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Poster 401 POEMS Syndrome Presenting as Gait Instability and Foot Drop: A Case Report
PM&R
Poster 399 Recurrent Episodes of Extreme Somnolence Post Artery of Percheron Infarct: A Case Report. Nasim Chowdhury, M.D (New York PresbyterianColumbia and Cornell, New York, NY, United States); C. David Lin, MD. Disclosures: N. Chowdhury, No Disclosures: I Have No Relevant Financial Relationships to Disclose. Case Description: An 81-year-old man with heart disease presented to the emergency department (ED) with altered mental status. In the ED he was noted to have dysarthria, dysfunction of the 3rd, 4th, and 6th cranial nerves with diplopia, and right sided dysmetria. He subsequently became markedly somnolent. Computed tomography angiography (CTA) revealed thalamic infarcts but no vessel obstruction. Magnetic resonance imaging revealed bilateral acute/subacute thalamic infarcts extending to the midbrain. Given his findings, the diagnosis of artery of Percheron infarct was made. After medical stabilization, he was transferred to the acute inpatient rehabilitation unit for visual derangements, gait ataxia, and speech impairment. While there, he suffered from 3 episodes of extreme somnolence (minimally arousable only for a few seconds) over five days. Serial head CTs were negative. Patient’s insomnia medication was discontinued. The episodes exposed no new focal neurological findings, lasted approximately 1 hour, and did not recur prior to discharge. Setting: Inpatient rehabilitation unit of large tertiary care hospital. Results or Clinical Course: The patient received multidisciplinary therapy on the inpatient rehabilitation unit and medical optimization by physicians for an artery of Percheron infarct with workup of his episodes of somnolence completely negative. Discussion: The artery of Percheron infarct is a rare entity stemming from a characteristic pattern of bilateral paramedian thalamic infarct that can involve the midbrain. Angiography does not reveal any occlusions. The syndrome is usually described as a triad of hypersomnolence, amnesia, and vertical gaze paresis. Conclusions: Hypersomnolence is a known finding in an artery of Percheron infarct. Given the findings in our case, one notes that the hypersomnolence is self limiting and improves with time and rehab even without the addition of stimulants. Hopefully this case can help avoid unnecessary medications and minimize patient radiation exposure. References: N.A. Lazzaro et al. Artery of Percheron Infarction: Imaging Patterns and Clinical Spectrum. American Journal of Neuroradiology 2010 31: 1283-1289. Originally published online on March 18, 2010, 10.3174/ajnr.A2044.
Poster 400 Tuberculosis of the Spine (Pott’s Disease) Presenting with Complete Paraplegia: A Case Report. Holly Asmussen, DO (Schwab Rehabilitation Hospital, Chicago, IL, United States); Raymond Lee, MD. Disclosures: H. Asmussen, No Disclosures: I Have No Relevant Financial Relationships to Disclose.
Vol. 6, Iss. 9S, 2014
S325
Case Description: A 21-year-old man originally from Sierra Leone who had been incarcerated for 12 to 15 months presented with a progressive 4 week bilateral lower extremity weakness, absent sensation below nipple line, stage 3 sacral ulcer and bowel and bladder incontinence while in jail. Setting: Inpatient rehabilitation hospital. Results or Clinical Course: Neuroimaging studies demonstrated a T5 and T6 enhancement suggestive of Potts disease with extramedullary compression and no intervertebral disc involvement. Quantiferon TB test was positive. Four acid fast bacilli sputum smears were negative, CXR was negative, IR guided biopsy was negative for growth of TB or other fungal/bacterial species but given appearance of imaging studies and positive quantiferon TB, he was started on anti-tuberculosis therapy. His neurological examination was significant for 5/5 strength in upper extremities, 0/5 strength in lower extremities and absent pinprick sensation from T6 and below with no volitional rectal tone or sensation to pinprick or deep pressure in rectal region. Patient’s clinical course in rehabilitation was significant for heterotopic ossification of the knees, spasticity, neurogenic bowel and bladder and treatment of sacral ulcer. Discussion: Literature review on epidemiological data indicates that spinal TB accounts for 1.7% of all cases of TB. The reported incidence of neurological deficit in spinal tuberculosis varies between 23 to 76%. Radicular pain, weakness, and numbness are the most commonly reported symptoms. Only approximately 10% of all spinal TB cases will present with paraplegia and rarely do disturbances of bowel and bladder sphincters occur at an early stage as seen in our patient. In our literature review, 0-1.3% of reported cases Potts disease with paraplegia are Frankel A, 2.7% are Frankel B, 2.4-10.8% are Frankel C, 2.4-39.4% are Frankel D and 48.6% are Frankel E. Conclusions: This is a rare case of Potts disease with complete paraplegia as the presenting symptom.
