Diagnostic Dilemma

Weakness with head lag

A 7-year-old boy born of non consanguineous marriage presented with fever, vomiting and increased sleepiness for 7 days and poor head control and weakness of lower limbs for 5 days. He had maculopapular rash 15 days ago which started over neck and then spread to rest of the body and then disappeared without desquamation. There are no cranial nerve involvement or seizures. Birth history is non contributory. He is immunized with measles vaccine and up to 2nd booster. Milestones are normal. On examination, vital parameters are normal. He is conscious and oriented with no cranial nerve palsy. Tone and power is normal. Reflexes in lower limbs are sluggish and planters are flexors. Superficial reflexes are present. He has wobbling on sitting up. There is no nystagmus. Other systems are normal and bladder and bowel are normal. Investigations showed:
• MRI brain = Focal gliotic area in right centrum semiovale
• CSF = 2 lymphocytes/hpf, proteins 12.5 mg/dl and sugar 70 mg/dl
• HIV tridot = Negative
• Serum electrolytes = Normal
• Hemoglobin = 9.8 gm/dl, WBC count = 10,700 (77% polymorphs, 16% lymphocytes)
• Liver and renal function tests = Normal
• Venous blood gas = No acidosis
• Serum ammonia, sugar = Normal
• MRI spine = Normal
• EMG/NCV = Normal

What is the diagnosis?
Expert Opinion :
This child presented with poor head control and wobbling with not able to maintain his balance which the parents took as weakness of the limbs. However, on examination, he has ataxia and power is alright. His deep tendon reflexes in lower limbs are sluggish though superficial reflexes are present. All this is just suggestive of cerebellar ataxia and no paraplegia or hemiplegia. The cause of his cerebellar ataxia could be a viral infection as he did have a maculopapular rash 15 days prior to this episode. Thus, the child could have had a post infectious acute cerebellitis which is a benign condition and improves on its own. It is commonly seen following chickenpox. No treatment is required and investigations in form of neuroimaging will be normal. Since this child presented with weakness and sluggish lower limb reflexes, extensive workup for ADEM, transverse myelitis and GBS was done, but the causes were overruled. Thus, all investigations were normal and acute cerebellitis improved on its own. This just highlights need for a proper clinical history and examination to prevent unnecessary investigations
Answer Discussion :
victoria Fanslow
3 Days ago
soundar rajan
post viral demyelination
11 Days ago

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