New onset refractory status epilepticus (NORSE) in a patient with paraneoplastic limbic encephalitis associated with craniopharyngioma: A rare case report.

Autor:	Lalwani, Jaya, Kumar, Sathish, Varadaraj, Priyadarshini, Karthik, Kishore, Thangaraj, Selvamani, Britto, Anthony Joseph, Gunasekaran
Předmět:	DIAGNOSIS of neurological disorders ENCEPHALITIS diagnosis ENCEPHALITIS COGNITION disorders APATHY METHYLPREDNISOLONE ANTICONVULSANTS PARANEOPLASTIC syndromes ADRENOCORTICAL hormones NEUROLOGICAL disorders STATUS epilepticus IMMUNOGLOBULINS PREDNISOLONE NEUROSURGERY IMMUNOSUPPRESSION MAGNETIC resonance imaging EARLY detection of cancer INTRAVENOUS immunoglobulins MEMORY disorders DEMENTIA DRUG therapy CRANIOPHARYNGIOMA COMPUTED tomography ANGER CEREBROSPINAL fluid SEIZURES (Medicine) RARE diseases DISEASE complications
Zdroj:	Critical Care & Shock; 2023, Vol. 26 Issue 1, p33-37, 5p
Abstrakt:	New onset refractory status epilepticus (NORSE) is a clinical presentation in patients, not a specific diagnosis characterized by the occurrence of a prolonged period of refractory seizures. NORSE is the presenting clinical feature in some patients with paraneoplastic etiology. Paraneoplastic limbic encephalitis (PLE) is a rare disorder of the nervous system associated with malignant disease. It presents as a subacute disease with symptoms like seizures, cognitive dysfunction, irritability, hallucination, and short-term memory loss. In our study, we reported a case of a 30- year-old woman with PLE associated with craniopharyngioma and serum anti-Hu antibodies. The patient presented with NORSE. Initial therapy with antiepileptic drugs failed to control seizures. The patient presented with short-term or recent memory loss, cognitive dysfunction, and behavioral changes (anger/apathy) for the last three months, suggesting a rapidly progressing dementia. Baseline investigations (blood counts, renal function, thyroid function, liver function, serum B12/folate level, serum cortisol, and urinalysis) were normal, and she had no background comorbidities. Computerized tomography (CT) scan and magnetic resonance imaging (MRI) revealed a sellar/suprasellar lesion suggestive of craniopharyngioma. The tumor was successfully removed and repeat imaging after surgery was clear and did not result in further seizures. Now the patient is on follow-up with tapering doses of prednisolone. This is a rare condition; we treated our patient successfully via surgical resection and postoperative chemotherapy. Also, we considered that her new onset refractory epilepsy was associated with her craniopharyngioma. Hence a high degree of suspicion is required, which can be guided by clinical findings and imaging. [ABSTRACT FROM AUTHOR]
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