Poster 401 POEMS Syndrome Presenting as Gait Instability and Foot Drop: A Case Report. Kristin Garlanger, DO (Mayo Clinic, Rochester, MN, United States); Morgan Brubaker, DO; Mark H. Winemiller, MD. Disclosures: K. Garlanger, No Disclosures: I Have No Relevant Financial Relationships to Disclose. Case Description: A 38-year-old man from Venezuela presented with 18 months of progressive gait ataxia, foot drop, and limb paresthesias. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and given intravenous immunoglobulin without benefit. Despite using a cane and self-fashioned ankle foot orthoses (AFOs), he reported over 20 falls in the past year. He also reported leg swelling, red hand discoloration, vision difficulties, intermittent dyspnea and erectile dysfunction. Physical examination revealed facial flushing, axillary lymphadenopathy, high steppage and ataxic gait, diffuse weakness and hyporeflexia. Setting: Academic medical center.
S326
Results or Clinical Course: Further work-up revealed hepatosplenomegaly, sclerotic bone lesions of the cervical vertebrae and pelvis, type 2 diabetes, hypogonadism, pancytosis, elevated VEGF and monoclonal protein. Electrodiagnostic studies showed a severe mixed demyelinating and axonal polyradiculoneuropathy. He was ultimately diagnosed with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. He subsequently underwent chemotherapy and hematopoietic stem cell transplantation. Concurrently, he worked with physical therapy on balance and gait training using custom posterior-leaf spring AFOs and a four-wheeled walker. His walking efficiency, stability and safety improved substantially prior to his return to Venezuela. Discussion: POEMS syndrome is a rare multisystem paraneoplastic blood disorder named for its clinically recognizable features. Peripheral neuropathy is frequently the presenting complaint. As with this case, patients are often misdiagnosed until other clinical manifestations arise. Electrodiagnostic studies consistent with demyelination and axonal degeneration together with serum monoclonal protein support the diagnosis. Conclusions: Physiatrists may be the initial referral for foot drop, therefore it is important to recognize POEMS syndrome as a possible cause. The role of rehabilitation in providing appropriate assistive devices can significantly improve lifestyle when facing severe disability.
Poster 402 Persistent Vestibular Symptoms in Patients with Post Concussion Syndrome and Treatment for Perilymphatic Fistula: A Case Series Report. Marina M. Ma, MD (Medstar Georgetown University Hospital/National Rehabilitation Network, Washington, DC, United States); Fabiolla Siqueira, MD; Marilyn F. Kraus, MD; Dennis C. Fitzgerald, MD. Disclosures: M. M. Ma, No Disclosures: I Have No Relevant Financial Relationships to Disclose. Case Description: We present 5 cases of post concussion syndrome with persistent vestibular dysfunction. All patients presented with persistent dizziness and headache lasting 3 months to 1 year post injury. Mechanism of injury include a pedestrian and a bicyclist who were struck by a motor vehicle, 2 sports related injury, and 1 head trauma after hitting a coffee table. All patients were diagnosed with concussion by an emergency physician and with a post concussion syndrome by a concussion specialist at an outpatient clinic. Patients were then sent to physical therapy specializing in vestibular rehabilitation. Setting: Outpatient Rehabilitation Hospital Clinic and Outpatient Acute Care Hospital. Results or Clinical Course: All patients were initially managed conservatively and completed at least one full course of vestibular therapy. Due to persistent vestibular symptoms, they were referred to a neurotologist (inner ear specialist) for further management. All received an audiogram, electrocochleography, videonystagmography and platform pressure test and were subsequently diagnosed with perilymphatic fistula (PLF). Four patients underwent a PLF repair with significant improvement of symptoms. Treatment consists of placing a tiny soft tissue graft over suspected leak sites in the oval and round window areas. One patient declined
PRESENTATIONS
