We describe an adolescent girl with non-paraneoplastic anti-NMDA-receptor encephalitis (ANMDARE), who despite persistence of the extreme delta brush (EDB) pattern for nearly 2 years in her serial EEGs, she exhibited a speedy and sustained response to immunotherapy. To the best of our knowledge, our patient had the longest persistence of the EDB pattern on EEG reported to date. Our patient illustrates that, although presence of EDB supports the diagnosis of ANMDARE, its presence and persistence may not be a reliable predictor of response to immunotherapy and overall clinical prognosis.
A 14‐year‐old boy presented with a prodromal respiratory infection followed by super refractory status epilepticus. A diagnosis of Febrile Infection‐Related Epilepsy Syndrome (FIRES) was made. Initial MRI study and CSF analysis were normal. He required multiple anticonvulsants owing to the refractory nature of the seizures. The course of the illness was rather stormy, laced with various medical problems viz. hepatic dysfunction, sepsis, hemodynamic, and hematological abnormalities which posed several challenges in the management. Hemophagocytic lymphocytic histiocytosis (HLH) was identified as the etiology of the illness and was treated but without success. The case report highlights the several immunomodulatory strategies that were employed to treat the disease, despite which the outcome was unfavorable.
Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyperinflammatory hematological syndrome characterized by cytokine storm and the diagnosis can be missed as it mimics sepsis or multi-organ dysfunction syndrome. Case Report: A 15-year old adolescent girl presented with six-day history of high grade intermittent fever with easy fatigability and dyspnea on exertion. She was found to have severe anemia elsewhere (Hemoglobin- 4 g/dl, normal 11-16.5 g/dl) and referred to our hospital due to persistent fever, tiredness and recurrent vomiting. On examination she was pale and febrile. Her blood investigations revealed moderate anemia, leukopenia and markedly elevated liver transaminases with coagulopathy. Dengue NS1 antigen and IgM antibodies were positive suggestive of acute dengue infection. Further workup revealed markedly elevated serum ferritin (37973 ng/ml, normal 10-120 ng/ml) and lactate dehydrogenase levels (5311 U/L, normal 180-360 U/L). Plasma fibrinogen level was low (164 mg/dl, normal 200-400 mg/dl). Bone marrow biopsy done on day 3 revealed hemophagocytosis. Serum TSH was mildly elevated (6.5 mU/L, normal 3.5-5 mU/L) and anti-thyroid peroxidase (TPO) antibodies (68 IU/ml, normal <5.6 IU/ml) were also significantly elevated. Diagnosis of Hemophagocytic lymphohistiocytosis (HLH) secondary to dengue infection and autoimmune thyroiditis was made. She was treated with dexamethasone 10 mg/m2 /day from day 3 of admission along with supportive treatment. She rapidly improved and her laboratory parameters returned back to normal at 1 week of follow up. Conclusion: Dengue fever with unexplained severe anemia in the absence of bleeding should be evaluated for HLH as timely initiation of treatment will be life saving
Aims
. Differentiation between syncope secondary to epileptic seizures and cardiac disease in patients displaying transient loss of consciousness associated with convulsive movements is a diagnostic challenge both for neurologists and cardiologists. In such patients, prolonged video‐EEG monitoring not only helps in identifying asystole as the cause of syncope, but also in categorizing asystole as primarily cardiac in origin (cardiac asystole) and secondary to epileptic seizures (ictal asystole). We carried out this study to ascertain the prevalence of asystole in an epilepsy monitoring unit, and to contrast the clinical and electrophysiological characteristics between ictal asystole and cardiac asystole.
Methods
. Through a retrospective search, we identified patients who were shown to have had asystole using a database of patients who underwent prolonged video‐EEG monitoring during a 68‐month period. We compared the data of 18 consecutive patients; five with ictal asystole and 13 with cardiac asystole, with 121 and 64 events recorded from them, respectively.
Results
. Of the 10,096 patients who underwent prolonged video‐EEG monitoring during the study period, we identified 18 (0.17%) patients with asystole. Cardiac asystole was 2.6 times more frequent than ictal asystole. Older age at onset, heralding symptoms of presyncope, occurrence during wakefulness, and brief duration of the events supported the diagnosis of cardiac asystole. Ictal asystole events were more protracted, and prolonged asystole more frequently occurred in patients with extratemporal seizures compared to temporal lobe seizures. Asystole occurred in only half of the recorded seizures.
Conclusions
. The accurate categorization of asystole as seizure‐related or heart disease‐related has huge implications for management strategy and outcome. The necessity of permanent pacemaker implantation is more frequent and urgent in patients with cardiac asystole because of the greater risk of sudden death. Hence, in patients with an ominous diagnosis of cardiac asystole, a thorough cardiac evaluation should surpass neurological evaluation.
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