Vomiting, quadriparesis and tremors
Author:
Pediatric Oncall
Question
A 4 years old boy presented with projectile vomiting for 3 months which subsided for 15 days in between, weakness of both lower limbs since 2 months which has now involved even the upper limbs for past one month and head lag for one month. There is inability to stand, walk and sit and child falls when made to sit. However the child is able to move all 4 limbs and there is no spasticity and mother finds the child to be `loose`. There is history of tremors at the onset of the disease. There is no fever, headache, seizures or cranial nerve involvement. There is no drug ingestion.
What is the likely diagnosis?
Expert Opinion :
At a glance, this appears to be an ascending paralysis like Guillian Barre syndrome (GBS) or poliomyelitis. But it still does not explain the vomiting, tremors in this child. If we start with the onset of symptoms, projectile vomiting is suggestive of central nervous system pathology such as raised intracranial pressure or involvement of vomiting centre. Weakness starting in both lower limbs suggests involvement of white matter (pyramidal tracts). Involvement of bilateral pyramidal tracts with predominantly lower limbs being involved suggests periventricular problem. A possibility of hydrocephalus thus comes into the picture. Head lag suggests a bulbar involvement. Thus primary lesion seems to be in the posterior fossa that is leading to hydrocephalus. A posterior fossa lesion in the brainstem would lead to other cranial nerve involvement. Thus brainstem lesion is less likely. With hydrocephalus, one would have expected the child to have spasticity of lower limbs. However the mother does not find the child to be stiff. Infact she finds him `loose` suggestive of hypotonia. Hypotonia in a CNS affection can occur either with neuronal shock or with cerebellar involvement. This child is not in neuronal shock. Thus cerebellar involvement is a possibility especially when there were tremors at the onset and inability to maintain balance when made to sit. Thus, this child is suspected to have hydrocephalus with cerebellar disease and either raised intracranial tension or involvement of vomiting center suggestive of a space occupying lesion at level of 4th ventricle such as medulloblastoma or ependymoma. On examination, this child was hypotonic, had brisk deep tendon reflexes with bilateral ankle clonus and extensor planters. Imaging proved a diagnosis of medulloblastoma. Thus every ascending paralysis is not GBS.