the procedure as his symptoms improved with vestibular therapy alone. Discussion: Vestibular dysfunction after concussion is common and can add to the severity of post concussion syndrome, including aggravation of anxiety, headache and cognitive complaints. Vestibular therapy should be considered first. If vestibular symptoms persist, PLF should be considered. Conclusions: This cases series demonstrates the importance of screening for perilymphatic fistulas in post concussive patients with persistent vestibular symptoms as treatment can improve functioning and quality of life. Poster 403 Effect of Repetitive Transcranial Magnetic Stimulation According to Stimulation Site in Stroke Patients with Dysphagia. Sook Joung Lee, MD (Dong-A University College of Medicine, Busan, Korea, Republic of); Min kyu Park, MD, PhD. Disclosures: S. Lee, No Disclosures: I Have No Relevant Financial Relationships to Disclose. Objective: To investigate the effect of repetitive transcranial magnetic stimulation (rTMS) according to the stimulation site in subacute stroke patients with dysphagia. Design: Age and lesion matched comparative study. Setting: Different repetitive transcranial magnetic stimulation site. Participants: Twenty-four patients who had dysphagia after ischemic stroke were recruited and were divided into two groups according to age and stroke lesion matched. Interventions: The patients in Group A received rTMS on the brain cortex where motor evoked potential (MEP) was obtained from the suprahyoid muscle. Group B received rTMS on the brain cortex where MEP was obtained from abductor pollicis brevis muscle. rTMS was performed at 100% of MEP threshold, 10Hz frequency for 10 seconds, and then repeated every minute for 10 minutes (Total 1000 stimulus, 5 times a week for 2 weeks). Both group received conventional dysphagia management with the same method. Main Outcome Measures: The results of video fluoroscopic swallowing study (Dysphagia status was measured by the Functional Dysphagia Scale (FDS), the Penetration Aspiration Scale (PAS) and the Dysphagia Outcome and Severity Scale (DOSS) using the results of video fluoroscopic swallowing study.) Results or Clinical Course: There were no significant differences in general characteristics of patients between group A and B. FDS score showed improvement in both group A and B. PAS and DOSS scores improved only in the group A. When treatment effects were compared between two groups, group A showed more significant improvement than group B immediately after rTMS (0.80.8 vs. 0.30.8, p<0.05) and 4 weeks after rTMS (1.20.8 vs. 0.61.2, p<0.05) in the DOSS score. There were no significant differences in the changes of FDS and PAS scores between group A and B. There were no significant differences in the changes of Mini Mental Status Examination (MMSE) and Korean version of Modified Barthel Index (K-MBI) scores between two groups. Conclusions: rTMS on the specific site related to dysphagia showed more improvement on swallowing function when compared to interconnected remote site. When rTMS was
Poster 399 Recurrent Episodes of Extreme Somnolence Post Artery of Percheron Infarct: A Case Report. Nasim Chowdhury, M.D (New York PresbyterianColumbia and Cornell, New York, NY, United States); C. David Lin, MD. Disclosures: N. Chowdhury, No Disclosures: I Have No Relevant Financial Relationships to Disclose. Case Description: An 81-year-old man with heart disease presented to the emergency department (ED) with altered mental status. In the ED he was noted to have dysarthria, dysfunction of the 3rd, 4th, and 6th cranial nerves with diplopia, and right sided dysmetria. He subsequently became markedly somnolent. Computed tomography angiography (CTA) revealed thalamic infarcts but no vessel obstruction. Magnetic resonance imaging revealed bilateral acute/subacute thalamic infarcts extending to the midbrain. Given his findings, the diagnosis of artery of Percheron infarct was made. After medical stabilization, he was transferred to the acute inpatient rehabilitation unit for visual derangements, gait ataxia, and speech impairment. While there, he suffered from 3 episodes of extreme somnolence (minimally arousable only for a few seconds) over five days. Serial head CTs were negative. Patient’s insomnia medication was discontinued. The episodes exposed no new focal neurological findings, lasted approximately 1 hour, and did not recur prior to discharge. Setting: Inpatient rehabilitation unit of large tertiary care hospital. Results or Clinical Course: The patient received multidisciplinary therapy on the inpatient rehabilitation unit and medical optimization by physicians for an artery of Percheron infarct with workup of his episodes of somnolence completely negative. Discussion: The artery of Percheron infarct is a rare entity stemming from a characteristic pattern of bilateral paramedian thalamic infarct that can involve the midbrain. Angiography does not reveal any occlusions. The syndrome is usually described as a triad of hypersomnolence, amnesia, and vertical gaze paresis. Conclusions: Hypersomnolence is a known finding in an artery of Percheron infarct. Given the findings in our case, one notes that the hypersomnolence is self limiting and improves with time and rehab even without the addition of stimulants. Hopefully this case can help avoid unnecessary medications and minimize patient radiation exposure. References: N.A. Lazzaro et al. Artery of Percheron Infarction: Imaging Patterns and Clinical Spectrum. American Journal of Neuroradiology 2010 31: 1283-1289. Originally published online on March 18, 2010, 10.3174/ajnr.A2044.
