School of Medicine and Health Sciences Poster Presentations

Will She Recover? A Prolonged Hospitalization for a Comatose Female with Neuropsychiatric Lupus

Poster Number

179

Document Type

Poster

Publication Date

3-2016

Abstract

25 year old African-American woman was brought into the ED by EMS after she was found unresponsive in her apartment. Police found the patient to be unconscious and unable to be aroused, lying in feces and urine, with no witnesses or medical history available. In the ED, physical exam showed an obtunded female with GCS of 6T. Neurological exam revealed a left eye gaze with horizontal nystagmus, absent Babinski reflex, 3+ reflexes in upper extremity, and 4+ reflexes in lower extremity bilaterally. CT head did not demonstrate acute hemorrhage or masses.

The patient was intubated to protect her and admitted to the ICU. She was started on empiric therapy for seizure with levetiracetam and for meningoencephalitis with ceftriaxone, vancomycin, and acyclovir. Lumbar puncture revealed no infection, and urine drug screen was normal. Brain MRI showed diffusion along the posterior frontal, parietal and temporal cortices bilaterally with diffuse pachymeningeal enhancement concerning for an autoimmune process.

Using the regional online health information exchange, we discovered that the patient had been diagnosed with SLE at a nearby hospital, two weeks prior to this admission. She had been started on steroids and hydroxychloroquine, and was discharged with plans for rheumatology follow-up. However, she missed that appointment, and medication compliance was unknown. Labs revealed a mixed lupus and Sjogren’s picture, with evidence of renal and hematologic involvement of her SLE.

This patient was treated with levetiracetam, lacosamide, and pregabalin for seizures, and diagnostic EEG was performed. She was also started on pulse dose steroids and cyclophosphamide for SLE. Her neurologic status remained unimproved and her ICU course was marked by multiple complications, including massive hemoptysis at her tracheostomy site, prolonged seizures, and anoxia. Her chance of recovery was believed to be very poor given intensity of her condition. She was transferred to the medical floor on day 26 after passing tracheostomy collar trial, but she continued to have no purposeful movements.

Over the next two months, she slowly regained neurological function in her extremities, and was discharged to inpatient rehabilitation on oral prednisone and lacosamide. She returned home, and in the rheumatology clinic one month after discharge, had regained full neurological function. She began her third dose of cyclophosphamide and was continued on her seizure medications, and returned to work. She continues to follow with rheumatology and neurology.

SLE affects the neurological system by affecting inflammatory, vasculitic, thromboembolic, and meningeal pathways through a series of complex mechanisms. As a result, patient may experience a range of symptoms including stroke, seizures, cognitive dysfunction, and headaches. Risk factors for neuropsychiatric SLE include the presence of antiphospholipid antibodies, nephritis, young age, and African American ethnicity.

Recognizing neuropsychiatric manifestations of SLE are important as management involves treating both the neurological symptoms and the underlying SLE. Symptoms of neuropsychiatric SLE should be evaluated and treated just as in patients without lupus. Our patient presented with a likely seizure, and was treated empirically for seizure. Lupus patients with stroke should be treated as any other stroke patients. Airway, breathing, and circulation should always be assessed first when a patient presents with severe neurological symptoms. Once the patient has been stabilized and started on appropriate neurological treatments, SLE treatments such as pulse dose steroids should be initiated to help recover from the underlying disease. Neurological symptoms may persist longer in SLE patients, leading to a long and difficult hospital course. Our patient was initially thought to have a very low chance of regaining neurological function. Despite these concerns, she recovered fully after 68 hospital days and an additional 16 days in inpatient rehab. Because neuropsychiatric symptoms of SLE are often indistinguishable from non-SLE neurological symptoms, diagnosis can be difficult, requiring strong clinical suspicion and accurate medical history.

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Presented at: GW Research Days 2016

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Will She Recover? A Prolonged Hospitalization for a Comatose Female with Neuropsychiatric Lupus

25 year old African-American woman was brought into the ED by EMS after she was found unresponsive in her apartment. Police found the patient to be unconscious and unable to be aroused, lying in feces and urine, with no witnesses or medical history available. In the ED, physical exam showed an obtunded female with GCS of 6T. Neurological exam revealed a left eye gaze with horizontal nystagmus, absent Babinski reflex, 3+ reflexes in upper extremity, and 4+ reflexes in lower extremity bilaterally. CT head did not demonstrate acute hemorrhage or masses.

The patient was intubated to protect her and admitted to the ICU. She was started on empiric therapy for seizure with levetiracetam and for meningoencephalitis with ceftriaxone, vancomycin, and acyclovir. Lumbar puncture revealed no infection, and urine drug screen was normal. Brain MRI showed diffusion along the posterior frontal, parietal and temporal cortices bilaterally with diffuse pachymeningeal enhancement concerning for an autoimmune process.

Using the regional online health information exchange, we discovered that the patient had been diagnosed with SLE at a nearby hospital, two weeks prior to this admission. She had been started on steroids and hydroxychloroquine, and was discharged with plans for rheumatology follow-up. However, she missed that appointment, and medication compliance was unknown. Labs revealed a mixed lupus and Sjogren’s picture, with evidence of renal and hematologic involvement of her SLE.

This patient was treated with levetiracetam, lacosamide, and pregabalin for seizures, and diagnostic EEG was performed. She was also started on pulse dose steroids and cyclophosphamide for SLE. Her neurologic status remained unimproved and her ICU course was marked by multiple complications, including massive hemoptysis at her tracheostomy site, prolonged seizures, and anoxia. Her chance of recovery was believed to be very poor given intensity of her condition. She was transferred to the medical floor on day 26 after passing tracheostomy collar trial, but she continued to have no purposeful movements.

Over the next two months, she slowly regained neurological function in her extremities, and was discharged to inpatient rehabilitation on oral prednisone and lacosamide. She returned home, and in the rheumatology clinic one month after discharge, had regained full neurological function. She began her third dose of cyclophosphamide and was continued on her seizure medications, and returned to work. She continues to follow with rheumatology and neurology.

SLE affects the neurological system by affecting inflammatory, vasculitic, thromboembolic, and meningeal pathways through a series of complex mechanisms. As a result, patient may experience a range of symptoms including stroke, seizures, cognitive dysfunction, and headaches. Risk factors for neuropsychiatric SLE include the presence of antiphospholipid antibodies, nephritis, young age, and African American ethnicity.

Recognizing neuropsychiatric manifestations of SLE are important as management involves treating both the neurological symptoms and the underlying SLE. Symptoms of neuropsychiatric SLE should be evaluated and treated just as in patients without lupus. Our patient presented with a likely seizure, and was treated empirically for seizure. Lupus patients with stroke should be treated as any other stroke patients. Airway, breathing, and circulation should always be assessed first when a patient presents with severe neurological symptoms. Once the patient has been stabilized and started on appropriate neurological treatments, SLE treatments such as pulse dose steroids should be initiated to help recover from the underlying disease. Neurological symptoms may persist longer in SLE patients, leading to a long and difficult hospital course. Our patient was initially thought to have a very low chance of regaining neurological function. Despite these concerns, she recovered fully after 68 hospital days and an additional 16 days in inpatient rehab. Because neuropsychiatric symptoms of SLE are often indistinguishable from non-SLE neurological symptoms, diagnosis can be difficult, requiring strong clinical suspicion and accurate medical history.