Utilization of sunglasses: A pilot study to examine a simple test for cognitive dysfunction.

OBJECTIVE: Utilization is an executive function disorder and implies an inappropriate response to external stimuli. The sunglasses test is a simple test to examine utilization by offering a pair of sunglasses to a patient without any instruction. PATIENTS AND METHODS: We performed a pilot study to examine the sunglasses test in 100 patients, of which 50 had cognitive symptoms and 50 did not have cognitive symptoms. When the patient put on the sunglasses, the test was considered positive. Cognitive dysfunction was based on the results of the MMSE, results of the clock test and neuropsychological examination (blinded). The final diagnosis was based on all results of the work-up (including MRI). RESULTS: Of the 50 patients with cognitive symptoms 30 had a positive test and 28 of them had cognitive dysfunction. Final diagnosis in most patients was Dementia due to probable Alzheimers disease, followed by Frontotemporal dementia. Seven of the 20 patients with a negative test also had cognitive dysfunction. None of the 50 patients without cognitive symptoms put on the sunglasses. Sensitivity of the sunglasses test to detect cognitive dysfunction was 80% and specificity was 97%, with a positive predictive value of 93%. CONCLUSION: Offering sunglasses is a simple test to screen for utilization. Putting on sunglasses without instruction to do so can be indicative for cognitive dysfunction and further cognitive evaluation should be considered. Future studies are needed in a larger group of patients and to determine the role of this test in different diseases with cognitive decline.

[Bell's palsy: different doctor, different care?] / Bellse parese: andere dokter, andere zorg?

OBJECTIVE: To compare the diagnostic work-up and treatment strategy of Bell's palsy by: general practitioners (GPs); ear, nose and throat (ENT) specialists; and neurologists in the Netherlands. DESIGN: Cross-sectional survey. METHOD: GPs, ENT specialists and neurologists were asked to participate in an online survey on the diagnosis and treatment of Bell's palsy. Results per specialty were analysed using descriptive statistics and chi-squared test. RESULTS: A total of 415 clinicians participated, including 149 GPs, 123 ENT specialists, and 143 neurologists. The answers from the three disciplines showed significant differences concerning history taking, physical examination, alarm symptoms, treatment strategy and follow-up. ENT specialists more frequently asked about hearing loss (90%), whereas neurologists enquired about weakness in arms and legs (62%). GPs less often ask about tick bites than clinicians in ENT and neurology, respectively (36% vs. 89% and 71%). Neurologists rarely performed otoscopy (21%), but more frequently tested eye movements (78%) than GPs (33%) and ENT specialists (20%). Compared to GPs and neurologist, ENT specialists more frequently reported the supplementation of Prednisone treatment with antiviral agents (45% vs. 9% and 6 % resp.). Furthermore, the time interval to the follow-up visit varied strongly between clinicians, ranging between a few days to six weeks after the first clinic visit. CONCLUSION: This study shows that the diagnostic work-up and treatment strategy for Bell's palsy in the Netherlands is highly dependent on the clinician's specialty. Despite the fact that equivalent guidelines are in place, GPs, ENT specialists and neurologists gave different answers to questions concerning diagnostics and treatment. This finding suggests that more attention should be paid to the implementation of and adherence to the multidisciplinary guideline for Bell's palsy.

Olfactory dysfunction in behavioral variant frontotemporal dementia.

OBJECTIVE: Several neurodegenerative disorders show olfactory dysfunction. In patients with frontotemporal dementia (FTD), olfactory impairment is probably due to the involvement of the temporal and orbitofrontal lobes. We hypothesized that due to the disrupted areas in FTD, there would be an impairment in smell identification, differentiation and association. Moreover, we hypothesized that there would be a correlation between the severity of FTD and the severity of odor dysfunction. METHODS: In the current study, we compared odor identification, discrimination and association of nine patients with behavioral variant FTD with eleven healthy controls using the Brief Smell Identification Test and the Odor Perception and Semantics Battery. RESULTS: The results showed significant differences in the odor association test, but not in the identification or discrimination test. There was no correlation between disease severity and the performance in the odor tests. CONCLUSION: We showed impairment of odor association that is most likely due to disruption of specific associative areas involved in olfactory processing. Specifically, we propose that the impairment may well be due to disrupted areas in the temporal lobe and amygdala.

EFNS guideline on the treatment of cerebral venous and sinus thrombosis in adult patients.

