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Poster 225 Bickerstaff’s Encephalitis with an Exemplary Recovery Following Rehabilitation: A Case Report
Abstracts / PM R 8 (2016) S151-S332 surgical candidacy. Lower extremity EMG demonstrated sensorimotor demyelinating and axonal polyneuropathy. Setting: Tertiary care hospital. Results: Three days after admission to acute rehabilitation, patient demonstrated increased strength and endurance. Patient able to ambulate at 7 days. Pain and glycemic control were managed. Clinical findings and improvement with conservative management demonstrates a likely diagnosis of diabetic amyotrophy. Discussion: This was an atypical presentation of diabetic amyotrophy. Weakness also involved bilateral scapulohumeral muscles, and patient had an acute presentation despite adequate glycemic control for 6 months. Conclusions: Glycemic control is not solely adequate in delaying or preventing the onset of diabetic amyotrophy. Level of Evidence: Level V Poster 223 Monomelic Peripheral Neuropathy after Exposure to Cold Weather in a Diplegic Cerebral Palsy Chong Tae Kim, MD, PhD (The Children’s Hospital of Philadelphia, Philadelphia, PA, United States) Disclosures: Chong Tae Kim: I Have No Relevant Financial Relationships To Disclose Case/Program Description: The patient is a 17-year-old male with diplegic cerebral palsy (Gross Motor Functional Classification System), but a very active snow boarding athlete. He acutely, newly developed sensory loss and weakness of the right lower leg after seven hours of snow boarding practice at an outdoor slope in the last January. The exact temperature at that time was not obtainable, but reportedly very cold. He realized the symptoms developed two to three days after the practice. Three weeks after the symptom development, he was referred to the rehabilitation outpatient clinic. He denied any improvement of the symptoms at the clinic visit. Setting: Academic Children’s Hospital outpatient clinic. Results: Cognitive, speech-language, fine motor, and sphincter functions were normal as the baseline. Both pin prick and light touch sensation of the right lower leg were lost, but normal of the left side. The active flexions of the right ankle and foot were remarkably decreased compared to the left side. Bilateral ankle dorsiflexion was trace (baseline) and difficult to notice the difference. Ankle jerk was not obtainable on the right side. The compound muscle action potentials (CMAPs) of the tibial nerves (Rt/Lt) showed distal latencies 3.9/3.7 msec, conduction velocities 35.9/41 m/sec, and amplitudes 0.21/8.3 mV. The CMAPs of the peroneal nerves (Rt/Lt) showed distal latencies 5.3/4.8 msec, conduction velocities 40.2/40.6 m/sec below knee and 46/58 m/sec across the knee, respectively, and amplitudes 0.88/0.95 mV. The sensory nerve action potentials (SNAP)of the right sural and peroneal nerves were not elicited, but those of the left sural and superficial nerve were distal peak latencies 3.2 and 3.1 msec and amplitudes 8.3 and 5.3 microV, respectively. No conduction block was found. No abnormal spontaneous resting potentials were noticed of the right lower extremity with a monopolar needle electromyography. Magnetic resonance imaging study of the lumbosacral spine was negative for cord or root lesions. Anti-neutrophil cytoplasmic antibody (ANCA) test for rheumatic vasculitis was negative. Discussion: The findings of the nerve conduction study were consistent with acute axonal monomelic peripheral neuropathy presumably due to hypothermia. However, the needle electromyographic study did not show any abnormal resting potentials of the affected extremity muscles even three weeks after the symptom developed. Conclusions: We report a case of monomelic peripheral neuropathy presumably due to hypothermia with unusual electromyographic findings of remarkably decreased amplitudes of the motor and sensory
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nerves without abnormal resting potentials at the three weeks after neurological symptoms develop. Level of Evidence: Level IV Poster 224 Giant Cell Myocarditis Masked by Acute Inflammatory Demyelinating Polyneuropathy: A Case Report Jessalynn Adam, MD (Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States), Kristin Gustafson, DO Disclosures: Jessalynn Adam: I Have No Relevant Financial Relationships To Disclose Case/Program Description: Patient: 42-year-old woman with acute inflammatory demyelinating polyneuropathy (AIDP) and giant cell myocarditis. Case Description: The patient first presented to the emergency department with dyspnea, dizziness, tingling in her arms, and profound weakness. She was hyponatremic, sodium of 103, and had profound sensory loss in stocking-glove distribution, with impaired proprioception, arreflexia, and