Poster 400 Tuberculosis of the Spine (Pott’s Disease) Presenting with Complete Paraplegia: A Case Report. Holly Asmussen, DO (Schwab Rehabilitation Hospital, Chicago, IL, United States); Raymond Lee, MD. Disclosures: H. Asmussen, No Disclosures: I Have No Relevant Financial Relationships to Disclose.
Vol. 6, Iss. 9S, 2014
S325
Case Description: A 21-year-old man originally from Sierra Leone who had been incarcerated for 12 to 15 months presented with a progressive 4 week bilateral lower extremity weakness, absent sensation below nipple line, stage 3 sacral ulcer and bowel and bladder incontinence while in jail. Setting: Inpatient rehabilitation hospital. Results or Clinical Course: Neuroimaging studies demonstrated a T5 and T6 enhancement suggestive of Potts disease with extramedullary compression and no intervertebral disc involvement. Quantiferon TB test was positive. Four acid fast bacilli sputum smears were negative, CXR was negative, IR guided biopsy was negative for growth of TB or other fungal/bacterial species but given appearance of imaging studies and positive quantiferon TB, he was started on anti-tuberculosis therapy. His neurological examination was significant for 5/5 strength in upper extremities, 0/5 strength in lower extremities and absent pinprick sensation from T6 and below with no volitional rectal tone or sensation to pinprick or deep pressure in rectal region. Patient’s clinical course in rehabilitation was significant for heterotopic ossification of the knees, spasticity, neurogenic bowel and bladder and treatment of sacral ulcer. Discussion: Literature review on epidemiological data indicates that spinal TB accounts for 1.7% of all cases of TB. The reported incidence of neurological deficit in spinal tuberculosis varies between 23 to 76%. Radicular pain, weakness, and numbness are the most commonly reported symptoms. Only approximately 10% of all spinal TB cases will present with paraplegia and rarely do disturbances of bowel and bladder sphincters occur at an early stage as seen in our patient. In our literature review, 0-1.3% of reported cases Potts disease with paraplegia are Frankel A, 2.7% are Frankel B, 2.4-10.8% are Frankel C, 2.4-39.4% are Frankel D and 48.6% are Frankel E. Conclusions: This is a rare case of Potts disease with complete paraplegia as the presenting symptom.
Poster 401 POEMS Syndrome Presenting as Gait Instability and Foot Drop: A Case Report. Kristin Garlanger, DO (Mayo Clinic, Rochester, MN, United States); Morgan Brubaker, DO; Mark H. Winemiller, MD. Disclosures: K. Garlanger, No Disclosures: I Have No Relevant Financial Relationships to Disclose. Case Description: A 38-year-old man from Venezuela presented with 18 months of progressive gait ataxia, foot drop, and limb paresthesias. He was previously diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and given intravenous immunoglobulin without benefit. Despite using a cane and self-fashioned ankle foot orthoses (AFOs), he reported over 20 falls in the past year. He also reported leg swelling, red hand discoloration, vision difficulties, intermittent dyspnea and erectile dysfunction. Physical examination revealed facial flushing, axillary lymphadenopathy, high steppage and ataxic gait, diffuse weakness and hyporeflexia. Setting: Academic medical center.
S326
Results or Clinical Course: Further work-up revealed hepatosplenomegaly, sclerotic bone lesions of the cervical vertebrae and pelvis, type 2 diabetes, hypogonadism, pancytosis, elevated VEGF and monoclonal protein. Electrodiagnostic studies showed a severe mixed demyelinating and axonal polyradiculoneuropathy. He was ultimately diagnosed with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. He subsequently underwent chemotherapy and hematopoietic stem cell transplantation. Concurrently, he worked with physical therapy on balance and gait training using custom posterior-leaf spring AFOs and a four-wheeled walker. His walking efficiency, stability and safety improved substantially prior to his return to Venezuela. Discussion: POEMS syndrome is a rare multisystem paraneoplastic blood disorder named for its clinically recognizable features. Peripheral neuropathy is frequently the presenting complaint. As with this case, patients are often misdiagnosed until other clinical manifestations arise. Electrodiagnostic studies consistent with demyelination and axonal degeneration together with serum monoclonal protein support the diagnosis. Conclusions: Physiatrists may be the initial referral for foot drop, therefore it is important to recognize POEMS syndrome as a possible cause. The role of rehabilitation in providing appropriate assistive devices can significantly improve lifestyle when facing severe disability.
