Giorgia Sciacca

Obsessive Compulsive Personality Disorder and Parkinson’s Disease

Objectives To evaluate the frequency of personality disorders in Parkinson’s disease (PD) patients and in a group of healthy controls. Methods Patients affected by PD diagnosed according to the United Kingdom Parkinson’s disease Society Brain Bank diagnostic criteria and a group of healthy controls were enrolled in the study. PD patients with cognitive impairment were excluded from the study. Structured Clinical Interview for Personality Disorders-II (SCID-II) has been performed to evaluate the presence of personality disorders. Presence of personality disorders, diagnosed according to the DSM-IV, was confirmed by a psychiatric interview. Clinical and pharmacological data were also recorded using a standardized questionnaire. Results 100 PD patients (57 men; mean age 59.0±10.2 years) and 100 healthy subjects (52 men; mean age 58.1±11.4 years) were enrolled in the study. The most common personality disorder was the obsessive-compulsive personality disorder diagnosed in 40 PD patients and in 10 controls subjects (p-value<0.0001) followed by the depressive personality disorder recorded in 14 PD patients and 4 control subjects (p-value 0.02). Obsessive-compulsive personality disorder was also found in 8 out of 16 de novo PD patients with a short disease duration. Conclusion PD patients presented a high frequency of obsessive-compulsive personality disorder that does not seem to be related with both disease duration and dopaminergic therapy.

Tremor pattern differentiates drug-induced resting tremor from Parkinson disease

OBJECTIVE DAT-SPECT, is a well-established procedure for distinguishing drug-induced parkinsonism from Parkinsons disease (PD). We investigated the usefulness of blink reflex recovery cycle (BRrc) and of electromyographic parameters of resting tremor for the differentiation of patients with drug-induced parkinsonism with resting tremor (rDIP) from those with resting tremor due to PD. METHODS This was a cross-sectional study. In 16 patients with rDIP and 18 patients with PD we analysed electrophysiological parameters (amplitude, duration, burst and pattern) of resting tremor. BRrc at interstimulus intervals (ISI) of 100, 150, 200, 300, 400, 500 and 750 msec was also analysed in patients with rDIP, patients with PD and healthy controls. All patients and controls underwent DAT-SPECT. RESULTS Rest tremor amplitude was higher in PD patients than in rDIP patients (p < 0.001), while frequency and burst duration were higher in rDIP than in PD (p < 0.001, p < 0.003, respectively). Resting tremor showed a synchronous pattern in all patients with rDIP, whereas it had an alternating pattern in all PD patients (p < 0.001). DAT-SPECT was normal in rDIP patients while it was markedly abnormal in patients with PD. CONCLUSIONS In the absence of DAT-SPECT, the pattern of resting tremor can be considered a useful investigation for differentiating rDIP from PD.

Electroencephalographic lateralization, clinical correlates and pharmacological response in untreated Parkinson's disease

BACKGROUND In Parkinsons disease (PD), different topographically defined cortical-subcortical oscillatory networks have been implicated in motor program dysfunction. Few studies have focused on clinical correlates of cortical activity asymmetry using quantitative electroencephalography. METHODS We retrospectively selected N = 34 L-dopa naïve PD subjects who had undergone standardized electroencephalography. We selected N = 18 subjects group-matched by age, sex and hand dominance with normal electroencephalography and no parkinsonism and/or cognitive decline as controls. A Welchs periodogram was applied to electroencephalographic signal epochs recorded from homologous pairs of electrodes over each hemisphere. An index of lateralization was then obtained as the absolute value of the electroencephalographic asymmetry index, computed by subtracting left from right-sided log power spectral density for each homologous site and frequency band. A standardized L-dopa acute challenge test was performed on all PD subjects to compute short-duration response magnitude. RESULTS In mid/lateral frontal regions higher index of lateralization for the beta band (p = 0.015) and lower index of lateralization for the theta band (p = 0.036) were found in PD subjects as compared to controls. Both parameters correlated with Hoehn-Yahr staging (beta: r = 0.428, p = 0.012; theta: r = -0.464, p = 0.006). In occipital region lower index of lateralization for the alpha band was found in PD correlating with L-dopa short-duration response magnitude (r = 0.456; p = 0.007). CONCLUSIONS Lateralization of frontal cortex beta electroencephalographic activity is associated with clinical disability. Occipital cortex alpha activity may relate to L-dopa responsiveness in untreated PD subjects.

