Manfredi Ventura

A French validation study of the Coma Recovery Scale-Revised (CRS-R)

Primary objective: The aim of the present study was to explore the concurrent validity, inter-rater agreement and diagnostic sensitivity of a French adaptation of the Coma Recovery Scale–Revised (CRS-R) as compared to other coma scales such as the Glasgow Coma Scale (GCS), the Full Outline of UnResponsiveness scale (FOUR) and the Wessex Head Injury Matrix (WHIM). Research design: Multi-centric prospective study. Method and procedures: To test concurrent validity and diagnostic sensitivity, the four behavioural scales were administered in a randomized order in 77 vegetative and minimally conscious patients. Twenty-four clinicians with different professional backgrounds, levels of expertise and CRS-R experience were recruited to assess inter-rater agreement. Main outcomes and results: Good concurrent validity was obtained between the CRS-R and the three other standardized behavioural scales. Inter-rater reliability for the CRS-R total score and sub-scores was good, indicating that the scale yields reproducible findings across examiners and does not appear to be systematically biased by profession, level of expertise or CRS-R experience. Finally, the CRS-R demonstrated a significantly higher sensitivity to detect MCS patients, as compared to the GCS, the FOUR and the WHIM. Conclusion: The results show that the French version of the CRS-R is a valid and sensitive scale which can be used in severely brain damaged patients by all members of the medical staff.

Is the Addenbrooke's cognitive examination effective to detect frontotemporal dementia?

Abstract.We evaluated the Addenbrooke’s cognitive examination (ACE), a simple instrument to differentiate frontotemporal dementia (FTD) from Alzheimer’s disease (AD), in our dementia patients clinic population. The Verbal-Language/Orientation-Memory (VLOM) ratio, which compares its language and memory scores, determines whether FTD or AD is more likely. The ACE was translated into French with adaptation maintaining the number of words in the name and address learning and delayed recall test, and with cultural adaptation for the semantic memory. The 85 included subjects had no evidence of two or more organic pathologies, after at least six months of follow-up, and an MMSE score ≥ 20/30. Patients with cognitive impairment due to alcohol intake were excluded. The diagnosis of a specific dementing illness was based on the consensus of the neurologist and neuropsychologists in the team. Thereafter, another neurologist expert in dementia, blinded to the ACE result and to the diagnosis and treatment, reviewed all cases files and proposed a diagnosis. A diagnostic agreement was reached for 79 cases (92.9%) with 40 (50.6%) dementia: 25 AD (62.5 %), 9 FTD (22.5 %).We estimated that the sensitivity for detecting dementia of an ACE score ≤ 83/100 was 90% with a specificity of 64.1%. When the ACE score was ≤ 88/100, the sensitivity for diagnosing FTD of a VLOM ratio < 2.2 was 11.1% with a specificity of 88 % and the sensitivity for diagnosing AD of a VLOM ratio > 3.2 was 72%,with a specificity of 69.4%. We conclude that, when used as originally proposed, ACE is very accurate for the detection of dementia, but much less effective in discriminating the most common frontal variant of FTD.

Alien hand syndrome without a corpus callosum lesion.

A 58 year old right handed woman developed a left alien hand syndrome after a right capsulothalamic haemorrhage with mesencephalic extension. No lesion was detected in the corpus callosum. Positron emission tomography suggested that right cortical dysfunction might account for the absence of recognition of the left hand whereas involuntary motor activity was attributed to the lesion itself. This case shows that the alien hand syndrome is not always the expression of a disconnection syndrome and may occur after a large, strategically located, posterior lesion.

Neuromuscular electrical stimulation of completely paralyzed abdominal muscles in spinal cord-injured patients : a pilot study

Study Design:Prospective placebo-controlled.Objective:The effect of abdominal neuromuscular electrical stimulation (NMES) in patients with spinal cord injury. The principal parameters observed in this study are lung capacity, colonic transit, patient satisfaction of used method and of aesthetics effect on abdominal wall.Settings:Centre de Traumatologie et de Réadaptation, Brussels, Belgium.Methods:A total of 10 volunteers participated in this study and were assigned to two groups—the effective electrical stimulation group (ESG) and the placebo-controlled group (PG). NMES of abdominal muscles was performed 25 min per day for 8 weeks.Results:NMES significantly decreased forced vital capacity (FVC) in ESG but not in PG. In ESG, colonic transit was accelerated in ascending, transverse and descending colon but transit in rectosigmoideum was not affected. In PG, no variations in colonic transit were observed. Satisfaction scale shows a better influence on aesthetics effect in ESG than in PG.Conclusion:This pilot study shows that NMES of paralyzed abdominal muscles positively affects colonic transit except in rectosigmoideum segment and negatively affects FVC. It could be a simple self-used method to regulate colonic transfer with considerably good cosmetic effect on abdominal wall. However, regular verification of FVC will probably be necessary.

