Rebelee C. Jeyasingh
Memorial Healthcare System, USAPresentation Title:
Rhombencephalitis: A case report
Abstract
Background:
Prompt diagnosis of neurological disorders is important as it helps with early intervention, prevents long-term disablement and improves quality of life. Rhombencephalitis, also called brainstem-encephalitis, is the inflammation of the brainstem and cerebellum. Common causes are autoimmune, infection, and paraneoplastic conditions.
Design/ Methods: Case report
Case presentation
A 65-year-old right-handed Brazilian female with no reported history of systemic illnesses admitted with chief complaints of new onset diplopia associated with dizziness, left CN VI palsy, and facial numbness. Patient had an MRI which was negative, and she was discharged. She also had a reported outpatient ophthalmology evaluation which was felt to be "unremarkable". A few days later she presented with worsening symptoms as well as gait disturbance/imbalance. Repeat Brain MRI at that time described some brainstem hyperintensities, an LP was obtained, revealing some lymphocytic pleocytosis. The patient was initiated on empiric antibiotics. Routine cultures and encephalitis panel had been negative. Mental status began to worsen requiring intubation. At the time, the patient received IVIG x 6, as well as high-dose steroids x days. The patient improved to the point that she was extubated but had to be reintubated after a few days as she had a declining respiratory status. The patient continued medical management. Evaluated by infectious disease, who feel patient has an extensive differential, including infectious etiology such as abscess, Listeria, toxoplasmosis, MTB, bartonellosis, fungal infections, Bickerstaff, or even autoimmune. The patient got treated with empiric antituberculous therapy (RIPE), and broad-spectrum antibiotics (vancomycin, Merrem, ampicillin). The patient was also seen by neurology service who noticed persistent pleocytosis on CSF analysis. Received a second course of IVIG (x 5 days), as well as a 3-day course of methylprednisolone. The patient was successfully extubated, and began to show clinical improvement, able to speak, feed herself, and moving her extremities more consistently. Repeat encephalitis panel including cryptococcus, Listeria, and HSV 1 have been negative to date, VDRL also negative. Hospital course also notable for some transaminitis; at this point INH and rifampin have been discontinued (low suspicion for TB in setting of no fevers, Karius test did not identify any microorganisms). Working diagnosis: Rhombencephalitis of unclear etiology. NMO was positive. ANA, anti-Jo antibody IgG negative; cytology negative for malignancy, oligoclonal band positive, zinc and copper WNL. Repeat MRI from continues to describe some abnormal signal intensity and enhancement in the brainstem, which may reflect changes of marked spectrum demyelinating disease versus autoimmune versus infectious encephalitis versus chronic lymphocytic inflammation with pontine perivascular enhancement; infiltrative neoplastic process such as angiocentric lymphoma and granulomatous disease as well as vascular inflammatory diseases may have similar appearance. Patient's hospital course has been notable for overall improvement in cognitive/communication function; there is some mild proximal lower extremity weakness/deconditioning, and persistent but improving perioral and facial numbness. Patient initiated on prednisone taper (60 mg with 2-week taper of 5 mg). Patient seen by speech pathologist, noticing some expressive/receptive language deficits, mild dysarthria versus apraxia, and moderate oropharyngeal dysphagia. Initiated rehabilitation efforts in the acute hospital floor then admitted to the Acute Inpatient Rehabilitation Institute improve ADL's and mobility while allowing for close medical supervision. Her symptoms gradually improved. Recommendation for outpatient follow-up neuro immunology) in 1 to 2 weeks post discharge.
Conclusion
This case illustrates the importance of a complete history, detailed exam, diagnostic evaluation and treatment plan. Although this neurological disorder seen in this patient is rare, it is important to know the different conditions and their management. Recognition of this condition is vital to the health care providers to provide appropriate therapy and prevention of disabilities by early treatment and rehabilitation.
Prompt diagnosis of neurological disorders is important as it helps with early intervention, prevents long-term disablement and improves quality of life. Rhombencephalitis, also called brainstem-encephalitis, is the inflammation of the brainstem and cerebellum. Common causes are autoimmune, infection, and paraneoplastic conditions.