BACKGROUND: Cerebral venous and sinus thrombosis (CVST) is a rather rare disease which accounts for <1% of all strokes. Diagnosis is still frequently overlooked or delayed as a result of the wide spectrum of clinical symptoms and the often subacute or lingering onset. Current therapeutic measures which are used in clinical practice include the use of anticoagulants such as dose-adjusted intravenous heparin or body weight-adjusted subcutaneous low-molecular-weight heparin (LMWH), the use of thrombolysis and symptomatic therapy including control of seizures and elevated intracranial pressure. METHODS: We searched MEDLINE (National Library of Medicine), the Cochrane Central Register of Controlled Trials (CENTRAL) and the Cochrane Library to review the strength of evidence to support these interventions and the preparation of recommendations on the therapy of CVST based on the best available evidence. Review articles and book chapters were also included. Recommendations were reached by consensus. Where there was a lack of evidence but consensus was clear we stated our opinion as good practice points. RESULTS AND CONCLUSIONS: Patients with CVST without contraindications for anticoagulation (AC) should be treated either with body weight-adjusted subcutaneous LMWH or with dose-adjusted intravenous heparin (level B recommendation). Concomitant intracranial haemorrhage (ICH) related to CVST is not a contraindication for heparin therapy. The optimal duration of oral anticoagulant therapy after the acute phase is unclear. Oral AC may be given for 3 months if CVST was secondary to a transient risk factor, for 6-12 months in patients with idiopathic CVST and in those with "mild" thrombophilia, such as heterozygous factor V Leiden or prothrombin G20210A mutation and high plasma levels of factor VIII. Indefinite AC should be considered in patients with recurrent episodes of CVST and in those with one episode of CVST and 'severe' thrombophilia, such as antithrombin, protein C or protein S deficiency, homozygous factor V Leiden or prothrombin G20210A mutation, antiphospholipid antibodies and combined abnormalities (good practice point). There is insufficient evidence to support the use of either systemic or local thrombolysis in patients with CVST. If patients deteriorate despite adequate AC and other causes of deterioration have been ruled out, thrombolysis may be a therapeutic option in selected cases, possibly in those without large ICH and threatening herniation (good practice point). There are no controlled data about the risks and benefits of certain therapeutic measures to reduce an elevated intracranial pressure (with brain displacement) in patients with severe CVST. However, in severe cases with impending herniation craniectomy can be used as a life-saving intervention (good practice point).

Neck pain in chronic whiplash syndrome treated with botulinum toxin. A double-blind, placebo-controlled clinical trial.

OBJECTIVES: Neck pain in chronic whiplash syndrome is a major burden for patients, healthcare providers and insurance companies. Randomized data on treatment of botulinum toxin in chronic whiplash syndrome are scarce. We conducted a randomized, placebo-controlled clinical trial to prove efficacy of botulinum toxin for neck pain in chronic whiplash syndrome. METHODS: 40 patients with chronic whiplash syndrome (whiplash associated disorders grade 1 and 2) were randomly assigned to receive botulinum toxin (maximum 100 units) or placebo (saline) in muscles with increased tenderness. RESULTS: After 12 weeks there was no significant difference between the two treatment groups in decrease of neck pain intensity on VAS (-7.0 mm, 95% confidence interval (CI) [-20.7 to +6.7]), mean number of neck pain days (-1%; 95% CI [-15% to +13%]), neck pain hours per day (-0.14; 95% CI [-3.0 to +2.7]), days on which symptomatic treatment was taken (-0.7%; 95% CI [-15% to +13%]) number of analgesics taken per day (-0.14; 95% CI [-0.6 to +0.4]) and total cervical range of motion (-11 degrees; 95% CI [-40 to +17]). There also was no significant difference in patient's assessment of improvement after week 4, 8 and 12. CONCLUSIONS: Botulinum toxin was not proven effective in treatment of neck pain in chronic whiplash syndrome. Increased muscle tenderness alone might not be the major cause of neck pain in whiplash syndrome.

EFNS guideline on the treatment of cerebral venous and sinus thrombosis.

