recurrent miller fisher variant of guillain-barré syndrome: a case report

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Complicating matters was her history of a bilateral transradial amputation due to a work injury in 1988. Prior to admission she lived alone and was independent with basic and advanced activities of daily living (ADLs) using bilateral body powered prostheses with voluntary opening hand hooks. She was procient enough with their use that she continued to be a one half pack per day smoker. Setting: Tertiary Care Hospital. Results or Clinical Course: The main goal of therapy was to help the patient return to her baseline status with ADLs. The major barrier was her left visual eld decit causing double vision. This was addressed with taping her glasses, which helped with the double vision, but resulted in a loss of depth perception and difculty donning her prostheses. Over time, her vision gradually improved and she was able to accommodate for her residual visual decits. She was discharged home with her daughter at a standby assist level given increased time to complete tasks. Discussion: To our knowledge there are no documented cases of visual decit from cardioembolic stroke in a bilateral hand amputee. As stroke survival rates increase we are faced with unique co-morbidities that affect function. Conclusions: As survival rates for both stroke and amputation increase we need to be prepared to address the challenges of both to keep our patients as functional and independent as we can. Poster 410 Rehabilitation of an Eighteen-Year-Old Male with a Severe Traumatic Brain Injury Complicated by Sinking Skin Flap Syndrome: A Case Report. Mary E. Russell, DO, MS (Baylor College of Medicine, Houston, TX, United States); Monica E. Crump, MD. Disclosures: M. E. Russell, No Disclosures: I Have Nothing To Disclose. Case Description: The patient is an 18-year-old male who presented to an outside hospital after a self-inicted gunshot wound to the head for which the patient underwent emergent hemicraniectomy and decompression. Patient had ventriculoper- itoneal shunt placement for hydrocephalus on hospital day 22. He presented to our inpatient rehabilitation hospital 28 days after injury. Patient was making great strides during his rehabilitation course and progressed from total assistance to minimal assistance with ADLs and transfers. However, six weeks into his rehabilitation stay, patient became extremely limited by pain from positional headaches. Multiple medications for pain, including norco, gaba- pentin and topiramate were started for headaches presumed to be secondary to an overdraining shunt. The shunt was adjusted and headaches improved slightly. Patient began to digress with thera- pies and was noted to have increasing lethargy, decreased respon- siveness, increased spasticity and irretractable nausea and vomiting. Head CT done during week seven of inpatient rehabilitation showed an increase in the sunken in appearance of the brain with increased left to right subfalcine herniation. Patient was transferred to an acute care hospital to expedite cranioplasty given neurologic deterioration from sinking skin ap syndrome. Program Description: 18-year-old male with severe neurolog- ical decits secondary to a gunshot wound to the head. Setting: Rehabilitation hospital. Results or Clinical Course: Three months after injury, patient had shown signicant improvement before neurologic deterioration secondary to sinking skin ap syndrome. It is uncertain what decits may remain after cranioplasty. Patient was discharged to a post-acute brain injury rehabilitation program. Further develop- ments will be discussed. Discussion: Sinking skin ap syndrome is a serious and unusual complication of hemicraniectomy after traumatic brain injury and should be considered given neurologic deterioration in the setting of subfalcine herniation in a patient with hydrocephalus managed with a ventriculoperitoneal shunt. Conclusions: Sinking skin ap syndrome is an unusual neuro- logical complication of hemicraniectomy in the traumatic brain injury population. Poster 411 Recurrent Miller Fisher Variant of Guillain-Barré Syndrome: A Case Report. Hannah A. Shoval (New York Presbyterian Hospital of Columbia and Cornell, New York, NY, United States); Allyson A. Shrikhande, MD. Disclosures: H. A. Shoval, No Disclosures: I Have Nothing To Disclose. Case Description: AF, a 33-year-old woman, presented with a second episode of Guillain-Barre syndrome Miller Fisher variant (MFS) 12 years after her rst episode. She reported numbness of the hands and feet, "lazy" left eye and ataxic gait starting 10 days after an upper respiratory illness. Brain magnetic resonance imaging showed enhancement of bilateral cranial nerve seven. Patients GQ1b anti- body was positive. Electromyography showed sensorimotor demy- elinating peripheral neuropathy. AF required mechanical ventilation and a percutaneous endoscopic gastrostomy (PEG). Exam showed absent reexes and near complete paralysis retaining only the ability to raise her left eyebrow and move her right toes. AF had unreactive pupils, no blink to threat, complete opthalmoplegia and absent gag. AF was treated with intravenous immunoglobulin. Methadone and clonazepam started for anxiety and pain. Metoprolol started for tachycardia to the 150s secondary to dysautonomia. Gabapentin started for neuropathic pain. AF improved and after one month was admitted to inpatient rehabilitation unit (IRU). Setting: Tertiary care hospital. Results or Clinical Course: In the IRU, AF was decannulated and underwent a trial of void successfully. Metoprolol was decreased; methadone discontinued. Re-empowering AF was crucial given her prior inability to communicate. Therapists encouraged, physicians explained medication choices carefully, and psychiatry supported with psychotherapy. After 2 1 / 2 weeks, AF ambulated 800 feet independently and was modied independent with activities of daily living. AF passed a modied barium swallow and advanced to a regular diet. Discussion: MFS is characterized by ataxia, areexia and oph- thalmoplegia. Recurrence does not exclude the diagnosis. It is mostly an acute self-limiting condition but can progress rapidly and close monitoring is critical. Early mobilization including ranging extremities is important. Admission to IRU enables a multi-disci- plinary team approach with physical and occupational therapists, speech and language pathologists, supportive counseling and close medical surveillance. This can re-empower the patient for an ef- cient transition to home. Conclusions: The effects of MFS can be devastating for patients and correct diagnosis is critical. IRU is a valuable resource for transitioning to a home setting. PM&R Vol. 5, Iss. 9S, 2013 S281

