A Curious Case of Convulsions Dhishna Chaudhary MD, Ramez Morcos MD, - PowerPoint PPT Presentation

A Curious Case of Convulsions Dhishna Chaudhary MD, Ramez Morcos MD, Mishah Azhar MD, John Malcynski MD, Patricio S. Espinosa MD Florida Atlantic University Charles E. Schmidt College of Medicine

Case Presentation at Bethesda • A 30-year-old Caucasian male with recent genital herpes infection (on valacyclovir) was brought to the hospital via EMS after having a new onset seizure at the mall. Apparently had neck pain but no fevers x 1 week • PMHx: Being treated for genital herpes for last 1 week • PSHx: No previous surgeries • Social Hx: Used cannabis oil via vaporizer. No smoking/alcohol use • Home MDx: Valacyclovir x 1 week • FHx: No hx of neurological disorders • Allergies: None In the ED: Day 1 of Presentation • Vitals normal • Physical exam: Normal except patient severely agitated and combative • Labs pertinent for WBC 24,000 (75% segs), elevated CPK, elevated Cr, and metabolic acidosis • Had another tonic-clonic seizure that was terminated via lorazepam (Ativan) 6mg IV and patient was loaded with levetiracetam (Keppra) 1000mg IV x1 • He was intubated for airway protection and transferred to the medical ICU

Case Presentation at Bethesda In the Medical ICU: Day 1 of Presentation • Valacyclovir discontinued • Started on levetiracetam 500mg IV BID and Ativan drip • CT brain w/o contrast: No evidence of an acute infarct, intracranial mass, hemorrhage or extra-axial fluid collection • LP done and removed 4mL of clear CSF • UA negative, ESR 8, ammonia 30, complement C3/complement C4/ANA/ANCA/dsDNA normal • HIV1/2, RPR, blood and urine cx, vit B12, folate levels normal • TSH 1.620, free T4 0.55 • UDS was positive for THC (patient used cannabis oil vaporizer). Differential Diagnoses: • HSV encephalitis or bacterial meningitis • Valacyclovir toxicity • Marijuana induced encephalopathy • Started on dexamethasone, ceftriaxone, vancomycin and acyclovir

Case Presentation at Bethesda In the Medical ICU: Day 2 of Presentation • Seizure around midnight  Ativan 1mg IV x 1 • On Ativan and propofol drips, Keppra 1000mg IV BID • LP results  CSF WBC 0, RBC 93, glucose 89, protein 77 • CSF fungus and bacterial cx negative • CSF cryptococcal Ag/ CSF HSV/ CSF VDRL all negative • Ceftriaxone, vancomycin, acyclovir and dexamethasone discontinued • EEG: There is evidence of marked diffuse symmetrical slowing that is non specific and can be seen in sedation and encephalopathy. There is no evidence for epileptiform abnormalities seen on the tracing. There is evidence of triphasic waves. There is muscle artifact. • At 5pm, another 15 minutes tonic-clonic seizure on Ativan drip Differential Diagnoses: • Valacyclovir toxicity • Marijuana induced encephalopathy

Case Presentation at Bethesda In the Medical ICU: Day 3 of Presentation • On Ativan and propofol drips, Keppra 1000mg IV BID • At 9AM, patient had 10 minute episode of seizure  phenobarbital 2g IV x1 and phenytoin 100mg IV q8h, lacosamide 200mg IV BID initiated • MRI brain w/o contrast: Unremarkable • EEG: There is no evidence for epileptiform abnormalities seen on the tracing. Abnormal EEG for age secondary to marked diffuse symmetrical slowing which is nonspecific. In the Medical ICU: Day 4 of Presentation • On Ativan and propofol drips, Keppra 1000mg IV BID, phenytoin 100mg IV q8h, lacosamide 200mg IV BID • Started weaning off Ativan drip • Decerebrate posturing noted • Transferred to BRRH for continuous video EEG • EEG: There is evidence of marked diffuse symmetrical slowing that is nonspecific and can be seen in sedation and encephalopathy. There is no evidence for epileptiform abnormalities seen on the tracing. Abnormal EEG for age secondary to marked diffuse symmetrical slowing which is nonspecific.

