Behavioural neurology (BEHAV NEUROL)

Publications in this journal

• Article: A case of REM sleep behavior disorder, narcolepsy-cataplexy, parkinsonism and rheumatoid arthritis.

  Filomena I I Cosentino, Angela Distefano, Giuseppe Plazzi, Carlos H Schenck, Raffaele Ferri
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  ABSTRACT: A patient is reported in whom signs and symptoms of REM sleep behavior disorder (RBD) and narcolepsy have been associated for almost two decades with a late development of parkinsonism and rheumatoid arthritis. A 78-year-old male patient in whom RBD was first diagnosed was followed-up by clinical examination, videopolysomnography, multiple sleep latency test, cerebral magnetic resonance imaging, and dopamine transporter imaging by single-photon emission computerized tomography. The patient was found to present for almost two decades, in addition to RBD, also narcolepsy. Moreover, a late development of parkinsonism and the occurrence of rheumatoid arthritis were detected and clinically and instrumentally characterized. Patients predisposed to RBD and later parkinsonism might be susceptible to a variety of triggers that, in our patient, might have been represented by a possible latent autoimmune process leading to the development of narcolepsy with cataplexy and rheumatoid arthritis, later.
  Behavioural neurology 04/2013;
• Article: Delayed-onset post-stroke delusional disorder: A case report.

  Raíssa B Barboza, Gabriel R De Freitas, Fernanda Tovar-Moll, Leonardo F Fontenelle
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  ABSTRACT: Although the prevalence of neuropsychiatric disorders among patients with cerebrovascular illness is relatively high, there are only few case reports describing post-stroke psychotic symptoms. In general, post-stroke psychoses have been reported to emerge few days after the vascular event and to vanish soon afterwards. In this report, we describe delayed-onset post-stroke delusional disorder, persecutory type. A middle-aged female patient developed a persistent delusional disorder with homicidal behavior about one year after a cerebrovascular accident affecting the right fronto-temporo-parietal region and a long period of chronic post-stroke mixed anxiety and depressive symptoms. Our case suggests that there might be long intervals between stroke and the appearance of psychotic symptoms.
  Behavioural neurology 04/2013;
• Article: Optic neuritis: From magnocellular to cognitive residual dysfunction.

  Anne-Claire Viret, Céline Cavezian, Olivier Coubard, Vivien Vasseur, Noa Raz, Netta Levin, Catherine Vignal, Olivier Gout, Sylvie Chokron
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  ABSTRACT: Optic Neuritis (ON) has been associated to both parvocellular dysfunction and to an alteration of the magnocellular pathway. After objective visual field and acuity recovery, ON patients may complain about their vision suggesting a residual subclinical deficit. To better characterize visual abnormalities, 8 patients recovering from a first ON episode as well as 16 healthy controls performed a simple detection task and a more complex categorization task of images presented in low spatial frequencies (to target the magnocellular system) or in high spatial frequencies (to target the parvocellular system) or of non-filtered images. When completing the tasks with their (previously) pathologic eye, optic neuritis patients showed lower accuracy compared to controls or to their healthy eye for Low Spatial Frequency images only. Conjointly, the longest reaction times were observed with the previously pathologic eye regardless the type of images and to a greater extent in the categorization task than in the detection task. Such data suggest two distinct, although associated, types of residual dysfunction in ON: a magnocellular pathway alteration and a more general (magno and parvocellular) visual dysfunction that could implicate the cognitive levels of visual processing.
  Behavioural neurology 04/2013;
• Article: Methodological considerations in conducting an olfactory fMRI study.

  Faezeh Vedaei, Mohammad Fakhri, Mohammad Hossein Harirchian, Kavous Firouznia, Mohammad Ali Oghabian
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  ABSTRACT: The sense of smell is a complex chemosensory processing in human and animals that allows them to connect with the environment as one of their chief sensory systems. In the field of functional brain imaging, many studies have focused on locating brain regions that are involved during olfactory processing. Despite wealth of literature about brain network in different olfactory tasks, there is a paucity of data regarding task design. Moreover, considering importance of olfactory tasks for patients with variety of neurological diseases, special contemplations should be addressed for patients. In this article, we review current olfaction tasks for behavioral studies and functional neuroimaging assessments, as well as technical principles regarding utilization of these tasks in functional magnetic resonance imaging studies.
  Behavioural neurology 04/2013;
• Article: Delusion of pregnancy: A case revisited.

