Interventions to improve outcomes in children and young people with unresponsive wakefulness syndrome following acquired brain injury: A systematic review.

Unresponsive wakefulness syndrome (UWS) is a disorder of consciousness (DoC) which describes a state of wakefulness without evidence of self or environmental awareness, or interaction. There is currently no universally accepted evidence-based intervention for the treatment of UWS. This systematic review aimed to identify interventions to improve functioning in children and young people (0-25 years) with UWS following acquired brain injury (ABI). A systematic review of electronic databases was conducted, consisting of CINAHL, EMBASE, Medline, PsycINFO, PubMed, Cochrane Library, Scopus and Google Scholar. Eight studies met inclusion criteria. In these studies, the outcomes of interventions which aimed to improve quality of life, functional outcomes and/or increases in level of consciousness of paediatric patients in UWS were reported. Retrieved studies presented some evidence for improved consciousness and functional outcomes, following multi-component neurorehabilitation programmes, sensory stimulation or pharmacological interventions. Quality appraisal, using a modified version of the Downs and Black (1998) checklist, revealed risk of bias in a number of sources, including insufficient control over confounding variables, the use of inadequately validated outcome measures and concerns regarding diagnostic accuracy. More robust research is needed to adequately determine which interventions are most valuable at improving outcomes in paediatric UWS and to provide an improved evidence base for clinicians to draw upon when selecting treatment for patients.

Characterizing memory loss in patients with autoimmune limbic encephalitis hippocampal lesions.

Since the publication of Scoville and Milner's (1957) seminal paper, the precise functional role played by the hippocampus in support of human memory has been fiercely debated. For instance, the single question of whether the hippocampus plays a time-limited or an indelible role in the recollection of personal memories led to a deep and tenacious schism within the field. Similar polarizations arose between those who debated the precise nature of the role played by the hippocampus in support of semantic relative to episodic memories and in recall/recollection relative to familiarity-based recognition. At the epicenter of these divisions lies conflicting neuropsychological findings. These differences likely arise due to the consistent use of heterogeneous patient populations to adjudicate between these positions. Here we utilized traditional neuropsychological measures in a homogenous patient population with a highly discrete hippocampal lesion (i.e., VGKCC-Ab related autoimmune limbic encephalitis patients). We observed consistent impairment of recent episodic memories, a present but less striking impairment of remote episodic memories, preservation of personal semantic memory, and recall but not recognition memory deficits. We conclude that this increasingly well-characterized patient group may represent an important homogeneous population in which the functional role played by the hippocampus may be more precisely delineated.

Assessing evidence quality in research reporting neurocognitive outcomes following paediatric temporal lobe surgery for epilepsy.

PURPOSE: RCTs are the gold standard in determining intervention efficacy with journal impact factor assumed to index research quality. Flint et al's (2017) systematic review examined neurocognitive outcomes following paediatric temporal lobe epilepsy surgery. Retrieved evidence was restricted to non-RCTs, which pose greater risk of bias and thus diminish research quality. The current study evaluated risk of bias in sources retrieved by Flint et al. and explored whether impact factor related to research quality within this selected field. METHODS: Methodological and reporting bias was evaluated using categories of bias specified by Cochrane. The relationship between the identified number of biases and journal impact factors of retrieved sources was examined. RESULTS: All studies carried substantial risk for bias. Methodology bias included low sample size (76.71%; 56/73), risk of confounding cognitive outcomes due to failure to report pre-surgery neurocognitive data (21.92%; 16/73) and to determine whether patients were prescribed antiepileptic drugs at follow-up (53.42%; 39/73). Reporting bias included overstating claims based on findings (53.42%; 39/73), failure to report individual patient characteristics (66%; 33/50) and omitting the nature of surgical interventions (15.07%; 11/73). The number of sources of common bias within studies was not associated significantly with journal impact factor (p = .878). CONCLUSION: This evaluation highlights risk of bias when sources are predominantly uncontrolled non-RCTs and provides evidence that journal impact factor is not a reliable indicator of quality within this field. Authors should limit bias in their methods and reporting of results, to ensure the highest quality evidence possible is used to inform treatment decisions and prognosis.

Neurocognitive, academic and functional outcomes in survivors of infant ependymoma (UKCCSG CNS 9204).

PURPOSE: This is the first UK multi-centre case-controlled study with follow-up in excess of 10 years to report the neurocognitive, academic and psychological outcomes of individuals diagnosed with a brain tumour in early childhood. Children enrolled into the UKCCSG CNS 9204 trial, diagnosed with intracranial ependymoma when aged ≤ 36 months old, who received a primary chemotherapy strategy to defer or avoid radiotherapy, were recruited. METHODS: Outcomes of those who relapsed and subsequently received radiotherapy (n = 13) were compared to those enrolled who did not relapse (n = 16), age-matched controls-diagnosed with solid non-central nervous system (SN-CNS; n = 15) tumours or low-grade posterior fossa pilocytic astrocytoma (PFPA; n = 15), and normative data. Analyses compared nine neurocognitive outcomes as primary measures with quality of survival as secondary measures. RESULTS: Relapsed ependymoma participants performed significantly worse than their non-relapsed counterparts on measures of Full Scale IQ, Perceptual Reasoning, Word Reading and Numerical Operations. The relapsed ependymoma group performed significantly worse than SN-CNS controls on all primary measures, whereas non-relapsing participants only differed significantly from SN-CNS controls on measures of Processing Speed and General Memory. Relapsed ependymoma participants fared worse than all groups on measures of quality of survival. CONCLUSIONS: The relapsed irradiated ependymoma group demonstrated the most significantly impaired neurocognitive outcomes at long-term follow-up. Non-relapsing participants demonstrated better outcomes than those who relapsed. Results tentatively suggest avoiding radiotherapy helped preserve neurocognitive and learning outcomes of individuals diagnosed with ependymoma when aged ≤ 36 months old. Prospective neurocognitive surveillance is required. Recommendations for clinical and research practice are provided.