Case Presentation at BRRH In the Neuroscience ICU: Day 4 of Presentation • On propofol and fentanyl drips, Keppra 1000mg IV BID • Discontinued phenytoin • Video EEG initiated (day 1) • No seizures noted • EEG: The best waking record showed fair organization at rest consistent of 7-8 Hz posterior activity with good reactivity, there was moderate beta activity bilaterally. There was bilateral slowing wide distribution at times rhythmic. However he was not epileptiform. In the Neuroscience ICU: Day 5 of Presentation • On Keppra 1000mg IV BID, Propofol and fentanyl drips weaned off • Discontinued lacosamide • Started precedex drip • Video EEG continued (day 2) • Paraneoplastic panel sent along with thyroid antibodies (anti-thyroid globulin and thyroid peroxidase antibodies) • No seizures noted • EEG: The walking and sleeping background was similar to the prior day.

Case Presentation at BRRH In the Neuroscience ICU: Day 6 of Presentation • On precedex drip, Keppra 1000mg IV BID • Severely agitated in the morning and both Propofol and fentanyl drips restarted • Discontinued phenytoin • Video EEG initiated (day 3) • CT chest/abdomen/pelvis w/o contrast done to rule out cancer given concerns of paraneoplastic syndrome • Methylprednisolone 500mg IV BID started (day 1 of 5) • No seizures noted • EEG: The walking and sleeping background was similar to the prior day. Differential Diagnoses: • Paraneoplastic limbic (autoimmune) encephalitis • Non-paraneoplastic limbic encephalitis

Case Presentation at BRRH In the Neuroscience ICU: Day 7 of Presentation • On Keppra 1000mg IV BID • Fentanyl and Propofol drips discontinued and patient extubated successfully • IVIG 0.4g/kg/day initiated (day 1 of 5) • Continued with methylprednisolone 500mg IV BID (day 2 of 5) • No seizures noted • EEG: The walking and sleeping background was similar to the prior day. There was improvement of the background. There was less bilateral slowing. Differential Diagnoses: • Non-paraneoplastic limbic encephalitis

Case Presentation at BRRH In the Neuroscience ICU: Day 8 of Presentation • On Keppra 1000mg IV BID • Continued with IVIG 0.4g/kg/day (day 2 of 5) • Continued with methylprednisolone 500mg IV BID (day 3 of 5) • Anti-thyroid globulin antibodies 23.1 IU/mL • Thyroid peroxidase antibodies 0.5 IU/mL • No seizures noted • EEG: The walking and sleeping background was similar to the prior day. The background of the EEG improved significantly to a normal EEG background. In the Neuroscience ICU: Day 9 of Presentation • On Keppra 1000mg IV BID • Continued with IVIG 0.4g/kg/day (day 3 of 5) • Continued with methylprednisolone 500mg IV BID (day 4 of 5) • MRI brain w/ and w/o contrast: unremarkable • Patient walking and no longer dysarthric • No seizures noted  video EEG discontinued

Case Presentation at BRRH In the Neuroscience ICU: Day 10 of Presentation • On Keppra 1000mg IV BID • Continued with IVIG 0.4g/kg/day (day 4 of 5) • Continued with methylprednisolone 500mg IV BID (day 5 of 5) • Paraneoplastic panel negative • No seizures noted In the Neuroscience ICU: Day 11 of Presentation • Decreased Keppra from 1000mg IV BID to 500mg IV BID • Continued with IVIG 0.4g/kg/day (day 5 of 5) • Discontinued methylprednisolone 500mg IV BID • Thyroid US negative • Patient discharged on Keppra 500mg IV BID Outpatient Follow Up • Patient continued Keppra 500mg IV BID and had IVIG every 3 weeks for total 3 months • Most recent anti-thyroid globulin level was 39 IU/mL and thyroid peroxidase level was 31 IU/mL

Discussion • Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) presents with nonspecific symptoms of confusion, seizures and stroke- like episodes. • Also known as Hashimoto’s encephalopathy (HE) • Characterized by high titers of anti-thyroid antibodies and is responsive to steroids. • SREAT is poorly recognized and often misdiagnosed. • The mechanism of SREAT or HE is unknown. • Most patients present in an euthyroid state and the levels of antithyroid antibodies do not correlate with neurologic symptoms or treatment duration. • Some propose that HE may result from a direct antibody-mediated neuronal injury or from immune complex deposition in the cerebral microvasculature. • Rapid response to steroid therapy within hours to days is well documented.

Conclusions • Suspicion of SREAT must be high in those patients with new onset seizures whose workup has been negative. • Although, ordering an autoimmune panel early is vital, initiation of treatment with steroids and possibly IVIG should not be delayed.

Thank you Thank you to Dr.Espinosa and the MNI team for giving me the opportunity to present this case report.