Design/ Methods: Case report
Case presentation
A 65-year-old right-handed Brazilian female with no reported history of systemic illnesses admitted with chief complaints of new onset diplopia associated with dizziness, left CN VI palsy, and facial numbness. Patient had an MRI which was negative, and she was discharged. She also had a reported outpatient ophthalmology evaluation which was felt to be "unremarkable". A few days later she presented with worsening symptoms as well as gait disturbance/imbalance. Repeat Brain MRI at that time described some brainstem hyperintensities, an LP was obtained, revealing some lymphocytic pleocytosis. The patient was initiated on empiric antibiotics. Routine cultures and encephalitis panel had been negative. Mental status began to worsen requiring intubation. At the time, the patient received IVIG x 6, as well as high-dose steroids x days. The patient improved to the point that she was extubated but had to be reintubated after a few days as she had a declining respiratory status. The patient continued medical management. Evaluated by infectious disease, who feel patient has an extensive differential, including infectious etiology such as abscess, Listeria, toxoplasmosis, MTB, bartonellosis, fungal infections, Bickerstaff, or even autoimmune. The patient got treated with empiric antituberculous therapy (RIPE), and broad-spectrum antibiotics (vancomycin, Merrem, ampicillin). The patient was also seen by neurology service who noticed persistent pleocytosis on CSF analysis. Received a second course of IVIG (x 5 days), as well as a 3-day course of methylprednisolone. The patient was successfully extubated, and began to show clinical improvement, able to speak, feed herself, and moving her extremities more consistently. Repeat encephalitis panel including cryptococcus, Listeria, and HSV 1 have been negative to date, VDRL also negative. Hospital course also notable for some transaminitis; at this point INH and rifampin have been discontinued (low suspicion for TB in setting of no fevers, Karius test did not identify any microorganisms). Working diagnosis: Rhombencephalitis of unclear etiology. NMO was positive. ANA, anti-Jo antibody IgG negative; cytology negative for malignancy, oligoclonal band positive, zinc and copper WNL. Repeat MRI from continues to describe some abnormal signal intensity and enhancement in the brainstem, which may reflect changes of marked spectrum demyelinating disease versus autoimmune versus infectious encephalitis versus chronic lymphocytic inflammation with pontine perivascular enhancement; infiltrative neoplastic process such as angiocentric lymphoma and granulomatous disease as well as vascular inflammatory diseases may have similar appearance. Patient's hospital course has been notable for overall improvement in cognitive/communication function; there is some mild proximal lower extremity weakness/deconditioning, and persistent but improving perioral and facial numbness. Patient initiated on prednisone taper (60 mg with 2-week taper of 5 mg). Patient seen by speech pathologist, noticing some expressive/receptive language deficits, mild dysarthria versus apraxia, and moderate oropharyngeal dysphagia. Initiated rehabilitation efforts in the acute hospital floor then admitted to the Acute Inpatient Rehabilitation Institute improve ADL's and mobility while allowing for close medical supervision. Her symptoms gradually improved. Recommendation for outpatient follow-up neuro immunology) in 1 to 2 weeks post discharge.
Conclusion
This case illustrates the importance of a complete history, detailed exam, diagnostic evaluation and treatment plan. Although this neurological disorder seen in this patient is rare, it is important to know the different conditions and their management. Recognition of this condition is vital to the health care providers to provide appropriate therapy and prevention of disabilities by early treatment and rehabilitation.
Biography
Rebelee C Jeyasingh is a board-certified Adult Nurse Practitioner. She received her bachelor’s degree in nursing from Kovai Medical Center and Hospital, Coimbatore, South India, received her master’s in nursing from Christian Medical College, South India, following that graduated as a Nurse Practitioner from Florida International University in 2012. She completed her Doctorate in Nursing Practice from Florida International University in December 2022. As a nurse and nurse practitioner, Rebelee has worked in various inpatient and outpatient settings, including cardiac and neuro ICU, stroke, and oncology units. Currently work as a Nurse Practitioner in Physical Medicine and Rehabilitation. Rebelee is a huge proponent of patient education and enjoys teaching her patients about ways to improve their health.