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Page 1: Recurrent Miller Fisher Variant of Guillain-Barré Syndrome: A Case Report

PM&R Vol. 5, Iss. 9S, 2013 S281

Complicating matters was her history of a bilateral transradialamputation due to a work injury in 1988. Prior to admission shelived alone and was independent with basic and advanced activitiesof daily living (ADLs) using bilateral body powered prostheses withvoluntary opening hand hooks. She was proficient enough withtheir use that she continued to be a one half pack per day smoker.Setting: Tertiary Care Hospital.Results or Clinical Course: The main goal of therapy was tohelp the patient return to her baseline status with ADLs. The majorbarrier was her left visual field deficit causing double vision. Thiswas addressed with taping her glasses, which helped with thedouble vision, but resulted in a loss of depth perception anddifficulty donning her prostheses. Over time, her vision graduallyimproved and she was able to accommodate for her residual visualdeficits. She was discharged home with her daughter at a standbyassist level given increased time to complete tasks.Discussion: To our knowledge there are no documented cases ofvisual deficit from cardioembolic stroke in a bilateral handamputee. As stroke survival rates increase we are faced with uniqueco-morbidities that affect function.Conclusions: As survival rates for both stroke and amputationincrease we need to be prepared to address the challenges of both tokeep our patients as functional and independent as we can.

Poster 410Rehabilitation of an Eighteen-Year-Old Male witha Severe Traumatic Brain Injury Complicated bySinking Skin Flap Syndrome: A Case Report.Mary E. Russell, DO, MS (Baylor College of Medicine,Houston, TX, United States); Monica E. Crump, MD.