Obsessive-compulsive personality disorder in drug-naïve Parkinson's disease patients.

Dear Sirs, In a previous study [1] we evaluated the presence of personality disorders (PeDs) according to the DSM-IV in PD patients and controls reporting a significantly higher frequency of obsessive–compulsive personality disorder (OCPeD) among PD patients. We carried out the present study to evaluate the frequency of OCPeD in drug-naive newly diagnosed PD patients and in a group of age and sex frequency-matched healthy controls. Drug-naive PD patients [2] were enrolled from the Movement Disorders Center of the University of Catania. Controls were recruited from 10 randomly selected general practitioners rosters in the Province of Catania. The study was approved by the local ethical committee and all the subjects signed the informed consent. Clinical evaluation was made using the unified Parkinson’s disease rating scale-motor examination section (UPDRS-ME) [3]. To exclude subjects with cognitive impairment and/or DSMIV axis I disorders, the mini mental state examination (MMSE) [4] and the structured clinical interview for DSMIV axis I (SCID I) [5] were administered. To diagnose the presence of PeDs, we adopted the structured clinical interview for DSM-IV personality disorders (SCID-II) and the associated SCID-II personality questionnaire (SCID-IIPQ) [6]. The diagnosis of PeD was confirmed by a psychiatrist who was blinded respect to the diagnosis of PD even if we cannot exclude that the presence of some motor features (i.e. tremor) could have reveled the presence of the disease. To confirm the psychiatric diagnosis, all the subjects with OCPeD have been re-evaluated by a second psychiatrist blinded regarding the first evaluation. Forty drug-naive PD patients (18 men, mean age 61.5 ± 10 years) and 40 controls (20 men, mean age 57.4 ± 10.4 years) were enrolled in the study. Age and sex distributions were not significantly different between cases and controls. The mean age at disease onset among PD patients was 59.5 ± 10.7 years with a mean disease duration of 1.7 ± 1.4 years. The UPDRS-ME mean score was 23 ± 10. OCPeD was the most common PeD diagnosed among 22 PD patients (55.0 %; 95 % CI 39.6–70.4) and 7 controls (17.5 %; 95 % CI 5.7–29.3) with an OR of 5.76 (95 % CI 2.06–16.07). The second most common PeD was the DPeD recorded among 8 (20 %; 95 % CI 0–16.41) PD patients and 4 controls (10 %, 95 % CI 0–10.07) with an OR of 2.25 (95 % CI 0.62–8.18) (Table 1). Among PD patients both OCPeD and DPeD were not significantly associated with age, sex and disease duration. Frequency of OCPeD among control subjects (17.7 %) is higher than frequency on average found in the general population, even if prevalence up to 22 % has been reported [7]. This higher frequency could be probably due to random variation related to the small sample and low number of events as suggested by the width of the 95 % CI. OCPeD is defined as a pervasive pattern of preoccupation with orderliness, perfectionism, and mental and interpersonal control at the expense of flexibility, openness, and efficiency, characteristics that overlap with the wellAlessandra Nicoletti and Antonina Luca have equally contributed to this work.

Side effects induced by the acute levodopa challenge in Parkinson's Disease and atypical parkinsonisms.

