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Poster 292: Stiff Person Syndrome Without Axial Stiffness or Hyperekplexia: A Case Report
S224
Abstracts / PM R 9 (2017) S131-S290
NEUROLOGICAL REHABILITATION POSTER HALL: CASE REPORTS
Poster 291: Severe Sensory Neuropathy vs Sensory Ganglionopathy in a Patient with Sjogren’s Syndrome: A Case Report Brennan Boettcher, DO (Mayo Clinic, Rochester, MN, United States), Terin Euerle, MD, Sarah E. Berini, MD Disclosures: Brennan Boettcher: I Have No Relevant Financial Relationships To Disclose Case/Program Description: A 42-year-old woman with a 5-year history of Sjogren’s syndrome, chronic steroid use and osteoporosis presented with complaints of numbness and pain in all four limbs and difficulty walking, which had progressed over several years. She had decreased light touch and pinprick sensation to the mid-thighs and elbows bilaterally. Her strength was poor to initial testing, but with visual reinforcement of the tested muscles, strength returned to normal. She was referred for electromyography (EMG) and nerve conduction studies. Antinuclear antibody and anti SS-A/La antibodies were elevated. Setting: Quaternary care academic center. Results: Motor nerve conduction studies and blink reflexes were normal. Sensory nerve conduction studies were non-reactive in the right upper and lower limbs, consistent with a severe sensory peripheral neuropathy vs. sensory ganglionopathy. Needle EMG of the right upper and lower limbs was normal other than short duration, low amplitude motor unit potentials with rapid recruitment in the triceps, perhaps related to steroid use. Rheumatology was consulted, and the patient was ultimately placed on azathioprine as a steroid sparing agent for Sjo ¨gren’s syndrome. She was also referred to physical and occupational therapy for fall prevention recommendations, ADL modifications, and adaptive equipment. Discussion: This is a case of a severe sensory neuropathy vs sensory ganglionopathy resulting from Sjogren’s syndrome. Central and peripheral nervous system involvement has been reported in up to 20% of individuals with Sjo ¨gren’s syndrome, with sensory ganglionopathy being the most common. Sensory ganglionopathy can be seen with toxic exposure as well as autoimmune, paraneoplastic disorders. The treatment of this condition is not clear, with plasma exchange, IV immunoglobulin, corticosteroids and immunosuppressants reported as possible treatments. Conclusions: Sjogren’s syndrome is usually limited to glandular symptoms but can result in a variety of neuropathic findings, including pure sensory, sensorimotor and small fiber neuropathies. Level of Evidence: Level V
Poster 292: Stiff Person Syndrome Without Axial Stiffness or Hyperekplexia: A Case Report Melody Lee, MD (Mayo Clinic of Rochester) Disclosures: Melody Lee: I Have No Relevant Financial Relationships To Disclose Case/Program Description: Ms. S presented to our emergency department with progressive bilateral leg pain and tightness for the past year. She was having worsening rigidity with resulting contractures, and started requiring a wheelchair for mobility. As she had fallen multiple times at home, she was admitted for further workup. PM&R was consulted for evaluation of her leg stiffness. On examination, there was notable rigidity and co-contraction in her bilateral lower extremities along with flexion contractures of her knees and ankles. She also had marked toe clawing. However, there was no axial
involvement or startle response, despite multiple attempts to illicit these signs. MRI of the neuroaxis was unremarkable, except for an amorphous, nonspecific, non-enhancing T2 cord signal abnormality at the cervical medullary junction. A movement lab study was suggestive of a CNS hyperexcitability disorder. An EMG did not show evidence for a peripheral cause for her symptoms, though it did suggest some functional overlay. An extensive lab workup was performed, including CSF studies, and was remarkable for high titers of GAD65 antibody in both the blood and the CSF. She was started on diazepam 5mg TID and a course of IVIG with rapid improvement in her symptoms. Setting: Tertiary care hospital. Results: At 2 weeks follow-up, her pain and rigidity is much more tolerable, and she can ambulate without a gait aid. Discussion: Cases of stiff person syndrome without axial involvement have been described, but this is the first reported case, to our knowledge, of stiff person syndrome (or stiff limb syndrome), without the characteristic hyperekplexia. Conclusions: Stiff person syndrome can be present even without the expected hyperekplexia and axial rigidity. Benzodiazepines can be beneficial in these cases. Level of Evidence: Level V
