There is a clear need for brief, but sensitive and specific, cognitive screening instruments as evidenced by the popularity of the Addenbrooke's Cognitive Examination (ACE).

Objectives
We aimed to validate an improved revision (the ACE-R) which incorporates five sub-domain scores (orientation/attention, memory, verbal fluency, language and visuo-spatial).

Methods
Standard tests for evaluating dementia screening tests were applied. A total of 241 subjects participated in this study (Alzheimer's disease = 67, frontotemporal dementia = 55, dementia of Lewy Bodies = 20; mild cognitive impairment–MCI = 36; controls = 63).

Results
Reliability of the ACE-R was very good (alpha coefficient = 0.8). Correlation with the Clinical Dementia Scale was significant (r = −0.321, p < 0.001). Two cut-offs were defined (88: sensitivity = 0.94, specificity = 0.89; 82: sensitivity = 0.84, specificity = 1.0). Likelihood ratios of dementia were generated for scores between 88 and 82: at a cut-off of 82 the likelihood of dementia is 100:1. A comparison of individual age and education matched groups of MCI, AD and controls placed the MCI group performance between controls and AD and revealed MCI patients to be impaired in areas other than memory (attention/orientation, verbal fluency and language).

Conclusions
The ACE-R accomplishes standards of a valid dementia screening test, sensitive to early cognitive dysfunction. Copyright © 2006 John Wiley & Sons, Ltd.

The Addenbrooke's Cognitive Examination Revised (ACE-R): a brief cognitive test battery for dementia screening

Background The differential diagnosis of disorders of consciousness is challenging. The rate of misdiagnosis is approximately 40%, and new methods are required to complement bedside testing, particularly if the patient’s capacity to show behavioral signs of awareness is diminished. Methods At two major referral centers in Cambridge, United Kingdom, and Liege, Belgium, we performed a study involving 54 patients with disorders of consciousness. We used functional magnetic resonance imaging (MRI) to assess each patient’s ability to generate willful, neuroanatomically specific, blood-oxygenation-level–dependent responses during two established mental-imagery tasks. A technique was then developed to determine whether such tasks could be used to communicate yes-or-no answers to simple questions. Results Of the 54 patients enrolled in the study, 5 were able to willfully modulate their brain activity. In three of these patients, additional bedside testing revealed some sign of awareness, but in the other two patients, no voluntary behavior could be detected by means of clinical assessment. One patient was able to use our technique to answer yes or no to questions during functional MRI; however, it remained impossible to establish any form of communication at the bedside. Conclusions These results show that a small proportion of patients in a vegetative or minimally conscious state have brain activation reflecting some awareness and cognition. Careful clinical examination will result in reclassification of the state of consciousness in some of these patients. This technique may be useful in establishing basic communication with patients who appear to be unresponsive.

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Willful Modulation of Brain Activity in Disorders of Consciousness

Background: Previously published studies have reported that up to 43% of patients with disorders of consciousness are erroneously assigned a diagnosis of vegetative state (VS). However, no recent studies have investigated the accuracy of this grave clinical diagnosis. In this study, we compared consensus-based diagnoses of VS and MCS to those based on a well-established standardized neurobehavioral rating scale, the JFK Coma Recovery Scale-Revised (CRS-R). Methods: We prospectively followed 103 patients (55 ± 19 years) with mixed etiologies and compared the clinical consensus diagnosis provided by the physician on the basis of the medical staff's daily observations to diagnoses derived from CRS-R assessments performed by research staff. All patients were assigned a diagnosis of 'VS', 'MCS' or 'uncertain diagnosis.' Results: Of the 44 patients diagnosed with VS based on the clinical consensus of the medical team, 18 (41%) were found to be in MCS following standardized assessment with the CRS-R. In the 41 patients with a consensus diagnosis of MCS, 4 (10%) had emerged from MCS, according to the CRSR. We also found that the majority of patients assigned an uncertain diagnosis by clinical consensus (89%) were in MCS based on CRS-R findings. Conclusion: Despite the importance of diagnostic accuracy, the rate of misdiagnosis of VS has not substantially changed in the past 15 years. Standardized neurobehavioral assessment is a more sensitive means of establishing differential diagnosis in patients with disorders of consciousness when compared to diagnoses determined by clinical consensus.

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Diagnostic accuracy of the vegetative and minimally conscious state: Clinical consensus versus standardized neurobehavioral assessment

Several brain regions and physiological processes have been proposed to constitute the neural correlates of consciousness. In this Review, Koch and colleagues discuss studies that distinguish the neural correlates of consciousness from other neural processes that precede, accompany or follow it, and suggest that the neural correlates of consciousness are localized to posterior cortical regions.

Neural correlates of consciousness: progress and problems

Some patients awaken from coma (that is, open the eyes) but remain unresponsive (that is, only showing reflex movements without response to command). This syndrome has been coined vegetative state. We here present a new name for this challenging neurological condition: unresponsive wakefulness syndrome (abbreviated UWS). Many clinicians feel uncomfortable when referring to patients as vegetative. Indeed, to most of the lay public and media vegetative state has a pejorative connotation and seems inappropriately to refer to these patients as being vegetable-like. Some political and religious groups have hence felt the need to emphasize these vulnerable patients' rights as human beings. Moreover, since its first description over 35 years ago, an increasing number of functional neuroimaging and cognitive evoked potential studies have shown that physicians should be cautious to make strong claims about awareness in some patients without behavioral responses to command. Given these concerns regarding the negative associations intrinsic to the term vegetative state as well as the diagnostic errors and their potential effect on the treatment and care for these patients (who sometimes never recover behavioral signs of consciousness but often recover to what was recently coined a minimally conscious state) we here propose to replace the name. Since after 35 years the medical community has been unsuccessful in changing the pejorative image associated with the words vegetative state, we think it would be better to change the term itself. We here offer physicians the possibility to refer to this condition as unresponsive wakefulness syndrome or UWS. As this neutral descriptive term indicates, it refers to patients showing a number of clinical signs (hence syndrome) of unresponsiveness (that is, without response to commands) in the presence of wakefulness (that is, eye opening).

/pdf/unresponsive-wakefulness-syndrome-a-new-name-for-the-3nkot6nyo4.pdf

Unresponsive wakefulness syndrome: a new name for the vegetative state or apallic syndrome

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...

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

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>or=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 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.

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

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.

https://jnnp.bmj.com/content/jnnp/58/6/735.full.pdf

Alien hand syndrome without a corpus callosum lesion.

Prospective placebo-controlled. 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. Centre de Traumatologie et de Readaptation, Brussels, Belgium. 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. 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. 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.

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Manfredi Ventura

Papers

Diagnostic accuracy of the vegetative and minimally conscious state: Clinical consensus versus standardized neurobehavioral assessment

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

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

Alien hand syndrome without a corpus callosum lesion.

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