COGNOS: care for people with cognitive dysfunction: a national observational study

Care plans are intended to improve the independence and functioning of patients with cognitive dysfunction and support the caregivers involved. They are an integral part of the Belgian reimbursement procedure for cholinesterase inhibitors. This nationwide, multicenter, observational study examined the content and implementation of the care plan along with patient satisfaction in community-dwelling patients newly diagnosed with Alzheimer disease in Belgium. The patients’ opinion of their quality of life was measured using Anamnestic Comparative Self-Assessment (ACSA) scale. A total of 720 participants (453 female) were enrolled with 86.0% (619/719) living at home alone or with their spouse/partner. Cognitive problems (627/717, 87.4%) were the main reason for initiation of the consultation. Most patients had a caregiver (646/719, 89.8%): generally the spouse/partner (351/646, 54.3%) or a child (232/646, 35.9%). A total of 511 patients (71.0%) were prescribed a cholinesterase inhibitor after the initial consultation. A total of 236 care plans were advised with 169 (71.6%) realized and 157 of these (92.9%) considered adequate. Most patients were satisfied with the help received in the care plan (service satisfaction range, 80.0% to 98.6% of patients). Quality of life as rated by the patient significantly increased between baseline (average ACSA score: 5.2±2.4) and follow-up (5.8±2.1). The use of care plans appears to improve management of care for Alzheimer disease patients.

Striatal lesions: an underestimated complication of cerebral malaria?

Sirs, Cerebral malaria (CM) is characterized by altered consciousness, acute confusional state, seizures, anaemia, hypoglycaemia, brainstem involvement and retinal haemorrhage with confirmation of Plasmodium parasitaemia, when other causes of encephalopathy have been excluded. (Nguyen et al., 1996; Newton andWarrell, 1998) Brain imaging in CM typically shows an increase in brain volumewith or without cerebral oedema whereas focal cerebral lesions are usually not present (Newtonand Warrell, 1998). We here report the first description of CM complicated by bilateral striatal lesions and propose a possible physiopathological mechanism. A37-year-old right-handedBelgianman presented with fever (40 C), diarrhoea, hypotension (71/43 mmHg) and anuric renal failure 5 days after his return from Kenya where he had not taken any antimalarial prophylactic therapy. His medical history was unremarkable. Peripheral blood smear was positive for Plasmodium falciparum (40% parasitaemia). Plasma lactate was >8 mEq/l with a pH of 7, 18. Treatment by quinine 1.5 g/day was initiated. He then presented generalized tonic-clonic seizures. Brain magnetic resonance imaging showed bilateral lesions affecting globus pallidus, subthalamic nucleus and substantia nigra (Fig. 1). When he was admitted in our rehabilitation unit 2 months later, he suffered from tetraparesis and parkinsonism. Neurological examination revealed saccadic pursuit and several extrapyramidal signs including bilateral upper limbs rigidity, postural tremor and bradykinesia, a tetraparesis predominantly affecting the lower limbs with bilateral lower limb hyporeflexia. There were no pyramidal or cerebellar signs. Electrophysiological investigations disclosed an ongoing axonal sensorimotor polyneuropathy suggesting intensive care unit polyneuropathy. Striatal lesions have never been described as a complication of CM. The identified causes of bilateral striatal necrosis are severe metabolic acidosis, methanol intoxication, carbon monoxide intoxication, hypoxic encephalopathy, cyanide poisoning, venous infarction, osmotic demyelination, Wilson’s disease, Leigh’s syndrome, mitochondrial encephalopathy, haemolytic-uraemic syndrome, sickle-cell disease acidosis, metformin-induced lactic acidosis,Mycoplasma pneumoniae infection, Arthrinium-produced mycotoxin, glutaric acidemia and rare cases of familial striatal necrosis (Goodman and Norenberg, 1983; He et al., 1995; Kamei et al., 1996; Mewborne et al., 1998; Faris et al., 2000; Zambrino et al., 2000). In most of these cases, the implicated pathogenic mechanism is thought to be a preferential sensibility of striatal neurons to lactic acidosis induced by disturbance of the mitochondrial metabolism (Kamei et al., 1996). Compared with these cases, our patient presented bilateral lesions of the striatum and parkinsonism almost disappeared after 1 year of follow-up. Interestingly, there are only few descriptions of extrapyramidal syndrome associated with CM (Arya et al., 1989; Chaudhary et al., 1992) or following chloroquine therapy (Singhiet al., 1979). In these cases, neither focal brain lesion were reported nor physiopathological mechanism proposed (Singhi et al., 1979; Arya et al., 1989; Chaudhary et al., 1992). However, mild parkinsonism because of striatal lesions may have been underestimated in developing countries where CM is more frequent. In our case the co-occurrence of CM, lactic acidosis and systemic hypotensionmay explain the severity of the clinicalradiological picture (Kamei et al., 1996). In conclusion, clinicians should be aware that CMmay cause striatal lesions and should systematically search for extrapyramidal signs in patients with CM and lactic acidosis, especially when systemic hypoxia or hypotension are present.

