S194 S290
Poster 193:
Dramatic Improvement in Functional and Mental
Status in a Patient with Bing-Neel Syndrome Treated
With Ibrutinib: A Case Report
Megan Loftsgaarden (National Capital Consortium PM&R Program,
Bethesda, MD, USA)
Disclosures:
Megan Loftsgaarden: I Have No Relevant Financial
Relationships To Disclose
Case/Program Description:
A 71-year-old Caucasian man, previ-
ously functioning at modified independence with ADLs and general
mobility with history of splenic marginal zone lymphoma treated
with splenectomy and 6 cycles of bendamustine/Rituxan presented
with bilateral lower extremity weakness with 5 falls in 9 days.
During this admission, he exhibited waxing and waning mental
status and a grand mal seizure. MRI and lumbar puncture confirmed
diagnosis of Waldenstrom Macroglobulinemia with CNS involve-
ment: Bing-Neel Syndrome. He showed rapid improvement with
oral ibrutinib. He reached modified independence level of function
after acute inpatient rehabilitation stay and returned to home. He
was re-admitted to the ICU 3 months later for sepsis due to
C.difficile
, requiring subtotal colectomy with ileostomy and
discontinuation of ibrutinib. He was dependent for transfers and
ADLs, requiring nasogastric tube feedings; and at modified assis-
tance for bed mobility.
Setting:
Tertiary Care Hospital.
Results:
Within 7 days of resumption of modified consistency
ibrutinib, he was tolerating a regular consistency diet and
oral medications. Within 3 weeks, he was functioning at
supervision level for eating, grooming, dressing; minimum assis-
tance for toileting, supervision for bed mobility and transfers, and
walking short distances with rolling walker, and returned to his
home setting.
Discussion:
Bing-Neel is an uncommon complication of Waldenstrom
Macroglobulinemia, and ibrutinib is a non-standard treatment for CNS
involvement of this chronic lymphoproliferative disease. This patient
had great increase in his rehabilitative potential following initiation of
this medication on two separate occasions.
Conclusions:
Novel treatments and administration of biologic and
chemotherapy agents can improve cognitive and functional status,
thereby improving quality of life, for cancer rehabilitation
patients.
Level of Evidence:
Level V
Poster 194:
Management of Severe Autonomic Instability in a
Patient with Multiple System Atrophy and a
Concurrent Blood Pressure Cap: A Case Report
Mark A. Linsenmeyer, MD (University of Pittsburgh Medical Center),
Julie Lanphere, DO
Disclosures:
Mark Linsenmeyer: I Have No Relevant Financial Re-
lationships To Disclose
Case/Program Description:
A 74-year-old man with multiple system
atrophy (MSA) was admitted to inpatient rehabilitation (IPR) following
a right thalamic hemorrhagic stroke and labile blood pressures. He had
a longstanding history of syncope from orthostasis, as well as aortic
dissection with a permanent systolic blood pressure (SBP) cap of 140.
SBP was 210 upon arrival to acute care and remained persistently
above his cap especially while supine, despite multiple medication
adjustments. During IPR, orthostasis significantly limited ambulation
tolerance. Multiple interventions were implemented and allowed a
successful IPR program. Home fludrocortisone was continued for
orthostasis and antihypertensive medication administration was
spaced throughout the day for more stable blood pressures. Propran-
olol 10mg Q6H was successfully trialed, as it is well known for crossing
the blood brain barrier providing stability in autonomic storming. Staff
was instructed to transfer the patient very slowly and to maintain the
head of his bed at greater than 45 degrees while supine. Compression
stockings and abdominal binders were utilized when the patient was
out of bed. To avoid postprandial hypotension, no therapies were
scheduled within 30 minutes of meals.
Setting:
Tertiary care academic hospital.
Results:
Prior to admission the patient required a wheelchair for
community mobility due to severe orthostasis, yet also had poorly
controlled hypertension resulting in a hemorrhagic stroke. With the
above interventions, he successfully ambulated 300 feet with no
assistive device. Blood pressures remained labile and changes in
position were found to have the greatest immediate effect on raising
or lowering his pressures.
Discussion:
In a patient with MSA with severe autonomic instability
and a SBP cap, multiple interventions improved ambulation tolerance
and reduced symptoms of orthostasis, allowing the patient to progress
with functional goals.
Conclusions:
In patients with autonomic instability, careful medical
management and supportive interventions may improve safety, blood
pressure control, and participation in daily activities.
Level of Evidence:
Level V
Poster 195:
Rehabilitation After Toxic Epidermal Necrolysis
Secondary to Adalimumab: A Case Report
Max B. Hurwitz, DO (Univ of WA Med Ctr), Jennifer M. Zumsteg, MD,
FAAPMR
Disclosures:
Max Hurwitz: I Have No Relevant Financial Relationships
To Disclose
Case/Program Description:
A 42-year-old man with toxic epidermal
necrolysis (TEN) secondary to Adalimumab with 95% total body
surface area (TBSA) involvement. The patient received one dose of
Adalimumab for a new diagnosis of ulcerative colitis. He began
experiencing a rash which evolved into painful skin blistering and
sloughing. At the time of admission to our regional burn center his
skin involvement was classified as greater than 95% TBSA, with oral
and genitourinary mucosal involvement. He received xenografting
to the anterior and posterior torso, arms, legs and feet. For his
ulcerative colitis, he was started on a high dose steroid taper and
azathioprine. The functional impact of his TEN, associated treat-
ment, and prolonged intensive care unit stay, included profound
deconditioning with poor cardiovascular, pulmonary and musculo-
skeletal endurance. At admission to acute inpatient rehabilitation
impairments included balance and mobility, upper and lower limb
range of motion restrictions, proximal and distal weakness, and
poor exercise tolerance.
Setting:
Tertiary Care Hospital and Acute Inpatient Rehabilitation.
Results:
Following 14 days of acute inpatient rehabilitation his bal-
ance, endurance and strength had improved; he ambulated with a 4-
wheeled walker, and was modified independent for ADLs. He received
training to adjust to the role of caregiver and was excited to return
home to his 8-year-old son.
Discussion:
Cutaneous reactions are documented in 10-20% of pa-
tients started on TNF-alpha inhibitors, with the most common being
injection site reactions. Severe reactions, such as TEN, are less com-
mon but can have severe morbidity and result in functional impair-
ments appropriate to target with rehabilitation therapies. With
increased use of these medications for the management of inflam-
matory conditions, physiatrists should be aware of these risks and be
able to offer essential care facilitating recovery.
Conclusions:
While still rare, TEN can result in significant morbidity
affecting multiple systems, requiring a comprehensive interdisci-
plinary team for optimal functional recovery.
Level of Evidence:
Level V
S194
Abstracts / PM R 9 (2017) S131-S290