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Pilot study of nabilone in Huntington's disease (HD). Double-blind, placebo-controlled, cross-over study of nabilone versus placebo. Primary outcome, Unified Huntington's Disease Rating Scale (UHDRS) total motor score. Secondary measures: UHDRS subsections for chorea, cognition and behavior, and neuropsychiatric inventory (NPI). 44 randomized patients received either nabilone (1 or 2 mg) followed by placebo (n = 22), or placebo followed by nabilone (n = 22). Recruiting was straightforward. Nabilone safe and well tolerated, no psychotic episodes. Assessment of either dose of nabilone versus placebo showed a treatment difference of 0.86 (95% CI: -1.8 to 3.52) for total motor score; 1.68 (95% CI: 0.44 to 2.92) for chorea; 3.57 (95% CI: -3.41 to 10.55) for UHDRS cognition; 4.01 (95% CI: -0.11 to 8.13) for UHDRS behavior, and 6.43 (95% CI: 0.2 to 12.66) for the NPI. Larger longer RCT of nabilone in HD is feasible and warranted.

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.

Papers concerning the use of deep brain stimulation to the thalamus for relief of symptoms of Tourette syndrome have frequently cited a paper by Hassler and Dieckmann, published in 1970. We present a translation of this paper and discussion in English., ((c) 2008 Movement Disorder Society.)

Background: Patients on neurology wards have been shown to have high rates of psychiatric illness. Prevalence figures of 39-64% have been reported previously. However a low rate of recognition of psychiatric illness is also observed in this population., Objectives: To estimate the prevalence of psychiatric illness in neurology inpatients in a regional neuroscience centre and to assess the sensitivity and specificity of a batch of screening questionnaires., Method: Patients were assessed using the following screening questionnaires: Primary Care Evaluation of Mental Disorders, Mini Mental State Examination, Frontal Assessment Battery, Alcohol Use Disorders Identification Test and a neurologist-rating scale of organicity. All patients also had a full psychiatric assessment using the Diagnostic and statistical manual of mental disorders, 4th edition (DSM-IV). The screening questionnaires were then compared with our "gold standard", the psychiatric assessment., Results: The prevalence of psychiatric illness (as determined by the psychiatric interview) in neurology inpatients in a tertiary referral centre was found to be 51.3% (95% confidence interval 44 to 58%). The sensitivity of this batch of screening questionnaires is 81.2% and the specificity is 77.1%., Conclusion: A high prevalence of psychiatric disorder was observed in inpatients on a dedicated neurology ward. The screening questionnaires used had a high sensitivity and specificity and could therefore be used as a simple way of identifying those with psychiatric illness.

Purpose of Review: Depressions are a heterogeneous group of conditions that contribute significantly to impairments in quality of life, independent of the severity of neurological illness. Depression may predate neurological signs and symptoms in the evolution of neurodegenerative disorders, and there is some evidence that depressive illness itself may be a risk factor in the aetiology of some dementias. This review aims to summarize the relevant current literature on diagnosis, aetiology and treatment of depression in neurology., Recent Findings: Diagnosing depression in neurological conditions can be particularly difficult because of communication difficulties and changes in emotional expression as a result of the underlying neurological disease. Rating scales loaded towards somatic symptoms can show poor validity for screening or rating of severity in this setting. The evidence for the treatment of depression in neurological disease is scant, and often the treatment advice is based on consensus views of clinicians. Nevertheless, there have been some clinical trials, which are reported., Summary: Depression is common in neurology. It is underrecognized and undertreated. Recent research has allowed us to define depression more clearly in this setting. Trials of treatment are urgently needed, especially as depression is a significant factor in quality of life and may affect prognosis.

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.

Computer-mediated communication in individuals with Asperger syndrome, Tourette syndrome and normal controls was explored with a program called Bubble Dialogue (Gray, Creighton, McMahon, and Cunninghamn (1991)) in which the users type text into speech bubbles. Two scenarios, based on Happé (1994) were adapted to investigate understanding of figure of speech and sarcasm, and a third, developed by ourselves, looked at responses to inappropriate requests (lending money and disclosing home address on a first meeting). Dialogue transcripts were assessed by 62 raters who were blind to the clinical diagnoses. Hierarchical linear modelling revealed that rated understanding of a figure of speech was predicted mainly by verbal ability and executive ability, as well as by clinical diagnosis, whereas handling inappropriate requests was predicted by age, verbal ability, executive ability and diagnosis. Notably, the Tourette comparison group showed better understanding than the Asperger group in interpreting a figure of speech and handling inappropriate requests, and differences between these groups were possibly attributable to individual differences in executive ability. In contrast, understanding sarcasm was predicted by age but not by either verbal ability, executive ability or clinical diagnosis. Evidently, there is a complicated relation between Asperger syndrome, verbal ability and executive abilities with respect to communicative performance.