Cerebral venous and sinus thrombosis (CVST) is a rather rare disease which accounts for <1% of all strokes. Diagnosis is still frequently overlooked or delayed due to the wide spectrum of clinical symptoms and the often subacute or lingering onset. Current therapeutic measures which are used in clinical practice include the use of anticoagulants such as dose-adjusted intravenous heparin or body weight-adjusted subcutaneous low-molecular-weight heparin (LMWH), the use of thrombolysis, and symptomatic therapy including control of seizures and elevated intracranial pressure. We searched MEDLINE (National Library of Medicine), the Cochrane Central Register of Controlled Trials (CENTRAL) and the Cochrane Library to review the strength of evidence to support these interventions and the preparation of recommendations on the therapy of CVST based on the best available evidence. Review articles and book chapters were also included. Recommendations were reached by consensus. Where there was a lack of evidence, but consensus was clear we stated our opinion as good practice points. Patients with CVST without contraindications for anticoagulation should be treated either with body weight-adjusted subcutaneous LMWH or dose-adjusted intravenous heparin (good practice point). Concomitant intracranial haemorrhage related to CVST is not a contraindication for heparin therapy. The optimal duration of oral anticoagulation after the acute phase is unclear. Oral anticoagulation may be given for 3 months if CVST was secondary to a transient risk factor, for 6-12 months in patients with idiopathic CVST and in those with 'mild' hereditary thrombophilia. Indefinite anticoagulation (AC) should be considered in patients with two or more episodes of CVST and in those with one episode of CVST and 'severe' hereditary thrombophilia (good practice point). There is insufficient evidence to support the use of either systemic or local thrombolysis in patients with CVST. If patients deteriorate despite adequate anticoagulation and other causes of deterioration have been ruled out, thrombolysis may be a therapeutic option in selected cases, possibly in those without intracranial haemorrhage (good practice point). There are no controlled data about the risks and benefits of certain therapeutic measures to reduce an elevated intracranial pressure (with brain displacement) in patients with severe CVST. Antioedema treatment (including hyperventilation, osmotic diuretics and craniectomy) should be used as life saving interventions (good practice point).

['Migraine and a hole in the heart': causal relationship between migraine and patent foramen ovale not established]. / 'Migraine en een gaatje in het hart': oorzakelijk verband tussen migraine en foramen ovale persistens niet aangetoond.

The relationship between patent foramen ovale (PFO) and migraine is controversial. A common genetic background seems possible, but there is no evidence for a causal relationship. Despite the lack of evidence, PFO closure is recommended in many countries as a remedy for migraine. At present, PFO closure for migraine should only be carried out under strictly controlled clinical trial conditions.

[A meningioma resulting in drastic changes in behavior and life course]. / Een meningeoom als oorzaak van ingrijpende veranderingen in gedrag en levensloop.

Three patients, a 59-year-old woman, a 51-year-old man and a 66-year-old woman presented at a neurological or neurosurgical outpatient clinic after their lives had been drastically affected by behavioural changes. Neither the patients nor their relatives or friends were aware that the behavioural changes may be caused by a medical problem. The cause, a large meningioma, became obvious only after physical symptoms occurred a few years later. The patients recovered after surgery and regretted having made certain decisions with far-reaching repercussions for their lives and families. Behavioural disturbances are seldom due to slowly growing brain tumours, but consultation with a neurologist is recommended if there is any doubt regarding the possibility of somatic causes.

[Brain injury in boxers and soccer players; an advisory report from the National Health Council of the Netherlands]. / Hersenletsel bij boksers en voetballers; een adviesrapport van de Gezondheidsraad.

The National Health Council of the Netherlands has released a report entitled 'Brain damage in boxers and soccer players'. It examines epidemiology and prevention of cerebral damage from professional and amateur sports, focusing particularly on soccer and boxing. The connection between cerebral injuries and acute and chronic sports injuries is well established. However, proper diagnosis and therapy may be difficult in acute head trauma and during the chronic stage. It makes proposals for a simple cognitive screening test for acute injuries (soccer-related in particular) and more extensive testing for the detection and follow-up of chronic cerebral injuries (boxing-related in particular). Neuropsychological testing may be an important additional diagnostic tool. Professional boxing should be banned if the measures suggested fail to prevent chronic cognitive dysfunction. A dedicated knowledge centre for soccer and boxing would enhance the epidemiology and knowledge of brain injuries, and could help to develop more evidence-based guidelines in the future.

Treatment of chronic tension-type headache with botulinum toxin: a double-blind, placebo-controlled clinical trial.

Botulinum toxin is increasingly advocated as effective treatment in chronic tension-type headache. We conducted a randomized, placebo-controlled clinical trial to prove efficacy of botulinum toxin in chronic tension-type headache. Patients were randomly assigned to receive botulinum toxin (maximum 100 units) or placebo (saline) in muscles with increased tenderness. After 12 weeks there was no significant difference between the two treatment groups in decrease of headache intensity on VAS (-3.5 mm, 95% confidence interval (CI) - 20 to +13), mean number of headache days (-7%; 95% CI - 20 to +4), headache hours per day (-1.4%; 95% CI - 3.9 to +1.1), days on which symptomatic treatment was taken (-1.9%; 95% CI - 11 to +7) and number of analgesics taken per day (-0.01; 95% CI -0.25-0.22). There was no significant difference in patient's assessment of improvement after week 4, 8 and 12. Botulinum toxin was not proven effective in treatment of chronic tension-type headache. Increased muscle tenderness might not be as important in pathophysiology of chronic tension-type headache as hitherto believed.