pseudoathetosis. Mental status, cranial nerves, and cerebellar testing were normal. MRI brain and cervical spine were unremarkable. Lumbar puncture revealed acellular fluid, increased CSF protein, normal glucose, and no organisms or monoclonal bands. CSF paraneoplastic panel was negative. EMG showed absent sensory responses and decreased amplitude of motor responses. She was diagnosed with presumed AIDP, treated with IVIG, and improved somewhat. Of note, she had episodic chest pain in acute care but workup was unremarkable. While in rehab, she had severe orthostatic hypotension and persistent dyspnea despite normal oxygen saturations. After one week of rehab, she abruptly lost consciousness and seized. She was sent to the emergency department where she was noted to be in acute cardiogenic shock with native heart rate in the 30s, elevated troponins to 9.33, and severe biventricular failure due to complete non-perfusing heart block. She was placed on ECMO. Setting: Quaternary Academic Rehabilitation Hospital. Results: Cardiac biopsy revealed giant cell myocarditis. Discussion: Hyperacute onset of heart failure is a common presentation of giant cell myocarditis, however, this case is unusual as the AIDP may have obscured other symptoms and masked the diagnosis of giant cell myocarditis. Conclusions: Two thirds of patients with AIDP develop autonomic features, including postural hypotension, but sustained sinus tachycardia is the most common dysfunction. EKG is indicated in autonomic dysfunction with atypical cardiac responses, such as persistent dyspnea or hypotension with bradycardia. These symptoms should raise suspicion for other complications or diagnoses, and prompt further workup. Level of Evidence: Level V Poster 225 Bickerstaff’s Encephalitis with an Exemplary Recovery Following Rehabilitation: A Case Report Michael J. Irvine, DO (University of TX-UT Houston, Houston, Texas, United States), Paolo C. Mimbella, MD, Tomasz K. Podobinski, DO Disclosures: Michael Irvine: I Have No Relevant Financial Relationships To Disclose Case/Program Description: A 59-year-old man with no prior medical history who presented to the emergency department with rapidly progressing neurological symptoms. He had several days of flu like symptoms with sudden weakness, difficulty walking, and blurry vision prior to coming to the ED. He required intubation for airway protection and remained unresponsive for a period of 3 weeks. Based on serological testing and clinical findings, he was diagnosed with Bickerstaff’s encephalitis. He was treated with plasmapheresis and immunoglobulins and subsequently regained consciousness. The patient was medically stabilized, but he had tetraparesis, dysphagia, and
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visual deficits as a result of his condition for which he would undergo rehabilitation. Setting: Rehabilitation hospital, Outpatient clinic. Results: The patient was sent to a rehabilitation hospital requiring moderate to maximum assistance with daily activities and mobility. He had extensive daily rehabilitation with physical, occupational, and speech therapy services. The patient’s dramatic recovery and outpatient follow-up will be further discussed in this report. Discussion: Bickerstaff’s encephalitis is closely related to Miller Fisher Syndrome but generally distinguished by encephalopathy and hyperreflexia. The reason for these differentiating symptoms is the involvement of the brain stem in the disease process of Bickerstaff’s encephalitis. Both of these conditions along with pharyngeal-cervical-brachial-weakness have anti-GQ1b antibodies. There is debate over whether these are separate conditions or different manifestations of the same anti-GQ1b syndrome. Plasmapheresis and IVIG therapy have been found to be effective treatments in these patients. It is worth analyzing more examples of these conditions, especially those with a favorable outcome, in order to gain a better understanding of them. Conclusions: Bickerstaff’s encephalitis is a rare condition related to others in the GBS spectrum. It is important to look at potential factors affecting recovery in less studied disorders such as these in order to have a greater understanding of their treatment and resulting prognosis. Level of Evidence: Level V Poster 226 Unusual Presentation of Eight and a Half Syndrome in a Patient with Acute Pontine Medullary Stroke: A Case Report Phillip Acevedo, MD (TUH, Philadelphia, PA, United States), Katie Hatt, DO, Ning Cao, MD Disclosures: Phillip Acevedo: I Have No Relevant Financial Relationships To Disclose Case/Program Description: This 68-year-old woman with a past medical history of Type II diabetes mellitus, hypertension, and previous left cerebrovascular accident with residual right hemiparesis presented to the ER with nausea and