  A J Larner
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  ABSTRACT: A patient with delusion of pregnancy as an early feature of frontotemporal dementia with motor neurone disease (FTD/MND) who was reported some years ago was posthumously found to harbor the C9ORF72 hexanucleotide repeat expansion, now known to be the most common genetic cause of FTD/MND. Some series have found psychosis to be common in FTD associated with this mutation, so this test should be considered in individuals with clinical features of FTD and MND and/or with a family history of either disorder who present with bizarre delusions.
  Behavioural neurology 04/2013;
• Article: Topiramate for abnormal eating behaviour in frontotemporal dementia.

  Colin Singam, Mark Walterfang, Ramon Mocellin, Andrew Evans, Dennis Velakoulis
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  ABSTRACT: Topiramate is a sulfamate-substituted monosaccharide anticonvulsant that is associated with anorexia and weight loss and has been used to treat binge eating disorder and bulimia nervosa. This report describes a man with frontotemporal dementia, behavioural variant, associated with abnormal eating behaviour which appeared to respond to topiramate. We review the physiological basis of abnormal eating behaviour in frontotemporal dementia and explore possible mechanisms of action by which topiramate may modify eating behaviour in this condition.
  Behavioural neurology 04/2013;
• Article: Patterns of cognitive dysfunction in progressive MS.

  Peter Connick, Siddharthan Chandran, Thomas H Bak
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  ABSTRACT: BACKGROUND: Progressive MS is associated with a high frequency of cognitive impairment. However, it is not clear to what extent this reflects global dysfunction, or independent deficits in specific functions. OBJECTIVE: To characterise patterns of cognitive impairment in progressive MS on a multi-dimensional cognitive assessment tool well established in neurodegenerative diseases. METHODS: Patients with secondary (SPMS; n=60) and primary progressive MS (PPMS; n=28) were assessed using the Addenbrooke's Cognitive Examination-Revised (ACE-R) multi-dimensional cognitive assessment scale. Independent dimensions of impairment and their relative contribution to the overall burden of cognitive dysfunction were then determined by factor analysis. RESULTS: Two independent dimensions of impairment were seen: frontal-executive (attention, verbal fluency, recall) on one hand, and language and visuospatial functions on the other. These accounted for 55% and 45% respectively of the variance not explained by a global influence (14.2% and 11.6% respectively of total variance). Isolated language and visuospatial dysfunction was seen in both groups, whereas isolated impairment in frontal-executive functions was underrepresented in SPMS (p=0.001) and not seen in PPMS patients (p=0.040). CONCLUSIONS: In addition to a prominent global influence on cognitive performance, patients with progressive MS commonly exhibit language and visuospatial deficits. Evaluation of these abilities should therefore be included in clinical assessment of cognition in progressive MS.
  Behavioural neurology 04/2013;
• Article: Change of accent as an atypical onset of non fluent primary progressive aphasia.

  Susy Paolini, Lucia Paciaroni, Antonio Manca, Roberto Rossi, Daniela Fornarelli, Stefano F Cappa, Angela M Abbatecola, Osvaldo Scarpino
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  ABSTRACT: Language disorders can be the first symptom of many neurodegenerative diseases, including Alzheimer's disease (AD) and primary progressive aphasia (PPA). The main variants of PPA are: the non-fluent/agrammatic variant, the semantic variant and the logopenic variant.Several additional variants of PPA, however, have been described and are considered as atypical presentations. We describe the case of a woman presenting a progressive isolated language disturbance, characterized by an early dysprosodia, phonological and semantic paraphasias, agrammatism,. impairment in repetition, writing of non-words and sentence comprehension. This clinical picture pointed to an atypical presentation of the non-fluent variety. The frequent symptom overlap between the different variants of PPA, most likely reflecting differences in the topography of the pathological changes, needs to be considered in the definition of diagnostic criteria.
  Behavioural neurology 02/2013;
• Article: The qualitative scoring MMSE pentagon test (QSPT): A new method for differentiating dementia with Lewy Body from Alzheimer's disease.

