[The unresponsive wakefulness syndrome: Dutch perspectives]. / Als het bewustzijn niet meer terugkeert.

The unresponsive wakefulness syndrome (UWS), formerly known as the vegetative state, is one of the most dramatic outcomes of acquired brain injury. Patients with UWS open their eyes spontaneously but demonstrate only reflexive behavior; there are no signs of consciousness. Research shows that, for years now, the Netherlands has the world's lowest documented prevalence of UWS. Unfortunately, this small group of vulnerable patients does not receive the care it needs. Access to specialized rehabilitation is limited, misdiagnosis rates are high and a substantial number of UWS patients receive life-prolonging treatment beyond chances of recovery, despite a framework allowing for discontinuation of such treatment once recovery of consciousness has become unlikely. By comparing data from 2012 with that of 2003, this paper illustrates the current situation and outlook for UWS patients in the Netherlands and makes recommendations for the optimization of treatment and care, as well as for future research.

The vegetative state/unresponsive wakefulness syndrome: a systematic review of prevalence studies.

One of the worst outcomes of acquired brain injury is the vegetative state, recently renamed 'unresponsive wakefulness syndrome' (VS/UWS). A patient in VS/UWS shows reflexive behaviour such as spontaneous eye opening and breathing, but no signs of awareness of the self or the environment. We performed a systematic review of VS/UWS prevalence studies and assessed their reliability. Medline, Embase, the Cochrane Library, CINAHL and PsycINFO were searched in April 2013 for cross-sectional point or period prevalence studies explicitly stating the prevalence of VS/UWS due to acute causes within the general population. We additionally checked bibliographies and consulted experts in the field to obtain 'grey data' like government reports. Relevant publications underwent quality assessment and data-extraction. We retrieved 1032 papers out of which 14 met the inclusion criteria. Prevalence figures varied from 0.2 to 6.1 VS/UWS patients per 100 000 members of the population. However, the publications' methodological quality differed substantially, in particular with regards to inclusion criteria and diagnosis verification. The reliability of VS/UWS prevalence figures is poor. Methodological flaws in available prevalence studies, the fact that 5/14 of the studies predate the identification of the minimally conscious state (MCS) as a distinct entity in 2002, and insufficient verification of included cases may lead to both overestimation and underestimation of the actual number of patients in VS/UWS.

The prevalence and characteristics of patients with classic locked-in syndrome in Dutch nursing homes.

To establish the point prevalence and characteristics of patients with locked-in syndrome (LIS), particularly of the classic type, residing in Dutch nursing homes, a cross-sectional survey of Dutch nursing homes was conducted. The classic form of LIS was defined according to the criteria of the American Congress of Rehabilitation Medicine (1995). All Dutch long-term care organisations (n = 187) were asked if they had any patients with classic LIS as of December 5, 2011. The treating Elderly Care Physicians were then contacted to provide patient characteristics. Of all organisations, 91.4% responded, and 11 organisations reported a total of 12 patients. After analysing the questionnaires, it was determined that ten patients had LIS, and two patients were characterised with vegetative state. Only two patients met the criteria for classic LIS, while six patients showed partial LIS. One of these patients was admitted to the nursing home after December 5, 2011, and was therefore, excluded. LIS without accompanying pontine lesion was observed in the remaining two patients. For the first time, the prevalence of classic LIS has been established at 0.7/10,000 somatic nursing home beds in all Dutch long-term care organisations. Possible explanations for this low prevalence could be the Dutch provision of home care or the influence of end-of-life decisions, such as euthanasia and withholding or withdrawing all medical treatment, including artificial nutrition and hydration. These alternate outcomes should be explored in further studies.

The reliability and validity of the PALOC-s: a post-acute level of consciousness scale for assessment of young patients with prolonged disturbed consciousness after brain injury.