vomiting, hyperglycemia, and visual disturbances. Examination revealed one and a half syndrome presenting with right intranuclear ophthalmoplegia (INO) and horizontal gaze palsy. MRI of brain revealed an acute punctate infarct at the right dorsal pontomedullary junction in addition to chronic lacunar infarcts. On day 5, she was transferred to an inpatient rehabilitation facility. However, on rehab day number five, the patient developed acute right facial droop, incapable of closing her right eye, slurred speech and otalgia. Setting: Inpatient Rehabilitation Facility. Results: Neurology and ENT consults were obtained, MRI was repeated and progression of the right pontomedullary infarct with new right facial nerve enhancement was noted. Eight-and-a-Half Syndrome was diagnosed. A 7-day course of prednisone and valacyclovir with gabapentin for pain control was begun. Occlusion therapy with OT was initiated for her diplopia. On day 19, patient was able to be discharged home with resolving slurred speech and facial palsy. Discussion: Eight-and-a-half syndrome is caused by a lesion in the dorsal tegmentum of the caudal pons involving the parapontine reticular formation or abducens nucleus and MLF, and the nucleus/ fasciculus of the facial nerve. It is a rare presentation of pontine stroke. Our patient’s delayed presentation of facial palsy made the diagnosis very challenging. Conclusions: A rare case of “eight-and-a-half” syndrome is a unique combination of “one-and-a-half” syndrome plus ipsilateral fascicular cranial nerve seventh palsy. Thus, the recognition of the syndrome is
important for appropriate management and to ensure good functional outcomes. Level of Evidence: Level V Poster 227 Cauda Equina Syndrome after Lumbar Disk Herniation: A Case Report Siena N. Ona, MD (Vidant Rehab Center/East Carolina University/ Brod, Greenville, NC, United States), Raymund V. Millan, MD Disclosures: Siena Ona: I Have No Relevant Financial Relationships To Disclose Case/Program Description: HR is a 36-year-old man with chronic back pain who was admitted for sudden difficulty in ambulating associated with nonspecific lower extremity numbness, bowel dysfunction and urinary retention progressing to inability to void. He had a history of persistent chronic back pain which was diagnosed as severe sciatica on prior visits to the ED. Setting: 75 bed Rehabilitation Hospital. Results: On day of admission, MRI showed massive extrusion of L3-L4 disk material, segmental stenosis and severe narrowing of compression of thecal sac. Patient then underwent emergent lumbar decompression for cauda equina syndrome. Postop patient’s weakness improved but he was still having weakness on bilateral dorsiflexion, and still had bowel dysfunction requiring suppositories and bladder dysfunction requiring in and out cauterization. Discussion: The presentation of cauda equina syndrome varies from its classical presentation, especially in its early stages of compression. In this case for example, the patient’s symptoms were attributed to a severe case of sciatica. Delayed diagnosis and treatment of this syndrome could lead to poor outcomes. National annual incidence of low back pain is 5%. One quarter of patients with back pain have sciatica. The most common cause of sciatica is herniation of the lower lumbar intervertebral disks. In contrast to sciatica, cases of CES after disk herniation are relatively rare. In one study, the incidence of CES due to lumbar disk herniation has been reported to range from 1% to 10% of operated disk cases. Conclusions: Acute compression of the cauda equina is a neurologically compromising and debilitating condition. Physicians who evaluate low back pain must be able to recognize the signs and symptoms of this relatively rare but critical spinal syndrome and must expedite emergent evaluation and intervention. Level of Evidence: Level V Poster 228 Zolpidem for the Treatment of Neurologic Disorders: A Systematic Review Martin N. Bomalaski, MD (University of Michigan, Ann Arbor, MI, United States), Mark Peterson, PhD, Edward Claflin, MD Disclosures: Martin Bomalaski: I Have No Relevant Financial Relationships To Disclose Objective: To perform a systematic review of all studies using zolpidem to treat neurologic disorders not including insomnia. Design: Eligibility criteria included any article examining the use of zolpidem for non-insomnia neurologic disorders on humans, published and unpublished, written in English, and for all dates up to March 2015. Searched databases included PubMed, Scopus, Web of Science, Cochrane Library, and Embase. Positive publication bias was mitigated by searching ClinicalTrials.gov for unpublished studies. Two rounds of screening were performed based on title and then abstract, respectively. Setting: N/A. Participants: N/A. Interventions: N/A.
