Aetiological Profile and Short-Term Neurological Outcome of Haemorrhagic Stroke in Children.

BACKGROUND: Haemorrhagic stroke (HS) accounts for nearly half of the paediatric strokes. The aetiology of HS in childhood is not well defined in the Indian context. OBJECTIVES: To study the aetiological profile and short-term neurological outcome of children with HS from North India. METHODS: In a prospective observational study, consecutive patients >28 days to <12 years of age admitted with a diagnosis of HS were enrolled. Demography, clinical, radiological details and investigations were recorded. Short-term outcomes were assessed at three months follow-up with the Paediatric Cerebral Performance Category scale and Paediatric Stroke Outcome Measure (PSOM). RESULTS: A total of 48 children with HS were enrolled. The median age was 6 months (1-58 months), and 33 (69%) were <2 years old. Vitamin K deficiency-related bleeding disorder (VKDB, 44%), central nervous system infections (19%), arteriovenous malformations (13%) and inherited coagulation disorders (8%) were the most common risk factors for HS. VKDB and inherited coagulation disorders were more frequent in children <2 years of age, and arteriovenous malformations were more frequent in children >2 years of age (p = 0.001). During hospitalization, 21 (44%) children died. Older age, low Glasgow coma score (<8) at admission and paediatric intracerebral haemorrhage score ≥2 were associated with mortality at discharge (p = <0.05). Among survivors, 15 (56%) children had neurological deficits (PSOM >0.5) at three month follow-up. CONCLUSION: VKDB, inherited coagulation disorders, central nervous system infections and arteriovenous malformations were the most common risk factors for HS. VKDB is the single most important preventable risk factor for HS in infants.

Status Dystonicus in Children: A Cross-Sectional Study and Review of Literature.

Background: Status dystonicus is a life-threatening, underrecognized movement disorder emergency. We aimed to ascertain the etiology, clinical presentation, complications, and outcomes of status dystonicus in children and reviewed the literature for similar studies. Methods: Records of all children aged <14 years admitted to a single center with status dystonicus between 2014 and 2018 were reviewed. Results: Twenty-four children (75% male) were identified with status dystonicus. The annual incidence rate was 0.05 per 1000 new admissions <12 years of age. The mean age at presentation was 6.3 ± 3.6 years. Median duration of hospital stay was 10.5 days (interquartile range 5-21.7). The severity of dystonia at presentation was grade 3 (n = 9; 37.5%) and 4 (n = 9; 37.5%). The most common triggering factor was intercurrent illness/infection (n = 18; 75%). The most common underlying etiologies were cerebral palsy (n = 8; 33.3%), complicated tubercular meningitis (n = 3; 12.5%), and mitochondrial disorders (n = 3; 12.5%). Basal ganglia involvement was seen in 15 cases (62.5%). Respiratory and/or bulbar compromise (n = 20; 83.3%) and rhabdomyolysis (n = 15; 62.5%) were most commonly seen. Oral trihexyphenidyl (96%) followed by oral or intravenous diazepam (71%), oral baclofen (67%), and midazolam infusion (54%) were the most common drugs used. Clonidine was used in 33% cases, without any significant side effects. Three children died owing to refractory status dystonicus and its complications; the mortality rate was 12.5%. Conclusion Status dystonicus is a neurologic emergency in children with severe dystonia, with significant complications and a high mortality rate. Static and acquired disorders are more common than heredo-familial causes. Identification and treatment of infection in children is important as the majority of cases are triggered by an intercurrent infection.

Comparison of 4 weeks versus 12 weeks antiseizure medication for acute symptomatic seizures in children with Acute Encephalitis Syndrome: An open-label, randomized controlled trial.

OBJECTIVE: To compare the proportion of children with seizure recurrence/s during 6-18 months of follow-up among children with acute symptomatic seizures underlying acute encephalitis syndrome (AES) treated with either 4 weeks or 12 weeks of antiseizure medication (ASM). METHODS: Eligible children aged 3 months to 12 years with acute symptomatic seizures underlying AES, receiving a single ASM at 4 weeks of illness, and without seizure recurrence from day 7-28 of illness were included in this comparative, parallel-group assignment, open-label, randomized control study (either 4 weeks or 12 weeks duration). The primary outcome was to compare proportions of children developing seizure recurrence over six months of follow-up. The secondary outcomes were to study seizure recurrence over 12-18 months follow-up and factor(s) associated with seizure recurrence. RESULTS: Of 232 children with AES screened, 60 eligible children were randomized in two groups. Baseline demographics were comparable (except the duration of illness) between the groups. Seizure recurrences at 6, 12 and 18 months were none, two (one in each group, relative risk 1.0, 95% CI 0.06-16.76; p-value >0.99), and six (one and five children in 4 and 12 weeks groups respectively, relative risk 0.17, 95% CI 0.01-1.62; p-value 0.19) respectively. There was no association of seizure recurrences with seizure characteristics, abnormal electroencephalography and neuroradiology. Children with disabilities at randomization had a higher risk of seizure recurrence at 18 months of follow-up (relative risk 7.16, 95% CI 1.1-43.9; p-value 0.049). SIGNIFICANCE: With limitations of the study design, this study provides Class I evidence that a shorter duration (4 weeks) of ASM is comparable with 12 weeks therapy for preventing seizure recurrences in children with AES. TRIAL REGISTRATION: Clinical Trials.gov identifier: NCT03181945; Clinical Trial Registry of India identifier: CTRI/2017/06/008783.

Chest wall tuberculosis in children: a mimicker of bone tumour.

Osteoarticular tuberculosis of flat bones of the chest wall such as sternum, scapula and rib is extremely rare in children. Because of its atypical clinical presentation mimicking malignant bone tumours, diagnosis remains a challenge. Histological and microbiological diagnosis remains confirmatory. Antitubercular therapy is the cornerstone in management.