Poster 293: Ogilvie Syndrome Identified in an Aphasic Stroke Patient: A Case Report Patrick Martone, DO (WA Hosp Cntr/Georgetown Univ), Rachna Malhotra, DO Disclosures: Patrick Martone: I Have No Relevant Financial Relationships To Disclose Case/Program Description: An 81-year-old man presenting for acute rehabilitation after suffering from a left middle cerebral artery ischemic stroke. On initial evaluation, the patient was noted to suffer from multiple deficits including right hemiparesis and severe nonfluent aphasia. On admission, it was also noted that the patient had abdominal distention, although the abdomen was non-tender with active bowel sounds. Over the course of 5 days, the patient’s appetite had decreased. Previously, he had been tolerating a pureed nectar thick diet. On repeat examination the abdominal distention had worsened, and therefore an abdominal x-ray was obtained. Setting: Acute Rehabilitation. Results: Abdominal x-ray demonstrated colonic distention measuring greater than 15cm in diameter at some points. Discussion: The patient was found to be suffering from acute colonic pseudo-obstruction, or Ogilvie syndrome. Ogilvie syndrome is a bowel obstruction that does not have a mechanical cause. Symptoms include abdominal discomfort, distention, and vomiting, which develop over several days. It is typically associated with electrolyte imbalances or occurs after abdominal surgery. However, some cases are associated with neurologic conditions such as spinal cord injury, multiple sclerosis and stroke. While the exact mechanism is unknown, it is likely that autonomic disturbances post-stroke result in complications with bowel motility. Gastroenterology and general surgery were consulted and the patient was scheduled for decompression and ultimately a hemi-colectomy due to ischemia of the bowel. Conclusions: Ogilvie syndrome is a potentially life threatening disease that can be difficult to identify. This is especially true in the case of an aphasic patient who is unable to express his symptoms. While acute colonic pseudo-obstruction is not common in post-stroke patients, cases have been reported and this warrants further investigation. Clinicians must be aware of this syndrome and its physical findings in order to identify it in our most vulnerable patient populations, those who are unable to express themselves. Level of Evidence: Level V
Abstracts / PM R 9 (2017) S131-S290
NEUROLOGICAL REHABILITATION POSTER HALL: CASE REPORTS
Poster 291: Severe Sensory Neuropathy vs Sensory Ganglionopathy in a Patient with Sjogren’s Syndrome: A Case Report Brennan Boettcher, DO (Mayo Clinic, Rochester, MN, United States), Terin Euerle, MD, Sarah E. Berini, MD Disclosures: Brennan Boettcher: I Have No Relevant Financial Relationships To Disclose Case/Program Description: A 42-year-old woman with a 5-year history of Sjogren’s syndrome, chronic steroid use and osteoporosis presented with complaints of numbness and pain in all four limbs and difficulty walking, which had progressed over several years. She had decreased light touch and pinprick sensation to the mid-thighs and elbows bilaterally. Her strength was poor to initial testing, but with visual reinforcement of the tested muscles, strength returned to normal. She was referred for electromyography (EMG) and nerve conduction studies. Antinuclear antibody and anti SS-A/La antibodies were elevated. Setting: Quaternary care academic center. Results: Motor nerve conduction studies and blink reflexes were normal. Sensory nerve conduction studies were non-reactive in the right upper and lower limbs, consistent with a severe sensory peripheral neuropathy vs. sensory ganglionopathy. Needle EMG of the right upper and lower limbs was normal other than short duration, low amplitude motor unit potentials with rapid recruitment in the triceps, perhaps related to steroid use. Rheumatology was consulted, and the patient was ultimately placed on azathioprine as a steroid sparing agent for Sjo ¨gren’s syndrome. She was also referred to physical and occupational therapy for fall prevention recommendations, ADL modifications, and adaptive equipment. Discussion: This is a case of a severe sensory neuropathy vs sensory ganglionopathy resulting from Sjogren’s syndrome. Central and peripheral nervous system involvement has been reported in up to 20% of individuals with Sjo ¨gren’s syndrome, with sensory ganglionopathy being the most common. Sensory ganglionopathy can be seen with toxic exposure as well as autoimmune, paraneoplastic disorders. The treatment of this condition is not clear, with plasma exchange, IV immunoglobulin, corticosteroids and immunosuppressants reported as possible treatments. Conclusions: Sjogren’s syndrome is usually limited to glandular symptoms but can result in a variety of neuropathic findings, including pure sensory, sensorimotor and small fiber neuropathies. Level of Evidence: Level V