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nerves without abnormal resting potentials at the three weeks after neurological symptoms develop. Level of Evidence: Level IV Poster 224 Giant Cell Myocarditis Masked by Acute Inflammatory Demyelinating Polyneuropathy: A Case Report Jessalynn Adam, MD (Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, United States), Kristin Gustafson, DO Disclosures: Jessalynn Adam: I Have No Relevant Financial Relationships To Disclose Case/Program Description: Patient: 42-year-old woman with acute inflammatory demyelinating polyneuropathy (AIDP) and giant cell myocarditis. Case Description: The patient first presented to the emergency department with dyspnea, dizziness, tingling in her arms, and profound weakness. She was hyponatremic, sodium of 103, and had profound sensory loss in stocking-glove distribution, with impaired proprioception, arreflexia, and pseudoathetosis. Mental status, cranial nerves, and cerebellar testing were normal. MRI brain and cervical spine were unremarkable. Lumbar puncture revealed acellular fluid, increased CSF protein, normal glucose, and no organisms or monoclonal bands. CSF paraneoplastic panel was negative. EMG showed absent sensory responses and decreased amplitude of motor responses. She was diagnosed with presumed AIDP, treated with IVIG, and improved somewhat. Of note, she had episodic chest pain in acute care but workup was unremarkable. While in rehab, she had severe orthostatic hypotension and persistent dyspnea despite normal oxygen saturations. After one week of rehab, she abruptly lost consciousness and seized. She was sent to the emergency department where she was noted to be in acute cardiogenic shock with native heart rate in the 30s, elevated troponins to 9.33, and severe biventricular failure due to complete non-perfusing heart block. She was placed on ECMO. Setting: Quaternary Academic Rehabilitation Hospital. Results: Cardiac biopsy revealed giant cell myocarditis. Discussion: Hyperacute onset of heart failure is a common presentation of giant cell myocarditis, however, this case is unusual as the AIDP may have obscured other symptoms and masked the diagnosis of giant cell myocarditis. Conclusions: Two thirds of patients with AIDP develop autonomic features, including postural hypotension, but sustained sinus tachycardia is the most common dysfunction. EKG is indicated in autonomic dysfunction with atypical cardiac responses, such as persistent dyspnea or hypotension with bradycardia. These symptoms should raise suspicion for other complications or diagnoses, and prompt further workup. Level of Evidence: Level V Poster 225 Bickerstaff’s Encephalitis with an Exemplary Recovery Following Rehabilitation: A Case Report Michael J. Irvine, DO (University of TX-UT Houston, Houston, Texas, United States), Paolo C. Mimbella, MD, Tomasz K. Podobinski, DO Disclosures: Michael Irvine: I Have No Relevant Financial Relationships To Disclose Case/Program Description: A 59-year-old man with no prior medical history who presented to the emergency department with rapidly progressing neurological symptoms. He had several days of flu like symptoms with sudden weakness, difficulty walking, and blurry vision prior to coming to the ED. He required intubation for airway protection and remained unresponsive for a period of 3 weeks. Based on serological testing and clinical findings, he was diagnosed with Bickerstaff’s encephalitis. He was treated with plasmapheresis and immunoglobulins and subsequently regained consciousness. The patient was medically stabilized, but he had tetraparesis, dysphagia, and
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Abstracts / PM R 8 (2016) S151-S332
visual deficits as a result of his condition for which he would undergo rehabilitation. Setting: Rehabilitation hospital, Outpatient clinic. Results: The patient was sent to a rehabilitation hospital requiring moderate to maximum assistance with daily activities and mobility. He had extensive daily rehabilitation with physical, occupational, and speech therapy services. The patient’s dramatic recovery and outpatient follow-up will be further discussed in this report. Discussion: Bickerstaff’s encephalitis is closely related to Miller Fisher Syndrome but generally distinguished by encephalopathy and hyperreflexia. The reason for these differentiating symptoms is the involvement of the brain stem in the disease process of Bickerstaff’s encephalitis. Both of these conditions along with pharyngeal-cervical-brachial-weakness have anti-GQ1b antibodies. There is debate over whether these are separate conditions or different manifestations of the same anti-GQ1b syndrome. Plasmapheresis and IVIG therapy have been found to be effective treatments in these patients. It is worth analyzing more examples of these conditions, especially those with a favorable outcome, in order to gain a better understanding of them. Conclusions: Bickerstaff’s encephalitis is a rare condition related to others in the GBS spectrum. It is important to look at potential factors affecting recovery in less studied disorders such as these in order to have a greater understanding of their treatment and resulting prognosis. Level of Evidence: Level V Poster 226 Unusual Presentation of Eight and a Half