Introduction Acute levodopa challenge may be performed to predict levodopa chronic responsiveness. The aim of the study was to investigate frequency of side effects during the acute levodopa challenge in PD and atypical parkinsonisms. Methods We enrolled 34 de novo PD patients and 29 patients affected by atypical parkinsonisms (Multiple System Atrophy, MSA, n = 10; Progressive Supranuclear Palsy, PSP, n = 12 and Corticobasal Degeneration, CBD, n = 7) who underwent an acute levodopa challenge. Side effects occurring during test were recorded. Results Side effects were more frequent among atypical parkinsonisms as unique group when compared to PD patients (64.3% versus 23.5%; p-value 0.002) with an adjusted OR of 4.36 (95%CI 1.40–13.5). Each atypical parkinsonisms showed almost double occurrence of side effects (MSA 90%, PSP 41.7% and CBD 57%). Conclusions Side effects during acute levodopa challenge may be frequent in atypical parkinsonisms. This information could be useful in order to better prepare the patient for the test. Furthermore, it could represent a useful cue in differential diagnosis with PD.

Looks can be deceiving: three cases of neurological diseases mimicking Guillain–Barrè syndrome

Guillain–Barrè syndrome (GBS) is an acute, paralyzing, inflammatory peripheral nerve disease, featured by monophasic disease course, symmetrical limb weakness and areflexia. Several pathologies can mimic the clinical presentation of GBS, making hard the differential diagnosis for patients complaining of acute flaccid paralysis. In this paper we describe three cases of different neurological diseases presenting with acute motor symptoms mimicking GBS, reviewing the relevant literature on misdiagnosis of GBS.

Switching L-dopa Therapy from Pulsatile to Pulse Administration Reduces Motor Complications in Parkinsonʼs Disease

Objective To evaluate the severity of wearing-off and dyskinesia in patients with complicated Parkinson disease (PD) after switching L-dopa oral therapy from a “pulsatile” administration, consisting in intermittent multiple daily small doses of the drug, to a “pulse” administration, consisting in standard oral doses given at specific interdose intervals. Methods Thirty-four PD patients with motor complications were monitored twice with standardized waking day motor status evaluations using the Unified Parkinson Disease Rating Scale-Motor Examination (UPDRS-ME) and the Abnormal Involuntary Movement Scale (AIMS) after switching L-dopa administration modality from “pulsatile” to “pulse.” To quantify predictable motor fluctuations, a Wearing Off Index was computed based on changes in treatment response magnitude. Results On the whole, after switching from “pulsatile” to “pulse” administration, there was a reduction in number of L-dopa daily doses and an increase in the amount of the dosage of the single doses, AIMS maximum score decreased without increasing motor disability. More specifically, in predominant fluctuating patients, there was a significant reduction in UPDRS-ME average score as well as in Wearing Off Index. In predominant dyskinetic patients, there was a significant reduction in average and maximum AIMS scores with no changes in average and maximum UPDRS-ME scores. Conclusions Switching L-dopa therapy from “pulsatile” to “pulse” modality may reduce the severity of wearing-off and dyskinesia in complicated PD.

Temperament Traits And Executive Functions In Parkinson's Disease.

INTRODUCTION Aim of the study was to evaluate the possible relationship between Temperament traits and executive dysfunction in patients with Parkinsons disease (PD). METHODS Patients affected by PD diagnosed according to the UK Parkinsons disease Society Brain Bank criteria were enrolled in the study. Patients with a Mini Mental State Examination <24 were excluded from the study. The Temperament and Character Inventory (TCI), a self-report questionnaire assessing the Harm Avoidance (HA), Novelty Seeking (NS) and Reward Dependence (RD) temperamental traits, has been performed. The executive functions were assessed with the Frontal Assessment Battery (FAB). RESULTS Fifty PD patients (28 men and 22 women; mean age 59.1 ± 10.1 years) were enrolled. High HA (mean score 73.3 ± 24.7) and a low NS score (24.2 ± 18.7) were recorded. Fifteen (30%) patients presented a pathological FAB score (≤13.5). Patients with a pathological FAB score presented an HA score significantly higher than patients with normal FAB score (respectively 84.9 ± 13.7 versus 69.8 ± 26.9; p = 0.045). At the univariate analysis an association between high HA score and pathological FAB score was found (OR 3.85, 95%CI 1.06-13.9; p-value 0.040). CONCLUSION Our study confirmed an association between executive disturbances and HA in PD patients, possibly related to a common impairment of the frontostriatal circuits.