Anticoagulation for cerebral sinus thrombosis.

BACKGROUND: Treatment of cerebral sinus thrombosis with anticoagulants has been controversial. Anticoagulants may prevent new venous infarcts, neurologic deterioration, and pulmonary embolism but may also promote haemorrhages. OBJECTIVES: To review the available evidence regarding the effectiveness and safety of anticoagulant therapy in patients with confirmed cerebral sinus thrombosis. SEARCH STRATEGY: We searched the Cochrane Stroke Group Trials Register (last searched 18 March 2002). We also searched MEDLINE (1966-Oct 2001), EMBASE (1980-Feb 2002) and the Cochrane Controlled Trials Register (Cochrane Library, 2002 Issue 1) and contacted authors to identify additional published and unpublished studies. SELECTION CRITERIA: Unconfounded randomised controlled trials in which anticoagulant therapy was compared with placebo or open control in patients with cerebral sinus thrombosis (confirmed by intra-arterial contrast or magnetic resonance angiography). DATA COLLECTION AND ANALYSIS: Two reviewers independently extracted outcomes for each of the two treatment groups (anticoagulant treatment and control). The outcome data for each patient are analysed in the treatment group to which the patient was originally allocated ('intention to treat' analysis). A weighted estimate of the treatment effects across trials (relative risk, absolute risk reduction) is calculated using the Cochrane statistical software. MAIN RESULTS: Two small trials involving 79 patients fulfilled the inclusion criteria. One trial (20 patients) examined the efficacy of intravenous, adjusted dose unfractionated heparin. The other trial (59 patients) examined high dose, body weight adjusted, subcutaneous, low-molecular weight heparin (Nadroparin). Anticoagulant therapy was associated with a pooled relative risk of death of 0.33 (95 % CI 0.08 to 1.21) and of death or dependency of 0.46 (95 % CI 0.16 to 1.31). No new symptomatic intracerebral haemorrhages were observed. One major gastro-intestinal haemorrhage occurred after anticoagulant treatment. Two control patients (placebo) had a diagnosis of probable pulmonary embolism (one fatal). REVIEWER'S CONCLUSIONS: Based upon the limited evidence available, anticoagulant treatment for cerebral sinus thrombosis appeared to be safe and was associated with a potentially important reduction in the risk of death or dependency which did not reach statistical significance.

Clinical features and prognostic factors of cerebral venous sinus thrombosis in a prospective series of 59 patients. For The Cerebral Venous Sinus Thrombosis Study Group.

The prognosis of cerebral venous sinus thrombosis (CVST) is variable, and outcome may range from complete recovery to death. Prognostic factors to predict outcome in the acute phase of CVST have not been analysed in a prospective study. Prognostic factors in patients enrolled in a clinical treatment trial were prospectively investigated. Poor outcome after 12 weeks, defined as death or dependency (Oxford handicap score > or =3), was used as the principle outcome measure. Univariate relations between possible prognostic factors and outcome at 12 weeks were analyzed with chi(2) tests. Treatment and all factors associated with prognosis (p< or = 0.25) were forced into a logistic regression model with a forward selection procedure. Fifty nine patients (50 women, nine men) were studied, with a mean age of 37 years (range 18 to 80 years). After 12 weeks 10 patients (17%) had a poor outcome. The univariate identified factors related to poor outcome were papilloedema, altered consciousness, coma, age older than 33 years, diagnostic delay < or =10 days, intracerebral haemorrhage, and involvement of the straight sinus. Isolated intracranial hypertension and a delta sign on CT were associated with good outcome. In the multivariate analysis coma and cerebral haemorrhage were significantly associated with a poor outcome, with odds ratios of 8.2 (95% confidence interval (95% CI) 1. 3-50.1) and 20.7 (95% CI 1.6-264.3) respectively. Involvement of the straight sinus was also weakly, but not significantly, associated with poor outcome. In conclusion, coma and intracerebral haemorrhage are independent predictors for poor outcome of CVST.

Long-term outcome of cognition and functional health after cerebral venous sinus thrombosis.

The authors examined cognition and functional health of 57 patients with sinus thrombosis 1 year or more after enrollment in a randomized trial of anticoagulant treatment or placebo. Eight patients died and two declined participation. Of the remaining 47 patients, 16 (35%) had cognitive impairments, 3 (6%) were dependent, 19 (40%) had symptoms that led to restrictions in lifestyle, and 19 (40%) could not resume their previous level of economic activity. Outcome after sinus thrombosis may be less favorable than reported previously, and was not significantly influenced by treatment.