ABSTRACT
BACKGROUND: Intrathecal baclofen (ITB) is a useful treatment for hypertonia where non-invasive treatments have been ineffective or poorly tolerated. There is an absence of national guidance on selection criteria and a lack of literature regarding patient characteristics and treatment details for children and young people (CYP) receiving ITB therapy in the UK and Ireland. We aimed to gather patient and treatment characteristics for CYP receiving ITB in the UK and Ireland. METHODS: An electronic survey was sent to all paediatric ITB centres in the UK and Ireland. Anonymised data were returned between December 2019 and April 2020. CYP >16 years and those awaiting ITB pump removal were excluded from the dataset. RESULTS: 176 CYP were identified as receiving ITB therapy across the UK and Ireland. The majority of CYP with ITB pumps were non-ambulant (93%) with a diagnosis of cerebral palsy (79%). Median age of ITB insertion was 9 years; median current age was 14 years. 79% of CYP had significant spasticity, 55% had significant dystonia. The most commonly used ITB dosing modes were continuous (73%) and flexible (23%). CONCLUSIONS: ITB pumps were most frequently used for non-ambulant CYP with cerebral palsy and existence of spasticity and/or dystonia in the UK and Ireland. Most CYP were receiving a continuous dose of ITB. There is significant variation in the number of paediatric ITB pumps across UK and Ireland. There is a need for development of nationally accepted paediatric referral criteria and clinical standards for ITB use.
Subject(s)
Baclofen/administration & dosage , Muscle Hypertonia/drug therapy , Muscle Relaxants, Central/administration & dosage , Muscle Spasticity/drug therapy , Adolescent , Baclofen/therapeutic use , Cerebral Palsy/diagnosis , Cerebral Palsy/drug therapy , Child , Child, Preschool , Cross-Sectional Studies , Humans , Injections, Spinal , Ireland , Male , Muscle Relaxants, Central/therapeutic use , Surveys and Questionnaires , Treatment Outcome , United KingdomABSTRACT
A 9-year-old boy presented with a 2-day history of vomiting, ataxia and reduced consciousness. He had vomited intermittently in the two preceding months, without headaches, visual disturbance or early morning symptoms. He had autism spectrum disorder, and restricted eating since aged 2 years, eating only corn-crisps, Rich Tea biscuits and chips (French fries), and drinking Coca-Cola (containing 10% glucose; figure 1). Recently a dietician had prescribed a multivitamin.
Subject(s)
Autism Spectrum Disorder , Child Nutrition Disorders , Malnutrition , Autism Spectrum Disorder/complications , Autism Spectrum Disorder/diagnosis , Child , Diet , Humans , Male , Malnutrition/diagnosis , Vitamins/therapeutic useABSTRACT
OBJECTIVES: To examine the short-term effects of selective dorsal rhizotomy (SDR) ± soft-tissue surgery on gait in cerebral palsy (CP) compared to matched controls with no surgical intervention. METHODS: Participants had gait analysis before and one year after SDR. Non SDR participants were retrospectively matched for age and all significant gait parameters. The SDR group was further subdivided into those who had concomitant orthopaedic surgery and those who had SDR only. RESULTS: The SDR group consisted of 29 participants (mean age 5.8 years at baseline, 7.7 years at follow-up). Of these, 13 had concomitant orthopaedic surgery. The non SDR group consisted of 18 participants (mean age at baseline 6.1 years, 8.1 years at follow-up). SDR ± soft-tissue surgery significantly improved step-lengths, knee flexion at initial contact and mid-stance, ankle dorsiflexion, foot progression and timing of peak knee flexion. None of these improvements in gait were seen without surgical intervention. While more improvements were seen in those who had SDR and orthopaedic surgery, SDR only resulted in improved step-lengths, knee extension, foot progression and timing of peak knee flexion. CONCLUSIONS: SDR ± soft-tissue surgery results in short-term improvements in gait which are not seen without surgical intervention. While those who had SDR and soft-tissue surgery demonstrated more changes in gait, many improvements were attributable to SDR only.