Disclosures: M. E. Russell, No Disclosures: I Have Nothing ToDisclose.Case Description: The patient is an 18-year-old male whopresented to an outside hospital after a self-inflicted gunshotwound to the head for which the patient underwent emergenthemicraniectomy and decompression. Patient had ventriculoper-itoneal shunt placement for hydrocephalus on hospital day 22. Hepresented to our inpatient rehabilitation hospital 28 days afterinjury. Patient was making great strides during his rehabilitationcourse and progressed from total assistance to minimal assistancewith ADLs and transfers. However, six weeks into his rehabilitationstay, patient became extremely limited by pain from positionalheadaches. Multiple medications for pain, including norco, gaba-pentin and topiramate were started for headaches presumed to besecondary to an overdraining shunt. The shunt was adjusted andheadaches improved slightly. Patient began to digress with thera-pies and was noted to have increasing lethargy, decreased respon-siveness, increased spasticity and irretractable nausea and vomiting.Head CT done during week seven of inpatient rehabilitationshowed an increase in the sunken in appearance of the brain withincreased left to right subfalcine herniation. Patient was transferredto an acute care hospital to expedite cranioplasty given neurologicdeterioration from sinking skin flap syndrome.Program Description: 18-year-old male with severe neurolog-ical deficits secondary to a gunshot wound to the head.Setting: Rehabilitation hospital.Results or Clinical Course: Three months after injury, patienthad shown significant improvement before neurologic deteriorationsecondary to sinking skin flap syndrome. It is uncertain what

deficits may remain after cranioplasty. Patient was discharged toa post-acute brain injury rehabilitation program. Further develop-ments will be discussed.Discussion: Sinking skin flap syndrome is a serious and unusualcomplication of hemicraniectomy after traumatic brain injury andshould be considered given neurologic deterioration in the settingof subfalcine herniation in a patient with hydrocephalus managedwith a ventriculoperitoneal shunt.Conclusions: Sinking skin flap syndrome is an unusual neuro-logical complication of hemicraniectomy in the traumatic braininjury population.

Poster 411Recurrent Miller Fisher Variant of Guillain-BarréSyndrome: A Case Report.Hannah A. Shoval (New York Presbyterian Hospital ofColumbia and Cornell, New York, NY, United States);Allyson A. Shrikhande, MD.

Disclosures: H. A. Shoval, No Disclosures: I Have Nothing ToDisclose.Case Description: AF, a 33-year-old woman, presented witha second episode of Guillain-Barre syndrome Miller Fisher variant(MFS) 12 years after her first episode. She reported numbness of thehands and feet, "lazy" left eye and ataxic gait starting 10 days after anupper respiratory illness. Brain magnetic resonance imaging showedenhancement of bilateral cranial nerve seven. Patient’s GQ1b anti-body was positive. Electromyography showed sensorimotor demy-elinating peripheral neuropathy. AF required mechanical ventilationand a percutaneous endoscopic gastrostomy (PEG). Exam showedabsent reflexes and near complete paralysis retaining only the abilityto raise her left eyebrow and move her right toes. AF had unreactivepupils, no blink to threat, complete opthalmoplegia and absent gag.AF was treated with intravenous immunoglobulin. Methadone andclonazepam started for anxiety and pain. Metoprolol started fortachycardia to the 150’s secondary to dysautonomia. Gabapentinstarted for neuropathic pain. AF improved and after one month wasadmitted to inpatient rehabilitation unit (IRU).Setting: Tertiary care hospital.Results or Clinical Course: In the IRU, AF was decannulatedand underwent a trial of void successfully. Metoprolol wasdecreased; methadone discontinued. Re-empowering AF wascrucial given her prior inability to communicate. Therapistsencouraged, physicians explained medication choices carefully, andpsychiatry supported with psychotherapy. After 21/2 weeks, AFambulated 800 feet independently and was modified independentwith activities of daily living. AF passed a modified barium swallowand advanced to a regular diet.Discussion: MFS is characterized by ataxia, areflexia and oph-thalmoplegia. Recurrence does not exclude the diagnosis. It ismostly an acute self-limiting condition but can progress rapidly andclose monitoring is critical. Early mobilization including rangingextremities is important. Admission to IRU enables a multi-disci-plinary team approach with physical and occupational therapists,speech and language pathologists, supportive counseling and closemedical surveillance. This can re-empower the patient for an effi-cient transition to home.Conclusions: The effects of MFS can be devastating for patientsand correct diagnosis is critical. IRU is a valuable resource fortransitioning to a home setting.