Poster 292: Stiff Person Syndrome Without Axial Stiffness or Hyperekplexia: A Case Report Melody Lee, MD (Mayo Clinic of Rochester) Disclosures: Melody Lee: I Have No Relevant Financial Relationships To Disclose Case/Program Description: Ms. S presented to our emergency department with progressive bilateral leg pain and tightness for the past year. She was having worsening rigidity with resulting contractures, and started requiring a wheelchair for mobility. As she had fallen multiple times at home, she was admitted for further workup. PM&R was consulted for evaluation of her leg stiffness. On examination, there was notable rigidity and co-contraction in her bilateral lower extremities along with flexion contractures of her knees and ankles. She also had marked toe clawing. However, there was no axial
involvement or startle response, despite multiple attempts to illicit these signs. MRI of the neuroaxis was unremarkable, except for an amorphous, nonspecific, non-enhancing T2 cord signal abnormality at the cervical medullary junction. A movement lab study was suggestive of a CNS hyperexcitability disorder. An EMG did not show evidence for a peripheral cause for her symptoms, though it did suggest some functional overlay. An extensive lab workup was performed, including CSF studies, and was remarkable for high titers of GAD65 antibody in both the blood and the CSF. She was started on diazepam 5mg TID and a course of IVIG with rapid improvement in her symptoms. Setting: Tertiary care hospital. Results: At 2 weeks follow-up, her pain and rigidity is much more tolerable, and she can ambulate without a gait aid. Discussion: Cases of stiff person syndrome without axial involvement have been described, but this is the first reported case, to our knowledge, of stiff person syndrome (or stiff limb syndrome), without the characteristic hyperekplexia. Conclusions: Stiff person syndrome can be present even without the expected hyperekplexia and axial rigidity. Benzodiazepines can be beneficial in these cases. Level of Evidence: Level V
Poster 293: Ogilvie Syndrome Identified in an Aphasic Stroke Patient: A Case Report Patrick Martone, DO (WA Hosp Cntr/Georgetown Univ), Rachna Malhotra, DO Disclosures: Patrick Martone: I Have No Relevant Financial Relationships To Disclose Case/Program Description: An 81-year-old man presenting for acute rehabilitation after suffering from a left middle cerebral artery ischemic stroke. On initial evaluation, the patient was noted to suffer from multiple deficits including right hemiparesis and severe nonfluent aphasia. On admission, it was also noted that the patient had abdominal distention, although the abdomen was non-tender with active bowel sounds. Over the course of 5 days, the patient’s appetite had decreased. Previously, he had been tolerating a pureed nectar thick diet. On repeat examination the abdominal distention had worsened, and therefore an abdominal x-ray was obtained. Setting: Acute Rehabilitation. Results: Abdominal x-ray demonstrated colonic distention measuring greater than 15cm in diameter at some points. Discussion: The patient was found to be suffering from acute colonic pseudo-obstruction, or Ogilvie syndrome. Ogilvie syndrome is a bowel obstruction that does not have a mechanical cause. Symptoms include abdominal discomfort, distention, and vomiting, which develop over several days. It is typically associated with electrolyte imbalances or occurs after abdominal surgery. However, some cases are associated with neurologic conditions such as spinal cord injury, multiple sclerosis and stroke. While the exact mechanism is unknown, it is likely that autonomic disturbances post-stroke result in complications with bowel motility. Gastroenterology and general surgery were consulted and the patient was scheduled for decompression and ultimately a hemi-colectomy due to ischemia of the bowel. Conclusions: Ogilvie syndrome is a potentially life threatening disease that can be difficult to identify. This is especially true in the case of an aphasic patient who is unable to express his symptoms. While acute colonic pseudo-obstruction is not common in post-stroke patients, cases have been reported and this warrants further investigation. Clinicians must be aware of this syndrome and its physical findings in order to identify it in our most vulnerable patient populations, those who are unable to express themselves. Level of Evidence: Level V