Additional considerations for "checklists to guide the supportive and critical care of tuberculous meningitis".

Checklists are pivotal in the systematic assessment of critically ill patients, pre-operative assessments and for patients with multisystem involvements. Management of tuberculous meningitis is challenging due to prolonged hospital stay, multiple neurological complications like seizures, stroke, raised intracranial tension, stroke, neurosurgical interventions, multiple invasive procedures, health-care-associated sepsis, and ventilation. All these complications are managed by separate checklists to avoid treatment-related errors. The current manuscript aims to ensure completeness of inpatient care addressing issues addressing diagnostic issues, supportive care, and intensive care related issues.

Comparative evaluation of IS6110 and protein antigen b PCR in cerebrospinal fluid for rapid diagnosis of tuberculous meningitis in children.

Introduction. Childhood tuberculosis meningitis is a severe form of tuberculosis with high morbidity and mortality. The diagnosis is frequently missed and delayed due to lack of sensitive tests like acid-fast bacilli (AFB) smear and delayed results by culture.Aims. To compare the role of IS6110 and protein antigen b PCR in cerebrospinal fluid (CSF) for rapid diagnosis of tuberculous meningitis (TBM) in children.Methodology. Forty-five cases of TBM and 20 controls were enrolled in this prospective study.Results. The mean ages of cases and controls were 4.2±0.5 years and 4.5±0.7 years, respectively. In the TBM group, two-thirds of the children were <4 years of age, and 62 % were males. Sensitivities of AFB smear examination, Löwenstein-Jensen (LJ) medium and bactenecin (BACTEC) culture in cases were 4.4, 0 and 2.2%, respectively. The protein antigen b PCR was most sensitive as it was positive in 35 (77.8 %) of TBM patients; IS6110 PCR was positive in 27 (60 %) patients. Both PCR-based tests had higher positivity than conventional tests and BACTEC culture. No significant difference was seen between the PCR tests. Excellent agreement was observed between both PCR-based tests as they were concordant for 26 positive samples and 35 negative samples.Conclusion. Protein b PCR is a sensitive and rapid method for the diagnosis of TBM (sensitivity 77.8 %). Both PCRs were more sensitive than smear, LJ and BACTEC. The specificity of both PCR was 100 %.

Black Holes in the Brain and Spine: A Dark Disease.

A 7-year-old girl presented with difficulty in walking and bilateral vision impairment since past 15 days. On examination, she had disc pallor, flaccid paraparesis with positive Babinski sign, and reduced sensations below clavicle. She was diagnosed as anti-aquaporin-4 (AQP-4)-positive neuromyelitis optica. This article emphasizes the importance of recognizing its classical neuroimaging findings distinct from other disorders.

Infection-Associated Opsoclonus: A Retrospective Case Record Analysis and Review of Literature.

Opsoclonus, an uncommon clinical sign, and is often described in the context of opsoclonus myoclonus ataxia syndrome (OMAS). OMAS may be paraneoplastic or postinfectious. However, opsoclonus with or without OMAS may occur in association with a wide gamut of infections. Infection-associated opsoclonus/OMAS (IAO) needs recognition as a separate entity, since it demands relatively brief immunosuppression, symptomatic treatment, and has a better outcome. Case records of children, who presented with opsoclonus to a tertiary-care teaching hospital of North India over a period of 1 year (2017-2018), were reviewed. Those with opsoclonus in the setting of an acute infection/febrile illness (symptomatic opsoclonus; IAO) were included. Of 15 children with opsoclonus, 6 children [median age: 42 months (range: 8 months to 7 years); 2 boys] had opsoclonus associated with an infective or febrile illness. Additional clinical findings in these children included myoclonus (n = 2), ataxia (n = 4) and behavioral abnormalities (n = 4). All these patients had an associated neurologic or nonneurologic illness- scrub typhus (n = 1), tuberculous meningitis (n = 1), mumps encephalitis (n = 1), brainstem encephalitis (n = 1), acute cerebellitis (n = 1), and subacute sclerosing panencephalitis (SSPE, n = 1). Children with acute cerebellitis, brainstem encephalitis, and mumps encephalitis were treated with steroids while those with scrub typhus, tuberculosis, and SSPE were treated with antibiotics, antitubercular therapy, and Isoprinosine, respectively. None of them needed long-term maintenance immunotherapy. The evaluation for tumor was negative in all. Three of the 6 children are functionally normal at the last follow-up. Acute neuro infections may trigger opsoclonus. A careful analysis of clinical data and suitable investigations can help differentiate these children from those with OMAS. This distinction may avoid unwarranted long-term immunosuppression.

Management Strategies of Melkersson-Rosenthal Syndrome: A Review.

Melkerrson-Rosenthal syndrome is a rare disorder of unknown aetiology and characterized by the triad of oro-facial edema, facial nerve palsy, and furrowing of the tongue. Two or more of the above are essential for making a clinical diagnosis. The mainstay of treatment is corticosteroids. Intralesional triamcinolone acetonide may be used for the treatment of oro-facial edema. Another treatment option for oro-facial edema includes intralesional betamethasone, along with oral doxycycline. The review discusses the management strategies in Melkersson-Rosenthal syndrome.

Wormian Bones and Dilated Scalp Veins in an Infant With Epilepsy.

A three-months boy presented with recurrent seizures. On examination, he was fair, had dilated scalp veins, sparse hypopigmented hair, and was hypotonic. X-ray of the skull showed wormian bones. The child was diagnosed with Menkes disease. The manuscript aims to emphasize dilated scalp veins in diagnosis of Menkes disease.