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4.
Acta Neurol Scand ; 126(6): 376-83, 2012 Dec.
Article in English | MEDLINE | ID: mdl-22404634

ABSTRACT

OBJECTIVES: People with Huntington's disease (HD) can exhibit interpersonal difficulties and deficits in recognizing emotional facial expressions. We investigated whether individuals with HD exhibit impairments in the understanding of other people's mental states, an aspect of Theory of Mind (ToM). MATERIALS AND METHODS: Sixteen patients with HD and sixteen healthy controls completed two ToM tasks. One task involved recognising socially inappropriate behaviour and the other task required participants to judge complex mental states from photographs of people's eyes alone. To assess relationships between executive function and ToM, participants completed measures of verbal fluency, working memory and inhibition. The Problem Behaviours Assessment-short form (Neuropsychiatry Neuropsychol Behav Neurol, 14, 2001and 219) was completed twice using information from patients and their close relatives (where possible) to identify relationships between ToM impairment and behavioural problems. RESULTS: Patients with HD made significantly more errors on ToM tasks than controls, exhibiting difficulties in judging the social appropriateness of story character's behaviour and problems inferring complex mental states from photographs of people's eyes. Patients with HD also exhibited executive dysfunction. However, there was little evidence that executive impairments were related to ToM deficits. No correlations were apparent between problem behaviours and ToM errors. CONCLUSIONS: HD is associated with deficits in ToM. Furthermore, some of patients' ToM difficulties appear independent of executive dysfunction.


Subject(s)
Cognition Disorders/etiology , Huntington Disease/psychology , Theory of Mind , Cognition Disorders/psychology , Humans , Huntington Disease/complications , Middle Aged , Neuropsychological Tests
5.
Lancet ; 376(9751): 1463-4, 2010 Oct 30.
Article in English | MEDLINE | ID: mdl-21036268
7.
Neurology ; 73(17): 1375-80, 2009 Oct 27.
Article in English | MEDLINE | ID: mdl-19858459

ABSTRACT

BACKGROUND: Eighteen patients with severe and refractory Tourette syndrome (TS) underwent bilateral thalamic deep brain stimulation (DBS). OBJECTIVE: To assess the long-term outcome on tics, behavioral symptoms, and cognitive functions in the largest case series of thalamic DBS for TS to date. METHODS: In this prospective cohort study, 15 of the original 18 patients were evaluated before and after surgery according to a standardized protocol that included both neuropsychiatric and neuropsychological assessments. RESULTS: In addition to marked reduction in tic severity (p = 0.001), 24-month follow-up ratings showed improvement in obsessive-compulsive symptoms (p = 0.009), anxiety symptoms (p = 0.001), depressive symptoms (p = 0.001), and subjective perception of social functioning/quality of life (p = 0.002) in 15 of 18 patients. There were no substantial differences on measures of cognitive functions before and after DBS. CONCLUSIONS: At 24-month follow-up, tic severity was improved in patients with intractable Tourette syndrome (TS) who underwent bilateral thalamic deep brain stimulation. Available data from 15 of 18 patients also showed that neuropsychiatric symptoms were improved and cognitive performances were not disadvantaged. Controlled studies on larger cohorts with blinded protocols are needed to verify that this procedure is effective and safe for selected patients with TS. LEVEL OF EVIDENCE: This study provides class IV evidence that bilateral thalamic deep brain stimulation reduces global tic severity measured 24 months after implantation in patients with severe intractable Tourette syndrome.


Subject(s)
Deep Brain Stimulation , Thalamus/physiopathology , Tourette Syndrome/therapy , Adolescent , Adult , Anxiety/physiopathology , Anxiety/therapy , Depression/physiopathology , Depression/therapy , Drug Resistance , Female , Follow-Up Studies , Humans , Male , Middle Aged , Neuropsychological Tests , Obsessive-Compulsive Disorder/physiopathology , Obsessive-Compulsive Disorder/therapy , Prospective Studies , Quality of Life , Self Concept , Severity of Illness Index , Tourette Syndrome/physiopathology , Treatment Outcome , Young Adult
9.
J Neuropsychiatry Clin Neurosci ; 17(1): 119-21, 2005.
Article in English | MEDLINE | ID: mdl-15746492

ABSTRACT

Patients with Huntington's disease show deficits in recognizing disgust in the facial expressions and vocal intonations of others. In this study, the authors demonstrate that these disgust-related deficits extend to foul-smelling olfactory stimuli and inappropriate combinations of taste stimuli.


Subject(s)
Huntington Disease/physiopathology , Perceptual Distortion/physiology , Smell/physiology , Taste/physiology , Humans
11.
Parkinsonism Relat Disord ; 10(2): 59-65, 2003 Dec.
Article in English | MEDLINE | ID: mdl-14643994

ABSTRACT

Depression is the most common psychiatric disturbance in Parkinson's disease. We conducted a Cochrane systematic review to assess the efficacy and safety of antidepressant therapies in idiopathic Parkinson's disease. Relevant trials were identified from electronic databases, reference lists and queries to antidepressant manufacturers. Three randomised controlled trials examined oral antidepressants in 106 patients with Parkinson's disease. No eligible trials of electroconvulsive or behavioural therapy were found. In the first arm of the crossover trial by Andersen et al. (n=22), nortriptyline treated patients showed a larger improvement than placebo in a unique depression rating scale after 16 weeks although significance levels were not provided. A parallel group trial by Wermuth et al. (n=37) did not show any significant difference between citalopram and placebo in Hamilton score after 52 weeks. Rabey et al. (n=47) performed an open-label trial comparing fluvoxamine with amitriptyline. Similar numbers in each group had a 50% reduction in Hamilton score after 16 months. Major side effects including visual hallucinations and confusion were reported with fluvoxamine and amitriptyline. Insufficient data on the effectiveness and safety of antidepressant therapies in Parkinson's disease are available on which to make recommendations for their use. Large scale randomised controlled trials are urgently required.


