ABSTRACT
Objective: We present three patients with insidious onset of high-frequency atypical seizures, in association with atypical autoantibodies, with significant improvement with immunotherapy. Background: Autoimmune epilepsy (AE) is a relatively newly discovered epilepsy etiology. Although, It was described to present as New-Onset Refractory Status Epilepticus (NORSE). Our understanding of the clinical presentations and autoantibodies linked to AE is still sparse. Design/Methods: Case Series Case 1: A 44-year-old man presented with more than 10 years of recurrent episodes of mild confusion. Patient presented to our ER during one of these episodes where EEG revealed right temporal lobe status epilepticus. He had suboptimal response to multiple Antiepileptic Drugs (AEDs). MRI brain showed T2/FLAIR hyperintensities in the right frontal, parietal, and temporal lobes consistent with postictal effect. CSF was positive Neuronal Intermediate Filament (NIF) heavy chain antibodies Treatment with plasmapheresis (PLEX) and intravenous immunoglobulin (IVIG) with a good response. Case 2: A 73-year-old woman presented with daily episodes of mild confusion and falls over few months. EEG was consistent with frontal lobe seizures. MRI brain and CSF were unremarkable. She was treated with multiple AEDs, without adequate control. Serum paraneoplastic panel was positive for voltage-gated potassium channel antibodies. Seizures were controlled with PLEX. Case 3: A 22-year-old woman presented with daily episodes of behavioral arrest and confusion few weeks after COVID-19 vaccination. EEG showed bitemporal seizures, refractory to AEDs, requiring pentobarbital induced coma. CSF and MRI brain were unremarkable. Thyroid peroxidase and anti-thyrotropin antibodies were highly elevated. Treatment with IVIG and PLEX for AE, with a prolonged recovery. Conclusions: Seizures associated with AE appear to be trivial;however, it can have an aggressive course. Among antibodies have been reported in AE, NIF antibodies has not been reported. AE should be considered in patients with High-frequency of atypical seizures. Early initiation of immunotherapy is the key for disease control.
ABSTRACT
Objective: To present two patients with COVID-19 and diffuse myoclonic disorder. Background: Neurological manifestations associated with COVID-19 are being increasingly reported in the literature. However, diffuse myoclonic disorder associated with COVID-19 is rarely reported. Design/Methods: NA Results: Two young women, 22 and 33 years old, presented with uncontrollable muscle jerks involving the torso, neck, and extremities;dystonic posturing in the extremities or neck;and anosmia. Both were diagnosed with COVID-19 three to four weeks before presentation. However, the 22-year-old remained positive and the 33-year-old was negative at presentation. Their myoclonus was triggered by voice and worsened with intention and action but decreased while laying quietly and stopped during sleep. Both were taking psychoactive medications for years without side effects. Strength, reflexes, and plantar response were normal, as were labs. CSF analysis was normal for the 33-year-old. LP was not performed in the 22-year-old due to increased risk of CSF leaks in patients with Ehlers Danlos syndrome. Non-contrast MRI brain was normal in both. All medications with potential interactions were discontinued. Both were given IV fluids and cyproheptadine without improvement. A hyper-serotonergic state and all other causes of myoclonus were ruled out. Both were started on high dose intravenous methylprednisolone, clonazepam, and IVIG. The 33-year-old exhibited dramatic resolution of her myoclonus by the end of the treatment. The 22-year-old developed bradycardia, prompting discontinuation of methylprednisolone and clonazepam. She had modest improvement after completing IVIG but by discharge had near complete resolution of her myoclonus. Conclusions: Diffuse myoclonic disorder should be recognized as a potential neurological complication of COVID-19. We suggest the name, COVID-19 Associated Diffuse Myoclonus and Dystonia (CADMAD) syndrome. Given the prompt response to immune-based therapy, it is hypothesized this syndrome could be a para-infectious or post-infectious immune-mediated disorder. Our report adds to the available literature to help with understanding and treatment of this disease.