Hematopoietic stem cell transplant(HSCT) is the only modality that, if performed in the early- stages of cerebral X-linked adrenoleukodystrophy(X-ALD) can lead to good outcomes. Loes- score, calculated based on the findings of the magnetic resonance imaging(MRI) of the brain, helps in the selection of patients for HSCT. As per recommendations, the Loes-score should be <10 for a patient to be eligible for HSCT. Here we report a child of cerebral X-ALD with Loes of 12, but a good neurological function score(NFS), who was treated with HSCT, and had good outcomes. It is therefore important to utilize other parameters along with the Loes score, in selecting patients for HSCT in X-ALD.

Paraneoplastic neurologic syndromes (PNS) are rare in pediatrics and are understood to be consequences of cross-reactivity against various neuroendocrine antigens expressed on cancer cells. Here, we report a case of autoimmune encephalitis, a type of paraneoplastic neurologic syndrome that was associated with a case of adrenocortical carcinoma and had some clinical response to immunosuppressive therapy. Adrenocortical carcinoma is a rare tumor with controversial tissue of origin but expresses various neuroendocrine antigens that could be the possible mechanism for this rare yet interesting association.