Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids is a rare inflammatory Central Nervous System disorder prominently affecting the brainstem. We present an adolescent’s reflection on this condition complicated with Epstein-Barr virus induced CNS Lymphoma. Case report: A 16-year-old boy presented about 5 years ago with a balance problem. He was diagnosed by MRI after ongoing debate whether this is juvenile multiple sclerosis. He initially responded to methylprednisolone but developed acute deterioration requiring 8 cycles of Infliximab and Methylprednisolone. He then confirmed cerebellar lymphoma 2 years later hence commenced on chemotherapy and radiotherapy after posterior fossa decompression. He gradually losses his motor skills, left hemiparesis and spasticity. He needs tracheostomy and gastrostomy due to poor bulbar function. Now, he is fully dependent and requires chest physiotherapy and suctioning and cystostomy for urinary incontinence. He has multiple PICU admissions due to recurrent aspiration, acute cerebellar oedema and post posterior fossa decompression. He worries about family breakdown as mum is his sole carer and having regular nightmare. He changed school to meet his care demands and unsure how to adapt to new environment. Social experience makes him anxious due to lack of understanding about his condition. He is evidently having low self-esteem and confidence. Discussion: Children palliative care team has been involved since diagnosis to support him and family. He is understandably depressed and clinical psychologist input bear minimal impact. He is fully aware of the current situation and his wish to rap Eminem’s songs like he used to do it before. Conclusion: Early aggressive treatment in CLIPPERS aim to prevent neuroaxonal loss. However, due to bellicose nature of the condition, the prognosis is relatively poor. Managing adolescent expectation after gradual deterioration is challenging especially, he is aware that achieving ‘normality’ is impossible as the treatment advancement still in limbo.
Acute onset quadriparesis can be a manifestation of a variety of neurological, metabolic or autoimmune conditions. Rarely, it could be one of many clinical presentations of juvenile dermatomyositis which belongs to the group of idiopathic inflammatory myopathies of childhood. We report the case of a 9-year old girl who presented with global myopathy over a two-month period. Case report: A 9-year-old girl referred with a 2 months history of generalised muscular pain and weakness. There were no significant history of preceded illness, trauma or excessive strenuous exercises. She had no other systemic complaints such as fever or skin rash. Past medical history was unremarkable except for recurrent tonsillitis. Physical examination revealed a child with normal alertness and behaviour. She has notable generalised swelling of all four limbs. Her neurological examination revealed normal tone however her power was definitely reduced in all four limbs (Graded 3/5). She demonstrated signs of proximal myopathy. Subsequent investigations revealed high Creatinine Kinase (CK) levels of 6470U/L, ESR 84mm/hr with deranged transaminases and positive serum mycoplasma and CMV IgM. Her MRI brain and spine along with lumbar puncture results were normal. She was referred to tertiary centre for further evaluation as her weakness progressed. An MRI muscle demonstrated marked inflammation in all four limbs including paravertebral muscles. Her muscle biopsy showed inflammatory myopathy leading to a diagnosis of juvenile dermatomyositis (JDM). She is now showing sustained clinical improvements following a course of immunoglobulin and corticosteroids therapy. Discussion: The diagnosis of JDM is generally considered in patients with rash-associated muscle weakness. Essentially, it involves assessment of muscle, skin, lung and cardiac involvement on top of baseline list of investigations that has been outline by Single Hub and Access point for pediatric Rheumatology in Europe (SHARE). This case reflects that JDM is possible despite non-existent of skin involvement.
Spinal tuberculosis in children is an established preventable disease in developing countries. Complications are devastating due to its aptitude to cause bone destruction, spinal deformity and paraplegia. Case report: We present an eight-month old girl with isolated gross motor regression and evolving spastic paraplegia. It highlights the challenge we encountered due to delay in garnering the pertinent investigation. She presented to clinic with history of legs weakness and loss of rolling after a period of prolonged febrile illness. Both parents are medical practitioners. Mother had history of SVT during pregnancy in spite on anti-arrhythmic treatments. Her father is a thalassaemia carrier. A thorough examination revealed gross motor delay and upper motor neuron signs. She had raised inflammatory markers, anaemia and thrombocytosis with persistent low-grade temperature. CT brain with contrast showed meningeal enhancement. Full septic work up revealed the CSF result reflecting partially treated meningitis. She was treated with third generation cephalosporin and acyclovir. Mother claimed exposure to TB patients hence Mantoux test was recommended which came positive. Her chest x-ray, sputum culture, CSF culture and NAA studies came non-conclusive for pulmonary tuberculosis. MRI for brain and spine showed features of tuberculous spondylodiscitis of T4-T5 vertebrae with associated subligamentous paravertebral spread and epidural extension causing spinal cord compression and T3-T6 hydro-syringomyelia. After multidisciplinary team discussion, patient started on intensive antituberculosis regimen with good initial response. Discussion: Clinically lower limbs power improved with good antigravity movement. Laboratory and radiological investigations have improved inflammatory markers and dropping trend thrombocytosis, and spinal gibbous stay stationary with improvement in plain radiology. She is under regular follow up awaiting serial MRI. Conclusion: The challenge in diagnosis of extra pulmonary tuberculosis in infants is getting them excluded early. High index of suspicion along with radiological investigation is vital to aid the diagnosis and establishment of treatment to expect a good outcome.
In Malaysia, a course of vaccination DTaP/IPV/Hib was introduced in 2008, replacing the 2006 DwPT-HBV/Hib+OPV vaccines. Severe systemic adverse reactions after diphtheria, tetanus and pertussis vaccination are uncommon. Cardiac complications are rarely reported and is most probably implicated to the pertussis component. We describe a rare case of acute myocarditis that developed 60 hours after DTaP/IPV/Hib vaccination. Case report: A 2-month old infant presented to emergency department after her first diphtheria, tetanus and pertussis vaccination due to severe respiratory distress and cyanosis. She had her BCG and two Hepatitis B vaccinations previously with no major side effects. Parents reported that she was feverish for 48 hours post vaccination with no other associated symptoms. Prior to presentation, she went floppy and was immediately brought to hospital. On arrival, she was tachypnoeic and cyanotic with hypoperfusion and hypotensive. She was also noted to have hepatomegaly. She was grunting and her level of consciousness deteriorated. She was immediately intubated and her first blood gas showed profound metabolic acidosis with pH 6.6, base excess -24mmol/L, lactate 14mmol/L and bicarbonate 4mmol/L. She required fluid boluses and inotrope infusion was commenced. She received antibiotics and sodium bicarbonate to correct her acidosis. Her echocardiography showed global hypokinesia, CK 3018 and positive Troponin. She was treated with immunoglobulin for myocarditis and on high frequency oscillation for 4 days before being extubated on day 11 of admission. All her viral serology and cultures came back negative. Discussion: Cardiac complications after diphtheria, tetanus and pertussis and other vaccinations are exceptionally uncommon. This patient developed sudden onset cardiogenic deterioration after an expected fever-like illness post vaccination. Given her viral screening and cultures were negative, this make acute myocarditis post vaccination a remote possibility. We concur that evaluation of cardiac state should be considered in recently vaccinated infants who manifest with cyanosis, hypoperfusion and drowsiness.