Agrypnia excitata as the main feature in anti-leucine-rich glioma-inactivated 1 encephalitis: a detailed clinical and polysomnographic semiological analysis.

BACKGROUND AND PURPOSE: The core manifestations of leucine-rich glioma-inactivated 1 (LGI1) autoantibody-mediated encephalitis are limbic encephalitis and faciobrachial dystonic seizures. Agrypnia excitata (AE) is a rare syndrome characterized by sleep-wake cycle disruption, autonomic hyperactivation and episodes of oneiric stupor. Only a few diseases are known to present with AE. An autoimmune etiology must be considered when accompanied by neuromyotonia. A case of anti-LGI1 encephalitis presenting with AE is reported. METHODS: Detailed clinical, video-polysomnographic, laboratory, radiological and long-term follow-up assessments were performed. RESULTS: A previously healthy 58-year-old man was referred for a rapidly progressive change in mental status, characterized by persistent drowsiness and confusion, accompanied by frequent episodes of unconscious gestures ranging from simple stereotyped movements to more complex actions mimicking various daily activities. Other symptoms included tachycardia, hyperhidrosis, mild hyponatremia, rare faciobrachial dystonic seizures, and a single generalized tonic-clonic seizure, but no neuromyotonia. Prolonged video-polysomnography excluded epileptic activity and showed continuous monomorphic slowing of background activity not consistent with a regular wakefulness or sleep state. A brain magnetic resonance imaging scan was unremarkable. Brain fluorodeoxyglucose positron emission tomography revealed hypermetabolism of the hippocampi, amygdala and basal ganglia. Anti-LGI1 antibodies were detected in the cerebrospinal fluid. The sleep disorder resolved progressively after starting immunotherapy. CONCLUSIONS: Agrypnia excitata can be a dominant, treatable manifestation of anti-LGI1 encephalitis. Oneiric stupor episodes are a useful clinical feature for establishing diagnostic suspicion and could provide a window to understanding the mechanisms behind some movement disorders in autoimmune encephalitis.

Neurocysticercosis-related seizures in the post-partum period: two cases and a review of the literature.

Neurocysticercosis, the infection of the CNS with larval cysts of Taenia solium, is a leading cause of seizures in low-income countries. The clinical presentation of neurocysticercosis is variable and depends on the number, size, and location of cysticerci, and on the immune response of the host. In most patients, the affected site is the brain parenchyma, where cysts can precipitate seizures. Neurocysticercosis has seldom been described in pregnant women. In this Grand Round, we report two cases of pregnant women who immigrated to Italy from Bolivia and Ecuador, and who developed seizures in the early post-partum period, due to calcified parenchymal neurocysticercosis lesions. We discuss the complex interactions between neurocysticercosis and the immune system in pregnancy and the post-partum period. Building on this scenario, we propose practices for the management of neurocysticercosis in pregnancy and the post-partum period, highlighting important gaps in the literature that should be addressed.

Carotid stenosis as predictor of stroke after transient ischemic attacks.

ABCD2 score identifies high-risk TIAs but its validity in different countries and hospitals is unknown. Doubts remain also about the role of diagnostic work up for patients with TIAs in the emergency department. The present study was undertaken to confirm the usefulness of ABCD2 score in the emergency department of Trento Hospital and to evaluate if other exams (carotid ultrasound or CT scan) commonly performed in TIA patients are helpful. We retrospectively analysed discharge diagnosis of around 120,000 patients seen at the first aid of Trento Hospital over a 28 month period. ABCD2 score, carotid ultrasound and CT scan were recorded and were correlated with recurrence of stroke at different time points (mean follow-up period of 11.4 months) in all patients with TIA. We identified 965 patients with focal neurologic deficit and 502 could be classified as TIA. An ischemic stroke recurred in 30 patients at the end of the follow-up (30% in the first two days). ABCD2 score confirmed its value. A significant carotid stenosis (>70%) was an independent risk factor for stroke at any time point. Our study confirms the role of ABCD2 in a large Italian cohort of TIA patients but also suggests the importance of performing a carotid ultrasound as soon as possible.