  Paolo Caffarra, Simona Gardini, Francesca Dieci, Sandra Copelli, Laura Maset, Elisabetta Farina, Enzo Grossi
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  ABSTRACT: The differential diagnosis across different variants of degenerative diseases is sometimes controversial. This study aimed to validate a qualitative scoring method for the pentagons copy test (QSPT) of Mini-Mental State Examination (MMSE) based on the assessment of different parameters of the pentagons drawing, such as number of angles, distance/intersection, closure/opening, rotation, closing-in, and to verify its efficacy to differentiate dementia with Lewy Body (DLB) from Alzheimer's disease (AD). We established the reliability of the qualitative scoring method through the inter-raters and intra-subjects analysis. QSPT was then applied to forty-six AD and forty-six DLB patients, using two phases statistical approach, standard and artificial neural network respectively. DLB patients had significant lower total score in the copy of pentagons and number of angles, distance/intersection, closure/opening, rotation compared to AD. However the logistic regression did not allow to establish any suitable modeling, whereas using Auto-Contractive Map (Auto-CM) the DLB was more strongly associated with low scores in some qualitative parameters of pentagon copying, i.e. number of angles and opening/closure and, for the remaining subitems of the MMSE, in naming, repetition and written comprehension, and for demographic variables of gender (male) and education (6-13 years). Twist system modeling showed that the QSPT had a good sensitivity (70.29%) and specificity (78.67%) (ROC-AUC 0.74). The proposed qualitative method of assessment of pentagons copying used in combination with non-linear analysis, showed to be consistent and effective in the differential diagnosis between Lewy Body and Alzheimer's dementia.
  Behavioural neurology 02/2013;
• Article: The Gilles de la Tourette Syndrome-Quality of Life Scale for children and adolescents (C&A-GTS-QOL): Development and validation of the Italian version.

  Andrea E Cavanna, Chiara Luoni, Claudia Selvini, Rosanna Blangiardo, Clare M Eddy, Paola R Silvestri, Paola V Calì, Stefano Seri, Umberto Balottin, Francesco Cardona, Renata Rizzo, Cristiano Termine
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  ABSTRACT: BACKGROUND: Gilles de la Tourette syndrome (GTS) is a chronic childhood-onset neuropsychiatric disorder with a significant impact on patients' health-related quality of life (HR-QOL). Cavanna et al. (Neurology 2008; 71: 1410-1416) developed and validated the first disease-specific HR-QOL assessment tool for adults with GTS (Gilles de la Tourette Syndrome-Quality of Life Scale, GTS-QOL). This paper presents the translation, adaptation and validation of the GTS-QOL for young Italian patients with GTS.METHODS: A three-stage process involving 75 patients with GTS recruited through three Departments of Child and Adolescent Neuropsychiatry in Italy led to the development of a 27-item instrument (Gilles de la Tourette Syndrome-Quality of Life Scale in children and adolescents, C&A-GTS-QOL) for the assessment of HR-QOL through a clinician-rated interview for 6-12 year-olds and a self-report questionnaire for 13-18 year-olds. RESULTS: The C&A-GTS-QOL demonstrated satisfactory scaling assumptions and acceptability. Internal consistency reliability was high (Cronbach's alpha > 0.7) and validity was supported by interscale correlations (range 0.4-0.7), principal-component factor analysis and correlations with other rating scales and clinical variables. CONCLUSIONS: The present version of the C&A-GTS-QOL is the first disease-specific HR-QOL tool for Italian young patients with GTS, satisfying criteria for acceptability, reliability and validity.
  Behavioural neurology 01/2013; 27(1):95-103.
• Article: Parkinson disease psychosis: Update.

  J H Friedman
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  ABSTRACT: Psychotic symptoms are common in drug treated patients with Parkinson's disease (PD). Visual hallucinations occur in about 30% typically paranoid in nature, occur in about 5% particularly the delusions, cause great distress for patient and caregivers, and are among the most important precipitants for nursing home placement. Psychotic symptoms carry a poor prognosis. They often herald dementia, and are associated with increased mortality. These symptoms often abate with medication reductions, but this may not be tolerated due to worsened motor function. Only clozapine has level A evidence to support its use in PD patients with psychosis (PDP), whether demented or not. While quetiapine has been recommended by the American Academy of Neurology for "consideration," double blind placebo controlled trials have demonstrated safety but not efficacy. Other antipsychotic drugs have been reported to worsen motor function and data on the effectiveness of cholinesterase inhibitors is limited. PDP remains a serious problem with limited treatment options.
  Behavioural neurology 12/2012;
• Article: Parkinsonian patients with deficits in the dysexecutive spectrum are impaired on theory of mind tasks.