Syndrome in a Patient with Acute Pontine Medullary Stroke: A Case Report Phillip Acevedo, MD (TUH, Philadelphia, PA, United States), Katie Hatt, DO, Ning Cao, MD Disclosures: Phillip Acevedo: I Have No Relevant Financial Relationships To Disclose Case/Program Description: This 68-year-old woman with a past medical history of Type II diabetes mellitus, hypertension, and previous left cerebrovascular accident with residual right hemiparesis presented to the ER with nausea and vomiting, hyperglycemia, and visual disturbances. Examination revealed one and a half syndrome presenting with right intranuclear ophthalmoplegia (INO) and horizontal gaze palsy. MRI of brain revealed an acute punctate infarct at the right dorsal pontomedullary junction in addition to chronic lacunar infarcts. On day 5, she was transferred to an inpatient rehabilitation facility. However, on rehab day number five, the patient developed acute right facial droop, incapable of closing her right eye, slurred speech and otalgia. Setting: Inpatient Rehabilitation Facility. Results: Neurology and ENT consults were obtained, MRI was repeated and progression of the right pontomedullary infarct with new right facial nerve enhancement was noted. Eight-and-a-Half Syndrome was diagnosed. A 7-day course of prednisone and valacyclovir with gabapentin for pain control was begun. Occlusion therapy with OT was initiated for her diplopia. On day 19, patient was able to be discharged home with resolving slurred speech and facial palsy. Discussion: Eight-and-a-half syndrome is caused by a lesion in the dorsal tegmentum of the caudal pons involving the parapontine reticular formation or abducens nucleus and MLF, and the nucleus/ fasciculus of the facial nerve. It is a rare presentation of pontine stroke. Our patient’s delayed presentation of facial palsy made the diagnosis very challenging. Conclusions: A rare case of “eight-and-a-half” syndrome is a unique combination of “one-and-a-half” syndrome plus ipsilateral fascicular cranial nerve seventh palsy. Thus, the recognition of the syndrome is
important for appropriate management and to ensure good functional outcomes. Level of Evidence: Level V Poster 227 Cauda Equina Syndrome after Lumbar Disk Herniation: A Case Report Siena N. Ona, MD (Vidant Rehab Center/East Carolina University/ Brod, Greenville, NC, United States), Raymund V. Millan, MD Disclosures: Siena Ona: I Have No Relevant Financial Relationships To Disclose Case/Program Description: HR is a 36-year-old man with chronic back pain who was admitted for sudden difficulty in ambulating associated with nonspecific lower extremity numbness, bowel dysfunction and urinary retention progressing to inability to void. He had a history of persistent chronic back pain which was diagnosed as severe sciatica on prior visits to the ED. Setting: 75 bed Rehabilitation Hospital. Results: On day of admission, MRI showed massive extrusion of L3-L4 disk material, segmental stenosis and severe narrowing of compression of thecal sac. Patient then underwent emergent lumbar decompression for cauda equina syndrome. Postop patient’s weakness improved but he was still having weakness on bilateral dorsiflexion, and still had bowel dysfunction requiring suppositories and bladder dysfunction requiring in and out cauterization. Discussion: The presentation of cauda equina syndrome varies from its classical presentation, especially in its early stages of compression. In this case for example, the patient’s symptoms were attributed to a severe case of sciatica. Delayed diagnosis and treatment of this syndrome could lead to poor outcomes. National annual incidence of low back pain is 5%. One quarter of patients with back pain have sciatica. The most common cause of sciatica is herniation of the lower lumbar intervertebral disks. In contrast to sciatica, cases of CES after disk herniation are relatively rare. In one study, the incidence of CES due to lumbar disk herniation has been reported to range from 1% to 10% of operated disk cases. Conclusions: Acute compression of the cauda equina is a neurologically compromising and debilitating condition. Physicians who evaluate low back pain must be able to recognize the signs and symptoms of this relatively rare but critical spinal syndrome and must expedite emergent evaluation and intervention. Level of Evidence: Level V Poster 228 Zolpidem for the Treatment of Neurologic Disorders: A Systematic Review Martin N. Bomalaski, MD (University of Michigan, Ann Arbor, MI, United States), Mark Peterson, PhD, Edward Claflin, MD Disclosures: Martin Bomalaski: I Have No Relevant Financial Relationships To Disclose Objective: To perform a systematic review of all studies using zolpidem to treat neurologic disorders not including insomnia. Design: Eligibility criteria included any article examining the use of zolpidem for non-insomnia neurologic disorders on humans, published and unpublished, written in English, and for all dates up to March 2015. Searched databases included PubMed, Scopus, Web of Science, Cochrane Library, and Embase. Positive publication bias was mitigated by searching ClinicalTrials.gov for unpublished studies. Two rounds of screening were performed based on title and then abstract, respectively. Setting: N/A. Participants: N/A. Interventions: N/A.