Does a regular Wessex Head Injury Matrix assessment identify early signs of infections in people with Prolonged Disorders of Consciousness?

BACKGROUND & OBJECTIVE: Patients with brain injury are at high risk for infections. Although infection and cognitive deterioration are established for people with dementia, this has not been shown for patients with a prolonged disorder of consciousness (PDOC). This study determines whether regular Wessex Head Injury Matrix (WHIM) assessments can identify early signs of infections in patients with PDOC. METHOD: Retrospective and prospective approaches were used to assess the WHIM scores of patients with a PDOC (N = 21 in the retrospective study and 22 in the prospective study). RESULTS: The WHIM total scores decreased due to infections in 17 of the 21 cases of infection (p < 0.001) in the retrospective study and 15 (p = 0.001) of the 22 prospective cases of infection. Patients in a minimally conscious state (MCS) showed a bigger proportion of change between their baseline score and the scores taken in the pre-infection stage in both the retrospective and prospective studies when compared to patients in a vegetative state (VS). CONCLUSION: The findings suggest the importance of serial WHIM assessments throughout the period of recovery, not only to measure cognitive changes but also to highlight underlying physical changes such as infections that will impact the response to rehabilitation and recovery.

Sheehan's syndrome and sickle cell disease: the story of Natasha.

Sheehan's syndrome (SS) is one of the pituitary disorders caused by severe blood loss during childbirth leading to necrosis of the pituitary gland. Diagnosis is made following severe haemorrhage, failure to produce milk and failure to menstruate. Rare in countries with good obstetric care, SS is still frequent in those countries with poor healthcare services. The majority of papers published on SS do not mention psychological or neuropsychological sequelae. Of those that do, mood disorders are sometimes reported and occasionally cognitive problems are noted, typically attention, memory or executive deficits. We report on Natasha, a British woman of Caribbean ancestry with sickle cell disease (SCD), diagnosed at age 33 years with SS following the birth of her second child. We know of no reports of a person with both SCD and SS. Natasha has been seen regularly for neuropsychological assessment and treatment for over two years. There has been a slight increase in her cognitive functioning but she remains with Balint's syndrome, so is unable to read. Treatment for this has been partially successful. Natasha presented with psychotic type symptoms when first seen but these have improved to a large extent. We believe that these symptoms are consistent with SS but the severe cognitive problems are more likely to be due to the strokes she suffered at the time of the haemorrhage and, possibly, to hypoxic damage caused by very low blood pressure.

Brugada syndrome and the story of Dave.

Brugada syndrome (BrS) is a little known genetic condition that causes severe disturbances in cardiac rhythm and may result in sudden unexpected cardiac death in an apparently healthy person. The heart structure is typically normal but there are problems with electrical activity. The syndrome is named after Spanish brothers who are cardiologists, Pedro and Josep Brugada. BrS is the major cause of sudden unexplained death syndrome (SUDS), also known as sudden arrhythmic death syndrome (SADS). Following a description of the syndrome, including its prevalence and incidence, how it is diagnosed and how it can be treated, we consider those who survive a cardiac arrest and what problems they may face. Most publications focus on the medical aspects of BrS but, of course, cardiac arrest can result in hypoxic brain damage. We conclude with the story of Dave, a 25-year-old man diagnosed with BrS following a nose bleed and subsequent cardiac arrest. He was left with a visual impairment, dystonia, hypersensitivity, and language and cognitive dysfunction. We look at Dave's strengths and weaknesses, his response to offered treatment, and his consequent improvement. We stress the contributions from members of the multidisciplinary team and offer suggestions for the rehabilitation of other survivors of BrS.

Alexander's disease and the story of Louise.

We describe the rare condition known as Alexander's disease or Alexander's leukodystrophy, which is essentially a childhood dementia. We then present the case of Louise Davies (we are using Louise's real name with the permission and special request of her mother), a woman who was diagnosed with this disease at the age of 5 years and is still alive at the age of 38, making her the longest known survivor of this condition. Although now severely impaired, both physically and mentally, and able to do very little, she has lived far longer than expected. We present some neuropsychological results from her childhood before measuring her decline over the past four years. We conclude by considering whether or not the diagnosis was correct and why she has lived so long.

The natural history of continuing improvement in an individual after a long period of impaired consciousness: The story of I.J.

