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Poster 324:
CLIPPERS (Chronic Lymphocytic Inflammation with
Pontine Perivascular Enhancement Responsive to
Steroids): A Treatable and Reversible Inflammatory
Disorder of the Central Nervous System. A Case
Report
Roberta Lui (Albert Einstein Col of Med), Francis J. Lopez, MD
Disclosures:
Roberta Lui: I Have No Relevant Financial Relationships
To Disclose
Case/Program Description:
We present a 54-year-old man with
medical history of CLIPPERS syndrome who presented after an acute
episode of tonic-clonic seizure at home and progressively worsening
gait to the point of immobility over the past 2 months. Incidentally, his
neurologist had titrated down his maintenance dose of prednisone
from 40 mg to 10 mg daily 2 months prior due to side effects. MRI of
the brain was performed showing multiple T2 hyper-intense and T1
enhancing lesions throughout the cerebral parenchyma, cerebellum,
and brainstem consistent with his known CLIPPERS syndrome, as well
as a new area of enhancement in the left mesial temporal area. He
was started high dose glucocorticoids (GCs) and maintained on a
slightly higher dose of mycophenolate.
Setting:
Inpatient Acute Rehabilitation.
Results:
He exhibited marked improvement in all fields of therapy
after initiation of medications and was discharged home with a cane
for ambulation.
Discussion:
CLIPPERS syndrome is a rare, central nervous system in-
flammatory disorder that has not been well documented and only
described as far back as 2010. Clinical presentations vary, including
ataxia and seizures. Most unique to this syndrome is its response to
high-dose steroids and recurrence of lesions without maintenance
therapy. The drug of choice is GCs but it is limited in large to its side
effects. Additional CGS-sparing agents such as mycophenolate,
methotrexate, and rituximab are commonly used, but there is no
widely-accepted dose for each medication and clinicians are often left
to trial and error dosing based on patient tolerance and symptoms.
Conclusions:
CLIPPERS syndrome remains to be a newly described
syndrome. It is highly responsive to steroids and patients often make
close to full recovery. They are excellent candidates for acute reha-
bilitation. Knowledge of CLIPPERS syndrome and its clinical pre-
sentations is imperative for diagnosis, treatment, and rehabilitation
planning for individuals with this disease.
Level of Evidence:
Level V
Poster 325:
Progressive Cervical Myelopathy Due to Sarcoidosis:
A Case Report
Ashley C. Schneider, MD (Univ of Toledo), Steven J. Farrell, MD
Disclosures:
Ashley Schneider: I Have No Relevant Financial Re-
lationships To Disclose
Case/Program Description:
The patient was referred for EMG testing
of the right upper extremity due to numbness, tingling, and wasting of
the extremity. Further history revealed multiple near falls and pro-
gressive difficulty ambulating. Physical exam findings included bilat-
eral upper extremity weakness, increased deep tendon reflexes in all 4
extremities, and a positive Romberg test. Due to clinical suspicion of
CNS pathology, EMG was deferred and a cervical MRI was performed.
Setting:
Physical Medicine and Rehab Outpatient Clinic.
Results:
MRI of the cervical spine demonstrated abnormal T2 hyper-
intensity and cord expansion through C7-T1 as well as patchy
enhancement at C4 and C5. A preliminary diagnosis of astrocytoma
was made until a CT scan of the chest performed for chronic dyspnea
revealed mediastinal and perihilar lymphadenopathy consistent with
sarcoidosis. Granulomas were identified on biopsy of mediastinal
lymph nodes and it was concluded that the patient was suffering from
neurosarcoidosis of the cervical spine. He had some improvement
clinically with oral steroids and is currently on a trial of
mycophenolate.
Discussion:
Thought to occur in less than 5% of patients with systemic
sarcoidosis, neurosarcoidosis is a rare disorder and initial manifesta-
tion as a cervical myelopathy is even more uncommon and notoriously
difficult to diagnose. High clinical suspicion, thorough analysis for signs
of systemic disease, and histology demonstrating classic granuloma-
tous inflammation aid in the appropriate identification of
neurosarcoidosis.
Conclusions:
The importance of obtaining a thorough history and
physical exam prior to the performance of EMG cannot be under-
estimated. The presence of upper motor neuron findings warrant a
thorough evaluation for CNS pathology and multisystemic symptoms
should prompt investigation for generalized inflammatory disorders
such as sarcoidosis.
Level of Evidence:
Level V
Poster 326:
Development of New Heterotopic Ossification 35 Years After
Spinal Cord Injury: A Case Report
Erika Gosai (Univ of TX Southwestern Med Ctr), Jennifer Yang, MD
Disclosures:
Erika Gosai: I Have No Relevant Financial Relationships
To Disclose
Case/Program Description:
This patient has a history significant for
C5 AIS C tetraplegia from a motor vehicle accident in 1981 and well-
managed symptomatic left hip osteoarthritis for 15 years. He pre-
sented to clinic with a 2-week history of increased pain and spasticity
in the left hip, partially relieved by NSAIDs. The patient was involved
as a restrained driver in a motor vehicle accident 7 months earlier,
with the only identified injury being muscular contusion of the chest
wall. His steering wheel spinner knob struck him in the left anterior
chest at the moment of impact.
Setting:
Spinal Cord Injury (SCI) Clinic.
Results:
On exam the patient had decreased range of motion in
bilateral hip flexion and abduction, with bilateral (L
>
R) groin pain
with the FABER maneuver. Bilateral hip radiographs showed bilateral
hip osteoarthritis with adjacent heterotopic ossification (HO) sites not
seen on imaging taken 3 years earlier. As the patient was already
taking a bisphosphonate for osteoporosis, and NSAIDs for osteoar-
thritis, we opted to continue these two medications in an effort to
slow down further bone formation.
Discussion:
HO most commonly occurs within the first 6 months and
may also occur even several years after spinal cord injury. This is the
first reported case, to our knowledge, of HO formation 35 years after
injury without evidence of localized trauma or inflammation (i.e.
pressure ulcer, fracture, deep venous thrombosis, septic arthritis).
Conclusions:
This case demonstrates that a new trauma, however
remote from the site of aberrant bone formation, can incite the for-
mation of new HO many years after the initial spinal cord injury.
Level of Evidence:
Level V
Poster 327:
Dysphagia as an Unusual Presenting Symptom of Chronic
Post-Hypoxic Myoclonus: A Case Report
Courtney J. Stefanski (Rehabilitation Institute of Chicago),
Sangeeta P. Driver, MD MPH
Disclosures:
Courtney Stefanski: I Have No Relevant Financial Re-
lationships To Disclose
Case/Program Description:
A 19-year-old man developed status
asthmaticus resulting in cardiac arrest and hypoxic-ischemic enceph-
alopathy (HIE). Approximately 4 weeks later, he developed dysphagia
and then subsequently was found to have new myoclonus of the
tongue, right face and right arm.
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Abstracts / PM R 9 (2017) S131-S290