S224 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 Re-
lationships To Disclose
Case/Program Description:
A 42-year-old woman with a 5-year his-
tory of Sjogren’s syndrome, chronic steroid use and osteoporosis pre-
sented 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 pe-
ripheral 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 pe-
ripheral 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 contrac-
tures, 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 examina-
tion, 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 Relation-
ships 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 non-
fluent 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 mecha-
nism 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
S224
Abstracts / PM R 9 (2017) S131-S290