The objective of the study was the validation of the Post-Acute Level of Consciousness scale (PALOC-s) for use in assessing levels of consciousness of severe brain injured patients in a vegetative state or in a minimally conscious state. A cohort of 44 successively admitted patients (between 2 and 25 years of age), who were treated in an early intensive neurorehabilitation programme, were included in the study. Each patient was examined, using the Western Neuro Sensory Stimulation Profile (WNSSP) and the Disability Rating Scale (DRS), once every two weeks resulting in 327 examinations (all videotaped). To determine the reliability of the PALOC-s, six observers rated one videotape of each patient. One of the observers rated the same tapes a second time, 3-4 months later. Validity was determined by correlating 100 ratings of one observer with the scores on the WNSSP and the DRS. To determine the responsiveness of the PALOC-s, the size of change between the scores of the first and last examinations was calculated. The inter-observer correlations and agreement scores varied between .82 and .95. The intra-observer correlation and agreement scores varied between .94 and .96. Correlations with the WNSSP varied between .88 and .93, and with the DRS between .75 and .88. The responsiveness was significantly high (t=8.2), with a standardised effect size of 1.30. It is concluded that the PALOC-s is a reliable, valid, and responsive observation instrument provided it is administered after a structured assessment by an experienced and trained clinician. The PALOC-s is feasible for use in clinical management, as well as in outcome research.

Prevalence and characteristics of patients in a vegetative state in Dutch nursing homes.

OBJECTIVES: To establish the prevalence of vegetative state in Dutch nursing homes, describe the patient characteristics, and highlight the possible influence of medical decisions at the end of life. DESIGN: A cross-sectional survey. The vegetative state was defined according to the Multi Society Task Force on PVS. All Dutch nursing homes were approached to provide data on patients in a vegetative state. In cases of doubt, the researcher discussed the diagnosis with the patient's physician and, if necessary, examined the patient. Information on patients in a vegetative state in care between 2000 and September 2003 and end of life decisions for them were also recorded. RESULTS: All nursing homes and physicians participated. After assessment of 12 doubtful patients, 32 met the criteria of vegetative state lasting longer than one month, a prevalence of 2/1,000,000. Of these, 30 patients' data were analysed: age 9-90 years; 73% female; duration of vegetative state 2 months-20 years (26 surviving >1 year, 13 >5 years). Stroke was the commonest cause. Between 2000 and September 2003, there were 76 patients in a vegetative state in care of whom 34 died of complications and nine after withdrawal of artificial nutrition and hydration. CONCLUSIONS: The prevalence of vegetative state in Dutch nursing homes has been established for the first time. The figures are lower than suggested in the literature. The study included a heterogeneous group of patients, of which a substantial number survived for many years. The results cannot be explained by a policy of systematically withdrawing artificial nutrition and hydration.

[Allowing a patient in a vegetative state to die in hospital under the direction of a nursing-home physician]. / Het laten sterven van een patiënt in een vegetatieve toestand in het ziekenhuis met de verpleeghuisarts als hoofdbehandelaar.

In the case of a 52-year-old man, who was in a vegetative state after resuscitation following an unwitnessed cardiac arrest, the nursing-home physician assumed responsibility for the total treatment in a transfer unit of the hospital. His systematic, multidisciplinary approach enabled direction of the complex situation in which many medical and paramedical personnel were involved. When an airway infection occurred as a complication shortly after responsibility for the patient had been assumed, the nursing-home physician could take a well-considered decision to withdraw medical treatment on the basis of the treatment plan which had been formulated in a short time and contact with the family. In clinical practice it is difficult to determine the most appropriate moment to withdraw all medical therapy, including artificial nutrition and hydration, in order to prevent a hopeless vegetative state. This case illustrates how hospital physicians and nursing-home physicians may cooperate during the 'waiting phase' of the clinical course of a vegetative state. A hopeless vegetative state can be prevented by using these transmural possibilities for cooperation, including an early input of knowledge and experience about the long-term course of a vegetative state.

[Diagnosis of vegetative state as a basis for medical treatment on the borderline between life and death]. / Diagnostiek van vegetatieve toestand als basis voor medisch handelen op de grens van leven en dood.

The term 'vegetative state' is most appropriate for the state which develops when patients open their eyes after a comatose phase, without regaining consciousness. The definition and the diagnostic criteria from the Multi Society Task Force on Persistent Vegetative State are usable for the clinical practice in the Netherlands. The vegetative state must be differentiated from coma, locked-in syndrome and minimally conscious state. A systematic, multidisciplinary approach under the direction of a physician is key to diagnosing vegetative state. To this end, a clinical assessment is recommended with reassessment and verification of the diagnosis at appropriate moments. Careful observation remains the fundamental to the diagnosis.