Clinical utility and applicability of biomarker-based diagnostic criteria for Alzheimer’s disease: a BeDeCo survey

We conducted a survey regarding the medical care of patients with dementia in expert settings in Belgium. Open, unrestricted and motivated answers were centralized, blindly interpreted and structured into categories. The report of the results was then submitted to the participants in subsequent plenary meetings and through email. Fourteen experts responded to the questionnaire, confirming that recent propositions to modify Alzheimer’s disease (AD) diagnostic criteria and options have stirred up debate among well-informed and dedicated experts in the field. The opinions were not unanimous and illustrate how difficult it is to find a standardized method of diagnosing this disease. The responses to the survey suggest that application of a step-by-step pragmatic method is used in practice. Only when the combination of clinical findings and classical structural neuro-imaging is insufficient for a diagnosis or suggests an atypical presentation, additional biomarkers are considered. Interestingly, few differences, if any, were observed between the use of biomarkers in MCI and in AD. In conclusion, the Belgian experts consulted in this survey were generally in agreement with the new diagnostic criteria for AD, although some concern was expressed about them being too “amyloidocentric”. Although the clinical examination, including a full neuropsychological evaluation, is still considered as the basis for diagnosis, most experts also stated that they use biomarkers to help with diagnosis.

Alzheimer's disease and driving: review of the literature and consensus guideline from Belgian dementia experts and the Belgian road safety institute endorsed by the Belgian Medical Association.

Alzheimer’s disease (AD) is a highly prevalent condition and its prevalence is expected to further increase due to the aging of the general population. It is obvious that the diagnosis of AD has implications for driving. Finally, driving discussions are also emotionally charged because driving is associated with independence and personal identity. However, it is not clear how to implement this in clinical practice and the Belgian law on driving is rather vague in its referral to neurodegenerative brain diseases in general nor does it provide clear-cut instructions for dementia or AD compared to for example driving for patients with epilepsy and as such does not prove to be very helpful. The present article reviews what is known from both literature and existing guidelines and proposes a consensus recommendation tailored to the Belgian situation agreed by both AD experts and the Belgian Road Safety Institute endorsed by the Belgian Medical Association. It is concluded that the decision about driving fitness should be considered as a dynamic process where the driving fitness is assessed and discussed early after diagnosis and closely monitored by the treating physician. The diagnosis of AD on itself definitely does not imply the immediate and full revocation of a driving license nor does it implicate a necessary referral for a formal on-road driving assessment. There is no evidence to recommend a reduced exposure or a mandatory co-pilot. A MMSE-based framework to trichotomise AD patients as safe, indeterminate or unsafe is presented. The final decision on driving fitness can only be made after careful history taking and clinical examination, neuropsychological, functional and behavioral evaluation and, only for selected cases, a formal assessment of driving performance.

Acute Neurogenic Speechlessness

Acute loss of verbal expression after a neurological insult can prove to be challenging for the clinician. More than the neuropathological origin, the neurophysiological mechanisms which subtend the symptoms often constitute the major question to address. This is illustrated by the retrospective study of a patient we have seen in the acute phase of her illness, and have followed-up for 1½ year.