Nirmalya Sarkar

Acute disseminated encephalomyelitis in a child with chikungunya virus infection

An 8-year-old boy was admitted to the Pediatric ward with high fever and severe arthritis of 4 days duration. Chikungunya virus serology was positive. As he became afebrile one week after admission, he developed acute onset right sided hemiparesis and suffered from one episode of generalized tonic clonic seizure. The next day he developed flaccid quadriplegia, loss of all sensory modalities below the C5 dermatome and urinary retention. After 4 weeks of shock stage, spasticity appeared in all 4 limbs. Magnetic resonance imaging (MRI) brain and spine showed extensive areas of demyelination-suggestive of acute disseminated encephalomyelitis (ADEM). He was treated with intravenous methyl prednisolone for 5 days, followed by oral prednisolone for 6 weeks. He was left with gross neurodeficits, including confinement to a wheel chair, persistent sensory loss and need for bladder catheterization.

Flaccid quadriparesis due to severe myositis and Bell's palsy in pediatric dengue infection

A 10-year-old-boy was admitted with high grade continuous fever, rash, arthralgia and myalgia. From the 4 th day of fever, his myalgia increased in intensity and he developed flaccid quadriparesis on the 5 th day. Subsequently he was diagnosed to have dengue infection and myositis. He also developed right sided Bells palsy 4 days after he became afebrile. He was treated with intravenous methyl prednisolone for 3 days followed by oral prednisolone for 1 week with resultant dramatic resolution of myalgia and weakness. Physiotherapy was advised for Bells palsy. At follow up 2 months later, the child had regained grade 5/5 power in all four limbs, but the weakness of the facial muscles persisted.

Cholinergic crisis, intermediate syndrome and delayed polyneuropathy following malathion poisoning

Organophosphate poisoning is common in developing countries, more so in India. A 7-year-old boy with history of ingestion of unquantified amounts of malathion, presented to the pediatric emergency of Calcutta National Medical College, Kolkata in acute cholinergic crisis. He was treated with atropine and pralidoxime. The acute cholinergic crisis persisted for four days after admission, and as it subsided, the child developed respiratory distress, bulbar palsy, cyanosis, hypoxemia and hypercarbia. A diagnosis of intermediate syndrome was made and patient needed prolonged ventilation for 21 days for type II respiratory failure. Five days after discharge, the child developed distal weakness of both lower limbs, which later involved the hands. There was associated gross sensory loss. Nerve conduction velocity study and sural nerve biopsy predominantly showed evidence of sensory-motor axonopathy with associated demyelination. The child was treated with physiotherapy, but was left with disabilities like claw hand, foot drop and the inability to walk.

Lobar holoprosencephaly with Arnold-Chiari type III malformation

Holoprosencephaly (HPE) sequence is a rare spectrum of cerebral and facial malformations resulting from incomplete division of embryonic prosencephalon into diencephalon and telencephalon. De Meyer classified three ranges in order of in- creasing severity: lobar, semilobar and alobar HPE. A subtype called middle inter-hemispheric fissure variant has also been described. Arnold-Chiari III malformation is extremely rare and comprises of occipital encephalocele and associated cerebellar and brain stem abnormalities. This case report presents a male infant with lobar HPE and Arnold-Chiari III malformation without any facial defects and chromosomal abnormalities.

Developmental brain anomaly due to fetal hydrocephalus

In this article, the authors intend to illustrate a striking brain malformation due to early onset severe fetal hydrocephalus that superficially suggests the presence of semilobar holoprosencephaly.

Bickers-Adams syndrome with absent septum pellucidum – A case report

A 20-year-old primigravida with no antenatal care presented to the Obstetric outpatient department at 37 wk completed gestational age. Antenatal ultrasound demonstrated isolated gross fetal ventriculomegaly with absent septum pellucidum. Adduction deformity was present in both thumbs. He had tumultuous post natal life with seizures from 2 h of life, recurrent apnea and death at 72 h of age. Postnatal computed tomography showed hydrocephalus due to aqueductal stenosis and absent septum pellucidum. The clinical and radiological features were consistent with the diagnosis of Bickers-Adams syndrome.