  Alberto Costa, Antonella Peppe, Matteo Martini, Katia Coletta, Massimiliano Oliveri, Carlo Caltagirone, Giovanni A Carlesimo
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  ABSTRACT: Understanding the mental states of others entails a number of cognitive processes known as Theory of Mind (ToM). A relationship between ToM deficits and executive disorders has been hypothesized in individuals with Parkinson's disease (PD). The present study was aimed at investigating the effect of dysexecutive deficits on ToM abilities in PD patients without dementia. Participants included 30 PD patients and 30 healthy subjects (HC). PD patients were divided into two groups according to their executive test performance: patients with poor (dysexecutive group; n=15) and normal (executively unimpaired group; n=15) performance. All participants were administered faux pas recognition written stories. The dysexecutive PD patients performed less accurately than both HC and executively unimpaired PD individuals on all faux pas story questions (p< 0.05); the executively unimpaired PD group performed as accurately as the HC group on the ToM tasks. Results of the study clearly demonstrate that PD is not tout court associated with ToM impairments and that these may occur in PD patients as a function of the degree of their executive impairment. Our findings also indirectly confirm previous data on the role of the prefrontal regions in mediating ToM capacities.
  Behavioural neurology 12/2012;
• Article: Dopamine dysregulation syndrome in Parkinson's disease patients with unsatisfactory switching from immediate to extended release pramipexole: A further clue to incentive sensitization mechanisms?

  Paolo Solla, Antonino Cannas, Marta Corona, Maria Giovanna Marrosu, Francesco Marrosu
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  ABSTRACT: A small proportion of patients with Parkinson's disease (PD), chronically under dopamine replacement therapy, may undergo an addiction-like behavioral disturbance, named dopamine dysregulation syndrome (DDS). This behavioral disorder is characterized by the increase of doses beyond those required for motor control, and its management remains difficult; thus, early recognition and careful monitoring of at-risk individuals are crucial. We report the cases of two PD patients with a previous unsatisfactory switching from an immediate release (IR) to an extended release (ER) pramipexole formulation who developed DDS. PD patients unsatisfactorily switched from an IR to an ER formulation of dopamine agonists should be considered as at-risk individuals for DDS development.
  Behavioural neurology 12/2012;
• Article: Possible roles of the dominant uncinate fasciculus in naming objects: A case report of intraoperative electrical stimulation on a patient with a brain tumour.

  Keiko Nomura, Hiroaki Kazui, Hiromasa Tokunaga, Masayuki Hirata, Tetsu Goto, Yuko Goto, Naoya Hashimoto, Toshiki Yoshimine, Masatoshi Takeda
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  ABSTRACT: How the dominant uncinate fasciculus (UF) contributes to naming performance is uncertain. In this case report, a patient with an astrocytoma near the dominant UF was given a picture-naming task during intraoperative electrical stimulation in order to resect as much tumourous tissues as possible without impairing the dominant UF function. Here we report that the stimulations with the picture-naming task also provided some insights into how the dominant UF contributes to naming performance. The stimulation induced naming difficulty, verbal paraphasia, and recurrent and continuous perseveration. Moreover, just after producing the incorrect responses, the patient displayed continuous perseveration even though the stimulation had ended. The left UF connects to the inferior frontal lobe, which is necessary for word production, so that the naming difficulty appears to be the result of disrupted word production caused by electrical stimulation of the dominant UF. The verbal paraphasia appears to be due to the failure to select the correct word from semantic memory and the failure to suppress the incorrect word. The left UF is associated with working memory, which plays an important role in recurrent perseveration. The continuous perseveration appears to be due to disturbances in word production and a failure to inhibit an appropriate response. These results indicate that the dominant UF has multiple roles in the naming of objects.
  Behavioural neurology 12/2012;
• Article: Alexithymia and apathy in Parkinson's disease: Neurocognitive correlates.

  Yelena Bogdanova, Alice Cronin-Golomb
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  ABSTRACT: Non-motor symptoms such as neuropsychiatric and cognitive dysfunction have been found to be common in Parkinson's disease (PD) but the relation between such symptoms is poorly understood. We focused on alexithymia, an impairment of affective and cognitive emotional processing, as there is evidence for its interaction with cognition in other disorders. Twenty-two non-demented PD patients and 22 matched normal control adults (NC) were administered rating scales assessing neuropsychiatric status, including alexithymia, apathy, and depression, and a series of neuropsychological tests. As expected, PD patients showed more alexithymia than NC, and there was a significant association between alexithymia and disease stage. Alexithymia was associated with performance on non-verbally mediated measures of executive and visuospatial function, but not on verbally mediated tasks. By contrast, there was no correlation between cognition and ratings of either depression or apathy. Our findings demonstrate a distinct association of alexithymia with non-verbal cognition in PD, implicating right hemisphere processes, and differentiate between alexithymia and other neuropsychiatric symptoms in regard to PD cognition.
  Behavioural neurology 12/2012;
• Article: Attentional networks in Parkinson's disease.