Blink reflex recovery cycle to differentiate progressive supranuclear palsy from corticobasal syndrome

Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) may share similar clinical findings and tests to distinguish between the two disorders could be useful. We evaluated the blink reflex and R2 blink reflex recovery cycle (R2BRRC), determining diagnostic sensitivity, specificity and positive and negative predictive value of R2BRRC in differentiating patients with PSP from those with CBS.

Wernicke encephalopathy and systemic sclerosis: rare association of rare conditions

Wernicke encephalopathy (WE) is an acute neurological syndrome due to thiamine (vitamin B1) deficiency. The cardinal signs are ophthalmoplegia, ataxia, and disorientation. Predisposing conditions for thiamine deficiency are gastroenteric diseases and surgery, hyperemesis gravidarum, and starvation. However, the most common cause of thiamine deficiency throughout the world is alcoholism. Herein, we report the case of a patient affected by systemic sclerosis (SS) who developed WE. The association between SS and WE has never been reported; however, SS should be considered as a clinical condition affecting the correct absorption of thiamine when history, clinical presentation, and neuroradiological features lead to the diagnosis of WE. The knowledge of such association is helpful to the clinicians to allow an adequate therapy and recovery. The patient was a 67-year-old woman with a past history of SS. She was not a drinker and previous episodes of upper respiratory or gastrointestinal infections were not reported. The patient complained of nausea and vomit and progressive weakness in her extremities for 1 month. A week before admission to our clinic she experienced an acute onset of diplopia, spatial and temporal disorientation, and personality changes with impulsivity and irritability. Physical examination revealed calcinosis cutis, sclerodactyly, telangiectasia, Raynaud’s phenomenon, and esophageal dysmotility (Video). Neurological examination disclosed complete bilateral external ophthalmoplegia, distal-dominant weakness in the four limbs, dysmetria at finger–nose, and heel to shin tests and hypo-elicitable deep tendon reflex of lower limbs (Video). Nerve conduction study revealed a sensory axonal polyneuropathy. Gastrointestinal endoscopy showed dilated esophageal tract and gastrectasia with the absence of propulsive waves and consequent gastric fluid stasis (Fig. 1). Whole body Computed Tomography (CT) scan excluded other causes of gastrointestinal symptomatology. T2-fluid-attenuated inversion recovery (FLAIR) magnetic resonance (MR) depicted hyperintense bilateral and symmetrical lesions involving the periaqueductal region and extending superiorly to the thalami and the mammillary bodies, and inferiorly to the floor of the fourth ventricle, the cerebellar vermis, and the medulla (Fig. 2, left). Hyperintensity areas were also detected in the splenium of the corpus callosum. On the basis of history, clinical presentation and characteristic findings on neuroimaging, a diagnosis of WE was established. Therefore, the patient was treated with intravenous thiamine 100 mg per day and she recovered from ophthalmoplegia and from the disturbance of consciousness in few days. Brain MR scan was repeated 7 days after starting medical therapy, showing the disappearance of the hyperintensity areas (Fig. 2, right) with the exception of the lesions at the splenium of the corpus callosum. WE is a neurological disorder resulting from thiamine deficiency, presenting with a clinical triad of ocular signs, altered consciousness and ataxia. These features are simultaneously present in only 10 % of cases [1]. Ocular abnormalities occur in 29 % of patients approximately, and Electronic supplementary material The online version of this article (doi:10.1007/s10072-016-2704-9) contains supplementary material, which is available to authorized users.