Subject(s)
Cerebral Palsy/surgery , Gait Analysis/methods , Rhizotomy/methods , Biomechanical Phenomena , Case-Control Studies , Cerebral Palsy/physiopathology , Child , Child, Preschool , Female , Gait , Humans , Male , Range of Motion, Articular , Retrospective Studies , Treatment OutcomeSubject(s)
Mycobacterium bovis , Opportunistic Infections/complications , Severe Combined Immunodeficiency/complications , Tuberculosis/complications , BCG Vaccine/adverse effects , Humans , Infant , Male , Opportunistic Infections/diagnosis , Opportunistic Infections/etiology , Severe Combined Immunodeficiency/diagnosis , Tuberculosis/diagnosis , Tuberculosis/etiologyABSTRACT
We describe a case of acute functional deterioration in a 13-year-old girl with severe spastic diplegia (GMFCS III) and a new diagnosis of diffuse intrinsic pontine glioma (DIPG). She presented with acute deterioration in mobility and motor function over 1 month, which was associated with dysarthria, dysphagia and behavioural change. Her mother had noticed subtle functional deterioration over the 2 months prior to this. Her physiotherapist who was concerned about her acute functional deterioration referred her for emergency review. Neurological imaging revealed a diffuse pontine lesion consistent with DIPG. She was subsequently referred to oncology. She deteriorated further, clinically, over the next few days and following discussion with the team; her family opted for palliative treatment, given the poor prognosis associated with DIPG.
Subject(s)
Cerebral Palsy/complications , Acute Disease , Adolescent , Disease Progression , Female , Humans , Nervous System Diseases/etiologyABSTRACT
AIM: For the 9% to 16% of children with cerebral palsy (CP) who have normal brain imaging, further testing for metabolic and/or genetic conditions has been recommended. This study aimed to identify a cohort of children with CP with normal magnetic resonance imaging (MRI), clinically review and describe the cases, and assess the value of testing for inherited metabolic disorders in these children. METHOD: Children with congenital CP born from 1999 to 2005 were selected from a population register. Normal MRI reports were identified and the scans reassessed. Children whose scans were performed before 18 months were excluded, as were children with spastic CP (Gross Motor Function Classification System [GMFCS] level I). The remainder were reviewed clinically and offered investigations. RESULTS: Of 730 children identified, 515 had available imaging and 54 were confirmed as normal. Cases with non-spastic CP and those with milder clinical severity were more likely to have normal imaging. Twenty-three children (17 males, six females; mean age 6 y 11 mo, SD 1 y 10 mo, range 3 y 0 mo to 10 y 0 mo) were reviewed clinically and offered investigations. Twelve children had spasticity (11 with diplegia, one quadriplegia), three had dyskinesia, five ataxia, and three hypotonia. Two children functioned in GMFCS level I, 11 in level II, seven in level III and three in level IV. Four children with spasticity had unusual features. No alternative diagnoses were made. INTERPRETATION: Although important to consider in individual cases, comprehensive metabolic testing failed to clarify the aetiology of CP further in this large cohort of children with normal MRIs, even those with atypical features.
Subject(s)
Biomarkers/metabolism , Cerebral Palsy/etiology , Cerebral Palsy/metabolism , Magnetic Resonance Imaging , Metabolic Diseases/complications , Metabolic Diseases/diagnosis , Cerebral Palsy/congenital , Child , Child, Preschool , Female , Folic Acid/cerebrospinal fluid , Folic Acid Deficiency/diagnosis , Gestational Age , Humans , Male , Metabolic Diseases/genetics , Metabolic Diseases/metabolism , Motor Skills , Neopterin/cerebrospinal fluid , Prolactin/blood , Severity of Illness IndexABSTRACT
A 16-year-old boy presented with a number of asymptomatic pigmented macules on the volar aspect of his index fingers. Dermoscopy of each macule revealed a parallel ridge pattern of homogenous reddish-brown pigment. We propose that these lesions were induced by repetitive trauma from a Sony PlayStation 3 (Sony Corporation, Tokyo, Japan) vibration feedback controller. The lesions completely resolved following abstinence from gaming over a number of weeks. Although the parallel ridge pattern is typically the hallmark for early acral lentiginous melanoma, it may be observed in a limited number of benign entities, including subcorneal haematoma.