AIM: Recovery of consciousness and recovery of function among patients with prolonged disorders of consciousness rarely occur. Those patients who do regain consciousness typically remain with severe disability. The aim of this retrospective study is to suggest that continuing improvement is possible in a survivor of catastrophic brain injury after being in a prolonged state of disordered consciousness. CASE STUDY: This retrospective single case study follows the progress of a 29 year old man, I.J, who sustained a severe traumatic brain injury following an assault in October 2011. He was in a vegetative state for 15 months and in a minimally conscious state for a further 4 months. This was followed by a slow and steady recovery of motor and cognitive functions. At 3 years post-injury I.J is considered to be moderately disabled. He is now living in the community with continuing outpatient support. On the disability rating scale his level of functioning is rated as mildly dependent. CONCLUSION: This study shows that continuing recovery to a level of moderate disability is possible, even after a prolonged disorder of consciousness. Intense multi-disciplinary long-term rehabilitation and cranioplasty may be contributing factors for such an unexpected recovery.

Paradoxical improvement of function more than 2 years after onset of tuberculous meningitis.

OBJECTIVE: To document the unexpected improvement made by a 50 year-old patient over 2 years after being diagnosed with tuberculous meningitis (TBM). METHODS: Regular neuropsychological assessments were carried out, initially with a test for patients in reduced states of awareness and later with more demanding tests. RESULTS: The patient was diagnosed with TBM in November 2008 and was mute, stuporous and barely more than minimally conscious for over 2 years. By February 2011, following the cessation of TBM medication, her conscious level had improved and she could be assessed on a range of neuropsychological tests. The patient presented with diffuse cognitive impairments coupled with focal neurological signs, but showed marked improvements in cognitive functioning compared to when admitted. CONCLUSIONS: This study demonstrates that late stage neuropsychological improvement is possible, even after 2 years of showing minimal awareness. Such paradoxical improvement of function is considered in the light of other paradoxical phenomena in TBM, comparisons are offered with similar neurological conditions and possible mechanisms underlying the dramatic improvement that took place are suggested.

A case study of locked-in-syndrome: psychological and personal perspectives.

OBJECTIVES: Although patients with Locked-In-Syndrome (LIS) are often stated to have normal cognitive functioning, the few reports of neuropsychological assessment in these cases suggest this is not always true. This paper (a) reviews published reports of neuropsychological assessments of LIS patients, (b) presents a detailed neuropsychological assessment of a patient to determine if she has normal cognitive functioning and (c) presents the views of the patient on what has happened to her. METHODS AND PROCEDURES: An in-depth single case report assessing the cognitive and emotional functioning of a young woman with LIS plus a personal account from the patient. Detailed assessments were carried out using standardized neuropsychological tests and questionnaires measuring emotional functioning, pain and quality-of-life. The patient also states her views of what happened and how she feels. RESULTS: On most tests, cognitive functioning was average or above, but some impairments were found (consistent with published literature). No emotional problems were detected. Quality of life was satisfactory. Pain perception was normal. CONCLUSIONS: Although LIS patients have no severe cognitive deficits, some cognitive difficulties are common. Despite severe physical and communication problems and some mild cognitive deficits on visual reasoning tasks, this patient feels she has a reasonable quality-of-life.

Seeing red: Relearning to read in a case of Balint's Syndrome.

BACKGROUND AND AIMS: Balint's Syndrome is a rare condition, often associated with hypoxic brain damage. The major characteristic is an inability to localise objects in space, another is simultanagnosia frequently resulting in reading difficulties. We present RN, a 37 year old woman whose major problem with reading was her inability to recognise individual letters correctly in either lower or upper case. We noted, however, that she was better if the letters were shown in red type. The aims were to determine if RN could relearn letters of the alphabet, investigate whether colour affected her ability to learn, and to explore more specifically whether the red type also helped her to read words. METHOD: Using a single case experimental ABA design, we first determined that the optimal font for RN was size 16. In the baseline (A) phase, we assessed her ability to read all lower and upper case letters of the alphabet in black ink. In the intervention (B) phase we used font size 16 in red ink and an errorless learning approach to teaching the letters. Sessions ran 5 times per week (20 minutes per session). The intervention was then applied to picture recognition and word reading with four sets of 10 words and corresponding pictures. RESULTS: A consistent difference was noted between initial baseline and intervention. Improvement carried over when we returned to baseline. CONCLUSION: Using red type and an errorless learning approach enabled RN to re-learn letters of the alphabet and read words she was previously unable to read. This did not however generalise to her everyday life.