biomarkers in migraine. Transcranial sonography was performed on Aloka prosound α-10. Semiquantitative and planimetric methods were applied when basal ganglia changes were detected. Comparison between groups was performed by unpaired Student’s t test and Spearman’s correlation test. We analyzed 30 migraine patients and 30 age-/sex-matched controls. Substantia nigra hyperechogenicity was detected in 36.7% migraineurs and in 13.3% controls (t test, pxa0=xa00.036888). Hyperechogenic substantia nigra was found in 70% aura patients and in 20% patients without aura (pxa0=xa00.007384). Mean substantia nigra echogenic size of all migraine patients was 0.16xa0±xa00.07 and 0.12xa0±xa00.043xa0cm2 in controls (t test, pxa0=xa00.0011). Lentiform nucleus hyperechogenicity was seen in 50% migraine patients and 13.3% controls (t test, pxa0=xa00.002267). Mean lentiform nucleus echogenic size of all migrenous patients was 0.34xa0±xa00.08xa0cm2 and in controls 0.20xa0±xa00.008xa0cm2 (t test, pxa0=xa00.0021). Caudate nucleus hyperechogenicity was found in 26.7% migraine patients and in 6.6% controls (t test, pxa0=xa00.037667). Mean frontal horn width in migraine patients was 8.73xa0±xa01.76xa0mm and in controls 7.10xa0±xa01.71 (t test, pxa0=xa00.0006). Substantia nigra hyperechogenicity correlated with disease duration (rhoxa0=xa0−0.35521, pxa0=xa00.05467) and third ventricle width (rhoxa0=xa0−0.68221, pxa0=xa00.02976). No other differences between migraineurs and controls were found. Our study has revealed differences in transcranial findings between migraineurs and controls, but overall significance of those findings are still to be evaluated.

Collet-Sicard syndrome as a complication of chronic middle ear infection

1Department of Neurology, University of Zagreb, School of Medicine and University Hospital Centre Zagreb, Kišpatićeva 12, 10 000 Zagreb, Croatia 2Special Hospital for Chronic Diseases, Josefa Jeruzalema 7, 47 250 Duga Resa, Croatia 3Department of Neurology, University of Zagreb, School of Medicine and University Hospital Centre Zagreb, Kišpatićeva 12, 10 000 Zagreb, Croatia 4Department of Radiology, University of Zagreb, School of Medicine and University Hospital Centre Zagreb, Kišpatićeva 12, 10 000 Zagreb, Croatia

3.421 The impact of Parkinson's disease age at onset on quality of life: its effect on non-motor symptoms

Younger age at PD onset may have detrimental impact on QoL in PD patients. This could be attributed to more frequent non-motor symptoms in younger patients.