Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 37-year-old woman developed memory disturbance and seizures preceded by headache and high fever. Physical examination revealed persistent high fever, confusion, neck stiffness, anterograde and retrograde amnesia and disorientation. CSF showed pleocytosis and normal glucose level, and negative herpes simplex virus DNA on PCR. The fluid attenuated inversion recovery (FLAIR) MRI of the brain demonstrated nearly symmetric high signal intensity areas in the bilateral mesial temporal lobes. The tentative clinical diagnosis was non-herpetic acute limbic encephalitis (NHALE), and administration of methylprednisolone improved her conditions. Laboratory tests revealed mild hypothyroidism with high titers of serum antibodies against TPO and thyroglobulin, consistent with Hashimoto's disease. In addition, antibodies against amino terminal of alpha-enolase in the serum and those against glutamate receptor (GluR) epsilon2 in the serum and CSF were positive. A final diagnosis of Hashimoto's encephalopathy associated with GluRepsilon2 antibody was made. The present case suggests that NHALE-like clinical manifestation can be produced by autoimmune-mediated encephalopathies.
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PMID:[Non-herpetic acute limbic encephalitis-like manifestation in a case of Hashimoto's encephalopathy with positive autoantibodies against ionotropic glutamate receptor epsilon2]. 1809 94

The contribution of autoimmune phenomena to dysfunction of hypophysis or hypothalamus is far to be unraveled and also the specific pathways of hypophysitis are poorly understood until now, mostly for the pediatric population. Primary hypophysitis is rare in children and often regarded as an autoimmune disorder, following the evidence of lymphoplasmacytic infiltration in the pituitary gland, detection of anti-pituitary antibodies (APA) and anti-hypotalamus antibodies (AHA) by indirect immunofluorescence on cryostatic sections of human or primate hypophysis and hypothalamus, and coexistence with other autoimmune disorders. The rarity of this condition and the lack of ad hoc studies make hard any assessment of the real incidence of hypophysitis in pediatric patients, and also the role of APA and AHA has been poorly investigated in children with idiopathic hypopituitarism. Potential target autoantigens studied in autoimmune hypophysitis have been various pituitary-specific factors, chaperone proteins, alpha-enolase, secretogranins, chorionic somatomammotropin and intracellular transcription factors. Many clinical features both endocrine and neurologic or systemic can herald the onset of autoimmune hypophysitis. Antidiuretic hormone deficiency with central diabetes insipidus and growth retardation are the most significant presenting symptoms in children with hypophysitis, requiring a careful differential diagnosis with other causes of hypopituitarism, including tumors of the sellar region, differently from adults in whom adrenal insufficiency, hypogonadism, headache or diplopia might be the leading manifestations. Growth hormone deficiency is found in 3/4 of pediatric cases. Five histologic variants of primary hypophysitis have been described: lymphocytic, granulomatous, xanthomatous, IgG4-related and necrotizing: lymphocytic hypophysitis is the most frequent variant in the pediatric sceneries. Children with diagnosis of hypophysitis are also at risk of developing germinomas later in life, and require an extended follow-up in the long-term. Therapeutic options should be differentiated according to the rapidity of disease progression and modality of clinical onset, as acute pictures might require corticosteroids or immunosuppressant agents, while chronic forms may need a conservative management or appropriate hormone replacement therapies. This review updates and summarizes the most recent information related to the autoimmune involvement of hypophysis and hypothalamus in children, discusses the correlations between APA, AHA and disease activity, as well as the recommendations for treatment of primary hypophysitis from the pediatric perspective.
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PMID:Autoimmune phenomena involving the pituitary gland in children: New developing data about diagnosis and treatment. 3140 42