Poster 402 Persistent Vestibular Symptoms in Patients with Post Concussion Syndrome and Treatment for Perilymphatic Fistula: A Case Series Report. Marina M. Ma, MD (Medstar Georgetown University Hospital/National Rehabilitation Network, Washington, DC, United States); Fabiolla Siqueira, MD; Marilyn F. Kraus, MD; Dennis C. Fitzgerald, MD. Disclosures: M. M. Ma, No Disclosures: I Have No Relevant Financial Relationships to Disclose. Case Description: We present 5 cases of post concussion syndrome with persistent vestibular dysfunction. All patients presented with persistent dizziness and headache lasting 3 months to 1 year post injury. Mechanism of injury include a pedestrian and a bicyclist who were struck by a motor vehicle, 2 sports related injury, and 1 head trauma after hitting a coffee table. All patients were diagnosed with concussion by an emergency physician and with a post concussion syndrome by a concussion specialist at an outpatient clinic. Patients were then sent to physical therapy specializing in vestibular rehabilitation. Setting: Outpatient Rehabilitation Hospital Clinic and Outpatient Acute Care Hospital. Results or Clinical Course: All patients were initially managed conservatively and completed at least one full course of vestibular therapy. Due to persistent vestibular symptoms, they were referred to a neurotologist (inner ear specialist) for further management. All received an audiogram, electrocochleography, videonystagmography and platform pressure test and were subsequently diagnosed with perilymphatic fistula (PLF). Four patients underwent a PLF repair with significant improvement of symptoms. Treatment consists of placing a tiny soft tissue graft over suspected leak sites in the oval and round window areas. One patient declined
PRESENTATIONS
the procedure as his symptoms improved with vestibular therapy alone. Discussion: Vestibular dysfunction after concussion is common and can add to the severity of post concussion syndrome, including aggravation of anxiety, headache and cognitive complaints. Vestibular therapy should be considered first. If vestibular symptoms persist, PLF should be considered. Conclusions: This cases series demonstrates the importance of screening for perilymphatic fistulas in post concussive patients with persistent vestibular symptoms as treatment can improve functioning and quality of life. Poster 403 Effect of Repetitive Transcranial Magnetic Stimulation According to Stimulation Site in Stroke Patients with Dysphagia. Sook Joung Lee, MD (Dong-A University College of Medicine, Busan, Korea, Republic of); Min kyu Park, MD, PhD. Disclosures: S. Lee, No Disclosures: I Have No Relevant Financial Relationships to Disclose. Objective: To investigate the effect of repetitive transcranial magnetic stimulation (rTMS) according to the stimulation site in subacute stroke patients with dysphagia. Design: Age and lesion matched comparative study. Setting: Different repetitive transcranial magnetic stimulation site. Participants: Twenty-four patients who had dysphagia after ischemic stroke were recruited and were divided into two groups according to age and stroke lesion matched. Interventions: The patients in Group A received rTMS on the brain cortex where motor evoked potential (MEP) was obtained from the suprahyoid muscle. Group B received rTMS on the brain cortex where MEP was obtained from abductor pollicis brevis muscle. rTMS was performed at 100% of MEP threshold, 10Hz frequency for 10 seconds, and then repeated every minute for 10 minutes (Total 1000 stimulus, 5 times a week for 2 weeks). Both group received conventional dysphagia management with the same method. Main Outcome Measures: The results of video fluoroscopic swallowing study (Dysphagia status was measured by the Functional Dysphagia Scale (FDS), the Penetration Aspiration Scale (PAS) and the Dysphagia Outcome and Severity Scale (DOSS) using the results of video fluoroscopic swallowing study.) Results or Clinical Course: There were no significant differences in general characteristics of patients between group A and B. FDS score showed improvement in both group A and B. PAS and DOSS scores improved only in the group A. When treatment effects were compared between two groups, group A showed more significant improvement than group B immediately after rTMS (0.80.8 vs. 0.30.8, p<0.05) and 4 weeks after rTMS (1.20.8 vs. 0.61.2, p<0.05) in the DOSS score. There were no significant differences in the changes of FDS and PAS scores between group A and B. There were no significant differences in the changes of Mini Mental Status Examination (MMSE) and Korean version of Modified Barthel Index (K-MBI) scores between two groups. Conclusions: rTMS on the specific site related to dysphagia showed more improvement on swallowing function when compared to interconnected remote site. When rTMS was