Subject(s)
Antidepressive Agents/therapeutic use , Depression/drug therapy , Parkinson Disease/drug therapy , Randomized Controlled Trials as Topic/methods , Depression/complications , Depression/psychology , Humans , Parkinson Disease/complications , Parkinson Disease/psychology
13.
Exp Brain Res ; 148(3): 308-19, 2003 Feb.
Article in English | MEDLINE | ID: mdl-12541142

ABSTRACT

In both Parkinson's disease and Huntington's disease, proprioceptive sensory deficits have been suggested to contribute to the motor manifestations of the disease. Here, proprioceptive sensory function was investigated in Parkinson's disease patients, Huntington's disease patients, and healthy control subjects (each group n=8), using proprioception-related evoked potentials. Proprioception-related potentials were elicited by passive index finger movements and measured with high-density EEG. Conventional median nerve somatosensory evoked potentials (mnSEPs) were recorded in the same session. Analysis included amplitude and latency measures from selected scalp electrodes and dipole source reconstruction. We found a proprioception-related N90 component of normal latency in both Parkinson's disease and Huntington's disease. The source strength of the underlying cortical generator was normal in Parkinson's disease, but marginally reduced in Huntington's disease. Using the source location of the N20-P20 component of the mnSEP as a landmark for postcentral area 3b, the N90 was localized to the precentral motor cortex. At a latency around 170-180 ms proprioception-related potentials were explained by bilateral sensory cortex activation with an altered distribution in Parkinson's disease and a reduction of ipsilateral activation in Huntington's disease. Together, the results show largely normal early proprioception-related potentials, but changes in the cortical processing of kinaesthetic signals at longer latencies in both diseases.


Subject(s)
Electroencephalography , Evoked Potentials/physiology , Huntington Disease/physiopathology , Parkinson Disease/physiopathology , Proprioception/physiology , Adult , Aged , Brain Mapping , Evoked Potentials, Somatosensory/physiology , Female , Functional Laterality/physiology , Humans , Male , Median Nerve/physiopathology , Middle Aged , Scalp/physiopathology
16.
J Neurol Sci ; 166(2): 152-3, 1999 Jul 01.
Article in English | MEDLINE | ID: mdl-10475109
17.
Hosp Med ; 60(10): 740-2, 1999 Oct.
Article in English | MEDLINE | ID: mdl-10656068

ABSTRACT

Catatonia is commonly encountered in psychiatric and medical practice but is under-recognized. It occurs in association with a wide range of disorders and drugs. Psychiatric education and textbooks mistakenly only consider catatonia as a subtype of schizophrenia. This article, the first of two, reviews the development of the concept of catatonia, its epidemiology, clinical features and pathophysiology.


Subject(s)
Catatonia , Catatonia/diagnosis , Catatonia/epidemiology , Catatonia/history , Catatonia/physiopathology , History, 19th Century , History, 20th Century , Humans
18.
Hosp Med ; 60(11): 812-4, 1999 Nov.
Article in English | MEDLINE | ID: mdl-10707192

ABSTRACT

This is the second of two articles reviewing catatonia. In the first, catatonia was described as an under-recognized syndrome with a potentially fatal outcome. It was suggested that treatment with neuroleptics may exacerbate the syndrome. The differential diagnosis, management and prognosis of catatonia are reviewed below.


Subject(s)
Catatonia/diagnosis , Catatonia/therapy , Benzodiazepines/therapeutic use , Diagnosis, Differential , Electroconvulsive Therapy , Humans , Hypnotics and Sedatives/therapeutic use , Lorazepam/therapeutic use , Neuroleptic Malignant Syndrome/diagnosis , Prognosis , Serotonin Syndrome/diagnosis
19.
Mov Disord ; 12(4): 531-5, 1997 Jul.
Article in English | MEDLINE | ID: mdl-9251071

ABSTRACT

Retching and vomiting are common symptoms in childhood. We describe the cases of 10 patients with Gilles de la Tourette syndrome (GTS) for whom vomiting or retching tics were part of the clinical picture, and discuss other instances where retching and vomiting occur with neuropsychiatric or movement disorders.


Subject(s)
Gagging , Tourette Syndrome/complications , Vomiting/etiology , Adolescent , Adult , Age of Onset , Attention Deficit Disorder with Hyperactivity/complications , Child , Child, Preschool , England , Female , Humans , Male , Middle Aged , Obsessive-Compulsive Disorder/complications , Tourette Syndrome/physiopathology , Vomiting/physiopathology
20.
Exp Neurol ; 144(2): 420-2, 1997 Apr.
Article in English | MEDLINE | ID: mdl-9168842

ABSTRACT

Erythrocyte measures of copper-zinc superoxide dismutase (CuZnSOD) were performed on 11 subjects with a clinical diagnosis of Gilles de la Tourette syndrome (GTS) and 6 healthy controls at specified intervals throughout the day. There were no significant differences between GTS subjects and controls but in both subjects and controls there was a significant increase in SOD, 75 min postprandially, which decreased to baseline 135 min postprandially. This has implications for the timing of biological samples in future studies of SOD. Possible reasons for the increase are discussed.


Subject(s)
Eating/physiology , Erythrocytes/enzymology , Superoxide Dismutase/blood , Tourette Syndrome/enzymology , Adolescent , Adult , Circadian Rhythm , Fasting/physiology , Female , Humans , Male , Middle Aged
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