earlier documented precocious puberty may have promoted development of an occult müllerian or breast adenocarcinoma. In this immunological, genetic, and hormonal con-text, a dysregulated cdr2-specific immune response may have restrained the growth of an adenocarcinoma at the expense of neurological autoimmunity.
ARTICLE INFORMATION
Paraneoplastic cerebellar degeneration is rare and noteworthy in children. In this
7-year-old, it was documented to have occurred within a year of ataxia presentation. The
instigating cancer was stage III adrenal adenocarcinoma, remitted after surgical resection
at age 2. When her severe ataxia progressed, neuroinflammation was characterized by high
cerebrospinal fluid Purkinje cell cytoplasmic antibody type 1 titers, oligoclonal bands,
and neurofilament light chain. The immunotherapy strategy was to replace IV
methylprednisolone, which lowered Purkinje cell cytoplasmic antibody type 1 titers without
clinical improvement, with induction of adrenocorticotropic hormone/intravenous
immunoglobulin/rituximab (ACTH/IVIG/rituximab) combination immunotherapy,
ACTH/dexamethasone transition, and intravenous immunoglobulin maintenance. She became
self-ambulatory and cerebrospinal fluid inflammatory markers regressed. Down syndrome
predisposed her to a second cancer, pre-B acute lymphoblastic leukemia, 4 years later.
Despite reversible cytosine arabinoside-provoked cerebellar toxicity, the ataxia is stable
on monthly intravenous immunoglobulin without relapse, now 5 years after initial
diagnosis. This report illustrates the use of cerebrospinal fluid biomarkers to detect,
target, and monitor neuroinflammation, and successful combinations of immunotherapy to
better the quality of life.
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