S254 S290
Case/Program Description:
Bilateral facial paralysis is very rare.
Guillain-Barre syndrome (CBS) must always be among the differential
diagnoses. A 61-year-old woman presented with bilateral facial
weakness, dysphagia, bilateral ptosis, blurred vision, which pro-
gressed to decreased lower extremity strength, sensation, and co-
ordination. MRI of the brain and cervical and thoracic spine
performed were unremarkable. Lumbar puncture revealed cytoalbu-
minologic dissociation. Plasmapheresis treatment was initiated for
presumed GBS. Patient was transferred to intensive care unit due to
concern for respiratory compromise. Physiatry performed diagnostic
electromyography (EMG) and nerve conduction studies (NCS) on day 5
after onset. Following clinical improvement with plasmapheresis,
patient was transferred to inpatient rehabilitation (IPR). Lower ex-
tremity strength modestly improved by time of discharge from IPR.
Facial diplegia resolved over 6 weeks. Blurred vision also resolved
and the patient was able to fully close her eyes after 6 weeks. The
patient was able to swallow thin liquids without a straw after 4
months.
Setting:
Intensive Care Unit/Inpatient Rehabilitation.
Results:
EMG/NCS revealed sensory responses in upper extremities
with prolonged distal latencies. Sural and superficial fibular sensory
responses were normal. All distal motor latencies were prolonged in
upper and lower extremities. Motor conductions were slowed in the
upper extremity but normal in the lower extremity. Findings revealed
an early idiopathic demyelinating polyneuropathy consistent with GBS.
Ocular symptoms and ataxia were suggestive of the Miller-Fisher GBS
variant.
Discussion:
EMG and NCS studies on GBS patients with bilateral facial
paralysis have consistently shown evidence of a demyelinating process
with no evidence of an axonal-type neuropathy. Optimal outcomes
have typically been achieved with intensive pulmonary function as-
sessments and treatment with plasmapheresis or intravenous
immunoglobulin.
Conclusions:
Clinicians must be acutely aware of bilateral facial pa-
ralysis as a presenting feature of GBS. Any delay in treatment may lead
to compromise in both acute and rehabilitative outcome.
Level of Evidence:
Level V
Poster 384:
Anton-Babinski Syndrome Diagnosed During Inpatient
Rehabilitation
Brittni Micham (UPMC Medical Education PM&R Program, Pittsburgh,
PA, USA), Adam Lewno, DO, Julie Lanphere, DO
Disclosures:
Brittni Micham: I Have No Relevant Financial Relation-
ships To Disclose
Case/Program Description:
A 70-year-old man with prior right pos-
terior cerebral artery (PCA) stroke presented with confusion and
sudden onset vision loss. MRI revealed an acute ischemic left PCA
infarct. CT angiogram showed bilateral PCA occlusions. He was not a
candidate for reperfusion therapy and was discharged to inpatient
rehabilitation. On exam, he had no reaction to visual threat, but
reported he was able to perceive light and movement approxi-
mately 3 feet away. He was supervision level assistance for
grooming, feeding and dressing. He insisted he could see well
enough to perform activities of daily living and wished to be dis-
charged home. A detailed evaluation with the therapy team
revealed inability to navigate his room, visually track objects and
recognize safety hazards. He had no awareness of his visual deficits.
Despite inability to perform functional tasks safely, the patient
believed his visual deficits had resolved and he insisted on returning
home.
Setting:
Inpatient rehabilitation.
Results:
Based on clinical evidence of cortical blindness, bilateral
PCA strokes, and the patient’s confabulation and denial of deficits,
he was diagnosed with Anton-Babinski Syndrome. The rehabilitation
team discussed Anton-Babinski Syndrome with the patient and his
family and demonstrated his deficits, but both patient and family
continued to lack insight and family signed him out against medical
advice. At 6-month follow up, he was still unable to recognize his
visual deficits.
Discussion:
Patients with Anton-Babinski Syndrome can be excep-
tionally convincing and difficult to identify. Family members and even
physicians may not recognize complete cortical blindness due to the
patient appearing to have minimal functional deficits. These patients
require supportive care, continual communication, education, and
family meetings to assist in transition out of the hospital to the
community.
Conclusions:
Detailed evaluation in conjunction with speech, phys-
ical, and occupational therapists enhances the physician’s under-
standing of a patient’s true functional impairments and avoids
misdiagnosis.
Level of Evidence:
Level V
Poster 385:
Claude Syndrome: An Unusual Stroke Syndrome
Kadir J. Carruthers, BS (Univ Med Cntr of Pittsburgh),
Mary Ann Miknevich, MD
Disclosures:
Kadir Carruthers: I Have No Relevant Financial Re-
lationships To Disclose
Case/Program Description:
Claude’s syndrome is characterized by
ipsilateral oculomotor nerve palsy and contralateral cerebellar
ataxia due to a lesion of the oculomotor and red nuclei of the
midbrain and superior cerebellar peduncle. This syndrome is quite
rare with no precise incidence or prevalance data available. The
infrequency which it is encountered may lead to misdiagnosis. A 58-
year-old left-handed man presented with reduced arousability,
slurred speech, and inability to open left eye. Physical exam was
notable for left third nerve palsy and ptosis, bilateral vertical gaze
palsy, right-sided ataxia and intention tremor, right lower facial
weakness, and moderate dysarthria. Patient was initially assessed
to have Benedikt syndrome. MRI of the brain showed a faint signal
abnormality on the left thalamus with no clear thrombus. Given
occulomotor nerve involvement, a thrombus was postulated to have
been present at the junction of the basilar artery and posterior
cerebral artery prior to dissolving. Transthoracic echocardiogram
revealed a patent foramen ovale. Patient was started on anti-
coagulation due to concern for possible cardioembolic etiology.
Following acute management, patient was transferred to inpatient
rehabilitation with goals for independent management of self-care
and mobility prior to returning home.
Setting:
Inpatient Rehabilitation Unit.
Results:
The patient progressed from requiring moderate assistance
for ambulation with wheeled walker to contact-guard assist with
improved maintenance of posture by the second month of rehabilita-
tion. At 4 months, dysphagia and performance of activities of daily
living showed considerable improvement although left ptosis
persisted.
Discussion:
Discernment between midbrain stroke syndromes
affecting the oculomotor nerve can be challenging. Most involve a
component of both hemiataxia and hemiparesis. Small midbrain nuclei
are also not easily identified on neuroimaging. Prominent contralateral
hemiataxia present in this patient is most consistent with Claude’s
syndrome.
Conclusions:
Accurate diagnosis of Claude syndrome and other stroke
syndromes may lead to refinement of rehabilitation goals and
improved outcomes.
Level of Evidence:
Level V
S254
Abstracts / PM R 9 (2017) S131-S290