A 9 months old boy with encephalopathy, leucopenia and subdural hematoma

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A 9 months old boy with encephalopathy, leucopenia and subdural hematoma

Author: Pediatric Oncall

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Case Repot: - A 9 month old boy born of non-consanguineous marriage presented with cough and fever since 3 days and 2 episodes of generalized tonic convulsions since 1day. He had history of cough and cold 2 months back for which he was admitted for 8 days and started on 3 drugs. Antituberculous therapy {AKT} and was on the same since then. His birth was uneventful and he was immunized till date. He was on exclusive breast feeds and no weaning had been started. His milestones were delayed and he had achieved only head holding and babbling till date. On examination, his vital parameters were normal. He had pallor, hyperpigmented fingers. There were no meningeal signs. Systemic examination revealed bilateral crepitations on respiratory system and hepatosplenomegaly. He was altered but cranial nerves, tone and power were normal. Deep tendon reflexes were brisk and planters were flexors. Investigations showed:
- Hemoglobin = 11.3 mg, dl {MCV = 75 fl, MCH = 24.8 pg, MCHC = 32.7 gm, dl}
- White cell count = 1,900, cumm {10 percent polymorphs, 87 percent lymphocytes, 2 percent monocytes, 1 percent eosinophil}
- Platelet count = 2,60,000, cumm
- ESR = 15 mm at end of 1 hour
- CSF = Normal {No cells}
- X-Ray Chest = Bilateral bronchopneumonia
- SGOT, SGPT, Calcium, Random blood sugar, serum electrolytes, blood gases, serum ammonia = Normal
- HIV, Mantoux test = Negative
- Prothrombin Time {PT} and Partial thromboplastin Time {PTT} = Normal
- Urine = Normal
- CT brain = Intrahippocampal cyst on left side with bilateral subdural hematoma.
- Peripheral smear = Hypersegmented polymorphs with megaloblasts.

What is the diagnosis_?

Answer Discussion :

S

Sangeethaca

0

storage disorder

15 years ago

S

Sangeethaca

0

Adrenoleucodystrophy

15 years ago

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Answer 1

The author replies: The patient continued to remain unconscious and his pneumonia worsened inspite of potent antibiotics. His repeat hemogram after 6 days showed drop in hemoglobin by 2 gm percent leucopenia and thrombocytopenia. However child expired after 10 days of hospitalization. The child was considered to have low cell line due to megaloblastic anemia {in view of exclusive breast feeds, hyperpigmentation of skin and low cell counts with subdural bleeds}. The child was also suspected to have pulmonary bleeds which on X-ray may have appeared as bilateral bronchopneumonia. Thus, megaloblastic anemia may have most probably led to all complications.

A 9 months old boy with encephalopathy, leucopenia and subdural hematoma

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