More severe functional impairment in dementia with lewy bodies than Alzheimer disease is related to extrapyramidal motor dysfunction.

OBJECTIVE: The objective of this study was to compare functional impairments in dementia with Lewy bodies (DLB) and Alzheimer disease (AD) and their relationship with motor and neuropsychiatric symptoms. METHODS: The authors conducted a cross-sectional study of 84 patients with DLB or AD in a secondary care setting. Patients were diagnosed according to published criteria for DLB and AD. The Bristol Activities of Daily Living Scale (BADLS) was used to assess functional impairments. Participants were also assessed using the Unified Parkinson's Disease Rating Scale (motor section), the Neuropsychiatric Inventory, and the Mini-Mental Status Examination. RESULTS: Patients with DLB were more functionally impaired and had more motor and neuropsychiatric difficulties than patients with AD with similar cognitive scores. In both AD and DLB, there were correlations between total BADLS scores and motor and neuropsychiatric deficits. There was more impairment in the mobility and self-care components of the BADLS in DLB than in AD, and in DLB, these were highly correlated with UPDRS score. In AD, orientation and instrumental BADLS components were most affected. CONCLUSION: The nature of functional disability differs between AD and DLB with additional impairments in mobility and self-care in DLB being mainly attributable to extrapyramidal motor symptoms. Consideration of these is important in assessment and management. Activities of daily living scales for use in this population should attribute the extent to which functional disabilities are related to cognitive, psychiatric, or motor dysfunction.     

Targeted Intra-Arterial Chemoradiation for Advanced Tonsil Cancer

OBJECTIVES: To determine the effects of combined radiation and targeted, intra-arterial (IA) cisplatin infusions (RADPLAT) in patients with advanced squamous cell carcinoma (SCC) of the tonsil. STUDY DESIGN AND SETTING: Prospective study of treatment outcomes and toxicity of patients enrolled on the RADPLAT protocol, with specific analysis of patients with advanced SCC of the tonsil. RESULTS: Thirty patients with advanced tonsil carcinoma (17 T(4), 12 T(3), 1 T(2)) were enrolled, and 24 of 30 patients completed at least 3 IA cisplatin infusions and a minimum of 63 Gy or radiation therapy (minimum therapy). Two-year estimated overall and disease-specific survival was 42% and 50%, respectively, for all 30 patients (intent-to-treat group) and 49% and 58%, respectively, for the minimum therapy subgroup. The 2-year estimated local and regional disease control was 87% and 90%, respectively, for the intent-to-treat group, and 100% and 90% for the minimum therapy subgroup. Functional organ preservation was achieved in 92% of patients. CONCLUSIONS: Locoregional disease control achieved with this regimen appears to be significantly improved over that described in the literature for similarly staged tonsil cancer. Survival, on the other hand, remains comparable.     

Rett syndrome, EEG and the motor cortex as a model for better understanding of attention deficit hyperactivity disorder (ADHD)

EEG data in Rett syndrome (RS) are quite often characterized by spikes over the central region (especially in sleep). Furthermore, there has been a recent observation of a prominent and peculiar theta (4–6/s) rhythm over the central region and vicinity. In view of the clinical phenomena of RS, it is pointed out that clinical and electrical findings are based upon hyperexcitability of the motor cortex (or a state of motor cortex dyscontrol). States of dyscontrol (disinhibition) are caused by deficits in higher cortical functions. It is reasonable to assume a primary dysfunction for the frontal lobe which is also smaller in size than other cerebral regions. The behavioral (motor) disturbance in RS can be easily explained as the expression of disturbed prefrontal/premotor function with special effect on the „syntax of action”. It is further theorized that such a state of fronto-motor cortex uncoupling may also exist in attention deficit hyperkinetic disorder (ADHD) even though there is hardly any structural CNS impairment in that condition. A presumed „lazy frontal lobe” is likely to react favorably to the stimulation provided by methylphenidate (a therapeutic effect which should not be viewed as paradoxical). Accepted: 27 June 1997     

Does the MONARC transobturator suburethral sling cause post-operative voiding dysfunction? A prospective study

The aim of this study was to compare pre-operative and post-operative voiding parameters following insertion of the MONARC transobturator tape (TOT), for treating women with urodynamic stress incontinence. This prospective observational study was conducted at a tertiary referral urogynaecology unit, in North Queensland, involving 83 women who were prospectively assessed pre-operatively and at 6–8 weeks following the procedure. Information collected included patient demographics, concomitant surgery, pre-operative and post-operative symptomatology (using validated questionnaires), and pre-operative and post-operative urodynamic parameters. Parameters used to assess voiding function included symptoms of voiding difficulty (incomplete emptying and irritative symptoms) as well as objective parameters including maximum flow rate (Q_max), adjusted maximum flow rate (Q_maxadj) using the Liverpool nomogram (LN), maximal urethral pressure, and post-void residual (PVR). Pre-operative average Q_max was 23.7 ml/s compared to 21.1 ml/s post-operatively (p=0.064). When the Q_max was adjusted for voided volume using the LN, Q_maxadj was seen to decrease significantly from 26 ml/s to 18 ml/s (p<0.05). Women with PVR>50 ml did not differ significantly pre-operatively and post-operatively, 5/83(6%) vs 7/83 (8.4%) (p=0.75). The number of women with a flow rate <10th centile on LN was 22 (26.5%) pre-operatively vs 29 (34.9%) post-operatively (p=0.21). One (1.2%) post-operatively had voiding dysfunction diagnosed by an abnormal voiding pattern (p=0.728), which was not statistically significant. Objective voiding dysfunction as determined by adjusted flow rates <10th centile LN and >50 ml PVR was seen in four women (4.8%). Adjusted free flow rates are significantly reduced following insertion of the MONARC TOT, as are some symptoms related to voiding dysfunction. Despite this, satisfaction rates remain high with observed voiding dysfunction or objective measures of voiding dysfunction showing no statistical change in the short term. Long-term follow-up is planned at 1 year.     

Galactosylceramide-beta-galactosidase deficiency in association with cherry red spot

A 13-month-old white girl was the product of a normal pregnancy and delivered by caesarean section for breech presentation. Regression of motor milestones started by 11 months, when delayed language development was also noted. She was normocephalic without major dysmorphic features or organomegaly. Fundus examination disclosed a subtle cherry red spot bilaterally. No startle response was elicited. By 17 months she was extremely irritable and unable to tolerate liquids; there was symmetrical spasticity and florid cherry red spots. She died at 18 months of age. A systematic search for conditions associated with a cherry red spot was unrevealing. The absence of galactosylceramide galactosidase activity was unexpected and was confirmed on three occasions in two laboratories. Lactosylceramide I content, an enzyme thought to be identical to galactosylceramide-beta-galactosidase, was significantly decreased. The presence of a cherry red spot in Krabbe's disease, indicative of neuronal storage, has not been previously recognized. The existence of this variant has implications for genetic and biochemical studies.