  Chiara Cristinzio, Monica Bononi, Sylvie Piacentini, Alberto Albanese, Paolo Bartolomeo
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  ABSTRACT: We tested the efficiency of three attentional systems (spatial orienting, phasic alerting and executive control) in patients with Parkinson's disease (PD), by using a modified version of the Attention Network Test, which employs acoustic tones to modulate phasic alertness. PD patients were generally slower than age-matched controls, but they showed a similar pattern of effects and interactions. Responses were faster with congruent than with incongruent stimuli (executive control), with valid visual cues than with invalid or no cues (orienting), and when acoustic tones preceded the target (alerting). This last effect was significantly larger in PD patients than in controls. We concluded that, for the present group of patients, the activity of attentional networks was relatively normal, if slowed. Slowed responses in PD may be improved by the use of acoustic stimuli, with potential clinical implications.
  Behavioural neurology 12/2012;
• Article: Impulse control disorders in Parkinson's disease: Crossroads between neurology, psychiatry and neuroscience.

  Paulo Bugalho, Albino J Oliveira-Maia
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  ABSTRACT: Non-motor symptoms contribute significantly to Parkinson's disease (PD) related disability. Impulse control disorders (ICDs) have been recently added to the behavioural spectrum of PD-related non-motor symptoms. Such behaviours are characterized by an inappropriate drive to conduct repetitive behaviours that are usually socially inadequate or result in harmful consequences. Parkinson disease impulse control disorders (PD-ICDs) have raised significant interest in the scientific and medical community, not only because of their incapacitating nature, but also because they may represent a valid model of ICDs beyond PD and a means to study the physiology of drive, impulse control and compulsive actions in the normal brain. In this review, we discuss some unresolved issues regarding PD-ICDs, including the association with psychiatric co-morbidities such as obsessive-compulsive disorder and with dopamine related side effects, such as hallucinations and dyskinesias; the relationship with executive cognitive dysfunction; and the neural underpinnings of ICDs in PD. We also discuss the contribution of neuroscience studies based on animal-models towards a mechanistic explanation of the development of PD-ICDs, specifically regarding corticostriatal control of goal directed and habitual actions.
  Behavioural neurology 12/2012;
• Article: Towards a deeper comprehension of relationships among cognitive, behavioral and psychiatric symptoms in Parkinson's disease.

  Luigi Trojano, Gabriella Santangelo, Massimiliano Conson, Dario Grossi
  Behavioural neurology 12/2012;
• Article: Apathy in Parkinson's disease: Diagnosis, neuropsychological correlates, pathophysiology and treatment.

  Gabriella Santangelo, Luigi Trojano, Paolo Barone, Domenico Errico, Dario Grossi, Carmine Vitale
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  ABSTRACT: Apathy has been defined as lack of motivation. It has been traditionally considered as a symptom of psychiatric disorders, such as major depression and schizophrenia, but more recently it has been recognized as a specific neuropsychiatric syndrome associated with neurodegenerative such as Parkinson's disease (PD). As a consequence the reported prevalence of apathy in PD ranges from 13.9% to 70%; the mean prevalence is 35%. Prevalence of "pure apathy" (i.e., of apathy without comorbid depression and dementia) seems to be substantially lower, from 3 to 47.9%. High levels of apathy in PD are associated with decreased daily function, specific cognitive deficits and increased stress for families. Although neuroimaging studies do not provide a unique anatomic pattern, several data suggest that the ventromedial prefrontal cortex and the basal ganglia connected through frontal-subcortical circuits, are particularly involved in the genesis of apathy. At present, there are no approved medications for the treatment of apathy in and no proof of efficacy exists for any drug in current use. Further studies and innovative pharmacologic approaches are thus needed to ameliorate our understanding and treatment of apathy in PD.
  Behavioural neurology 12/2012;
• Article: Compulsive drumming induced by dopamine agonists treatment in Parkinson's disease: Another aspect of punding.

  Carmine Vitale, Luigi Trojano, Paolo Barone, Domenico Errico, Valeria Agosti, Giuseppe Sorrentino, Dario Grossi, Gabriella Santangelo
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  ABSTRACT: We report the case of a man affected by Parkinson's disease who developed an unusual, severe, repetitive behavior characterized by an irrepressible need to drum and beat percussion instruments following to the introduction of pramipexole. This compulsive behavior was not associated to a pattern of chronic inappropriate overuse of dopaminergic medication or other psychiatric symptoms. Sharing many features with other repetitive behaviors, compulsive drumming might be considered a distinct manifestation of punding in Parkinson's disease.
  Behavioural neurology 12/2012;