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.

Background: Gilles de la Tourette syndrome (GTS) is a neurobehavioural disorder of genetic origin, although the precise inheritance pattern is as yet undetermined. This study was designed to describe symptomatology in GTS subjects. It describes the severity of depressive, anxiety and obsessional symptoms when compared to other samples of GTS patients, matched controls and normative data., Methods: 87 consecutive GTS clinic referrals (children and adults) were interviewed in 1996 and 1997 using the National Hospital Interview Schedule, the Yale Global Tourette Severity Score, the Diagnostic Confidence Index, Beck Depression Inventory (BDI), Spielberger State-Trait anxiety Index (STAI) and Leyton Obsessional Inventory (LOI). 52 healthy controls were interviewed using the BDI, STAI and LOI. The clinical status of the subjects was described and ratings of psychopathology were compared with the control group and with normative data. Data were compared with previous data from similar clinic samples., Results: In this controlled study, GTS subjects scored highly on ratings of depression, state anxiety and obsessionality when compared with controls and with normative data. Scores in the subjects group were similar to previous findings in clinic samples of people with GTS., Conclusions: People with GTS who attend clinics have a variety of psychological symptoms that are significantly more severe than controls. Accessory symptoms (e.g. coprophenomena, echophenomena) were also very common in the subject group. Psychological symptoms may occur as part of the disease, a reaction to disability or as a result of ascertainment bias.

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.

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.

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.

The paper of Seignot in 1961 is the first clinical description of the use of haloperidol to treat the symptoms of Gilles de la Tourette syndrome (GTS). It is widely quoted in literature about GTS. However, it is not widely known that the single case involved had been subject to a frontal lobectomy prior to treatment with haloperidol. We present a translation of that paper and a discussion of its significance.

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.

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.

Fasting plasma levels of tryptophan, kynurenine and the pteridines, neopterin and tetrahydrobiopterin were measured in seven patients with Gilles de la Tourette syndrome (GTS) and 10 healthy controls. Plasma kynurenine was significantly elevated in the GTS patients. The lowest patient value was higher than the highest control value. Values for tryptophan, neopterin and tetrahydrobiopterin were similar in TS patients and controls. However, in TS patients only, there was a significant negative correlation between tryptophan and neopterin and a significant positive correlation between kynurenine and neopterin when controlling for tryptophan. This finding indicates that activation of cellular immune processes is a possible explanation for the rise in plasma kynurenine.

The Gilles de la Tourette syndrome (GTS) is a neurobehavioural disorder characterized by multiple motor and vocal tics. Accessory symptoms include coprophenomena, echophenomena and obsessive-compulsive behaviours. There is a significant genetic contribution to their aetiology. Simple tics may be mistaken for myoclonic epilepsy and complex tics for complex partial seizures. Like epilepsy, GTS is a brain disorder that leads to general disturbances in behaviour not just the cardinal symptoms (i.e. tics or seizures).

Four children presented to child psychiatric clinics with a variety of symptoms. They were all later recognised as having Crohn's disease. There was a significant delay between the onset of symptoms and diagnosis, compared with a control group of patients with Crohn's disease whose presentation was with predominantly gastrointestinal symptoms, which was associated with evidence of increased morbidity. Children with abdominal and psychiatric symptoms occurring in combination need serial assessments of physical status, including height and weight, and measurements of inflammatory and nutritional status.

Fasting plasma kynurenine concentrations were significantly elevated in a group of 7 patients (4 female) conforming to DSM-III-R of the American Psychiatric Association (1987) criteria for Tourette syndrome, in comparison with 10 healthy controls (7 female). Simultaneous normal plasma biopterin and neopterin concentrations indicated that this rise was probably not a consequence of peripheral cellular immune mechanisms.

The development of a malignant soft tissue tumour (malignant fibrous histiocytoma) in a surgical wound is reported. Although rare, this occurrence has important diagnostic implications and the management is discussed.