Artistic occupations are associated with a reduced risk of Parkinson’s disease

Parkinson’s disease (PD) is preceded by a premotor phase of unknown duration. Dopaminergic degeneration during this phase may lead to subtle cognitive and behavioural changes, such as decreased novelty seeking. Consequently, premotor subjects might be most comfortable in jobs that do not require optimal dopamine levels, leading to an overrepresentation in structured and predictable occupations, or an underrepresentation in artistic occupations. In a case–control study, 750 men with PD (onset ≥40 years) and 1300 healthy men completed a validated questionnaire about their lifetime occupational status. Occupations were classified using the RIASEC model. Odds ratios (ORs) were calculated for the conventional and artistic categories, both for the most recent occupation before symptom onset, and for the very first occupation. Because farming has been associated with a PD risk, ORs were calculated separately for farming. A reduced risk of PD was found for men with an artistic occupation late in life (OR 0.14, 95 % CI 0.04–0.53), while an artistic first occupation did not prevent PD (OR 0.72, CI 0.32–1.59). Conventional occupations showed no increased risk (recent: OR 1.07, CI 0.70–1.64; first: OR 1.14, CI 0.77–1.71). In support of previous reports, farming was associated with an increased risk of PD (recent: OR 2.6, CI 1.4–4.6; first: OR 2.7, CI 1.6–4.5). PD patients were older than controls, but various statistical corrections for age all lead to similar results. Artistic occupations late in life are associated with a reduced risk of subsequent PD, perhaps because this reflects a better preserved dopaminergic state. No initial occupation predicted PD, suggesting that the premotor phase starts later in life

Incidence and outcome of acquired demyelinating syndromes in Dutch children: update of a nationwide and prospective study

Introduction: Acquired demyelinating syndromes (ADS) are immune-mediated demyelinating disorders of the central nervous system in children. A nationwide, multicentre and prospective cohort study was initiated in the Netherlands in 2006, with a reported ADS incidence of 0.66/100,000 per year and MS incidence of 0.15/100,000 per year in the period between 2007 and 2010. In this study, we provide an update on the incidence and the long-term follow-up of ADS in the Netherlands. Methods: Children < 18 years with a first attack of demyelination were included consecutively from January 2006 to December 2016. Diagnoses were based on the International Paediatric MS study group consensus criteria. Outcome data were collected by neurological and neuropsychological assessments, and telephone call assessments. Results: Between 2011 and 2016, 55/165 of the ADS patients were diagnosed with MS (33%). This resulted in an increased ADS and MS incidence of 0.80/100,000 per year and 0.26/100,000 per year, respectively. Since 2006 a total of 243 ADS patients have been included. During follow-up (median 55 months, IQR 28–84), 137 patients were diagnosed with monophasic disease (56%), 89 with MS (37%) and 17 with multiphasic disease other than MS (7%). At least one form of residual deficit including cognitive impairment was observed in 69% of all ADS patients, even in monophasic ADS. An Expanded Disability Status Scale score of ≥ 5.5 was reached in 3/89 MS patients (3%). Conclusion: The reported incidence of ADS in Dutch children has increased since 2010. Residual deficits are common in this group, even in monophasic patients. Therefore, long-term follow-up in ADS patients is warranted

New perspectives on preclinical and early stage Parkinson's disease

Contains fulltext : 91390.pdf (publisher's version ) (Open Access)Radboud Universiteit Nijmegen, 06 december 2011Promotores : Bloem, B.R., Borm, G.F. Co-promotor : Kappelle, A.C.167 p

Phenytoin as a last-resort treatment in SCN8A encephalopathy.

Contains fulltext : 175085.pdf (publisher's version ) (Open Access

[A neonate with a sacrococcygeal mass]

Item does not contain fulltextA neonate was born with a sacrococcygeal mass. Initially, spina bifida was suspected. However, neurological examination was unremarkable. An MRI of the neuraxis showed a large cystic presacral lesion without signs of spina bifida. Surgical resection of the lesion was performed. Pathologic evaluation confirmed the diagnosis of a sacrococcygeal teratoma

About the anti-Parkinson equivalency of levodopa and dopamine agonists.

Contains fulltext : 53288.pdf (publisher's version ) (Closed access

[From psychiatric symptoms to paraneoplastic syndrome]

Contains fulltext : 69299.pdf (publisher's version ) (Open Access)Two patients, a 38-year-old man and a 32-year-old woman, were admitted to a psychiatric ward. The first patient suffered from a mood disorder, personality changes and complained of several, hitherto unexplained physical symptoms. Finally the patient was diagnosed with paraneoplastic cerebellar degeneration associated with Hodgkin's disease. The second patient presented with psychosis and panic disorders, but the condition was later found to be caused by paraneoplastic limbic encephalitis due to ovarian teratomas. These cases illustrate that patients with paraneoplastic neurological syndromes may present with psychiatric symptoms which can hamper an early diagnosis

Delayed amnesic syndrome after riluzole autointoxication in Huntington disease.

Contains fulltext : 51109.pdf (publisher's version ) (Closed access

Babinski, pseudo-Babinski, and dystonia.

Contains fulltext : 52504.pdf (publisher's version ) (Closed access

Freezer or non-freezer: clinical assessment of freezing of gait.

INTRODUCTION: Freezing of gait (FOG) is both common and debilitating in patients with Parkinson's disease (PD). Future pathophysiology studies will depend critically upon adequate classification of patients as being either 'freezers' or 'non-freezers'. This classification should be based ideally upon objective confirmation by an experienced observer during clinical assessment. Given the known difficulties to elicit FOG when examining patients, we aimed to investigate which simple clinical test would be the most sensitive to provoke FOG objectively. METHODS: We examined 50 patients with PD, including 32 off-state freezers (defined as experiencing subjective 'gluing of the feet to the floor'). Assessment including a FOG trajectory (three trials: normal speed, fast speed, and with dual tasking) and several turning variants (180 degrees vs. 360 degrees turns; leftward vs. rightward turns; wide vs. narrow turning; and slow vs. fast turns). RESULTS: Sensitivity of the entire assessment to provoke FOG in subjective freezers was 0.74, specificity was 0.94. The most effective test to provoke FOG was rapid 360 degrees turns in both directions and, if negative, combined with a gait trajectory with dual tasking. Repeated testing improved the diagnostic yield. The least informative tests included wide turns, 180 degrees turns or normal speed full turns. Sensitivity to provoke objective FOG in subjective freezers was 0.65 for the rapid full turns in both directions and 0.63 for the FOG trajectory. DISCUSSION: The most efficient way to objectively ascertain FOG is asking patients to repeatedly make rapid 360 degrees narrow turns from standstill, on the spot and in both directions