Clinical problem &its answer

Clinical problem-2-:  An 8 year old girl presented with fever, paralytic convergent squint since 2 months, weakness of right side of body since 15 days, inability to speak since 5 days and inability to eat since 3 days. There was no history of Koch’s. She had not been investigated in the past for her above complaints. There was no history of convulsions or altered sensorium. On examination, she had no meningeal signs and sensorium was normal. She had 3rd. 4th, 6th cranial nerve palsy with right lower motor neuron facial palsy. Her gag reflex was weak. Tone was increased on the right side and she had power of 1/5 on right side and speech had a nasal twang. Deep tendon reflexes were brisk bilaterally and planters were extensors. Other systemic examination was normal. Mantoux test, HIV, X-Ray Chest were normal.

Question:

What is the diagnosis? : Brainstem space occupying lesion. Since the child has multiple cranial nerve involvement with progressive symptoms over a period of 2 months, one should suspect a brainstem lesion. Since there are no meningeal signs, an infective cause and meningitis seem unlikely. Neuroimaging is required. CT done in this child showed hypodense non-enhancing area in the brainstem predominantly in the pons with compression of 4th ventricle suggestive of pontine glioma. She was started on radiotherapy and is on regular follow up. Brainstem gliomas are the 3rd most frequent posterior fossa tumor in children after cerebellar astrocytoma and medulloblastoma. The two varieties of brainstem gliomas are diffuse and low grade focal tumors. Diffuse gliomas are found to be anaplastic astrocytomas and infiltrate pons extending into the brainstem. Prognosis is poor. Focal tumors usually affect mid brain and pons and have excellent survival rates with surgery. Patients present with cranial nerve involvement such as diplopia, facial weakness, dysarthria, dysphagia and dysphonia. Gait abnormalities and spasticity may be seen due to pyramidal tract involvement. Raised intracranial tension occurs late in the course of the disease. Primary treatment of brainstem gliomas is irradiation. Chemotherapy is nor effective for management of brainstem gliomas. Focal tumor of midbrain or medulla responds extremely well to surgery and radical excision. Role of surgery in other types of brainstem gliomas is controversial. Top of Form