J Musculoskelet Neuronal Interact 2021; 21(2):322-325
Journal of Musculoskeletal
and Neuronal Interactions
Case Report
Rehabilitation in paraneoplastic stiff-person syndrome –
Case Report
Bilinc Dogruoz Karatekin1, Seyma Nur Sahin1, Afitap İcagasioglu2
Istanbul Medeniyet University Goztepe Training and Research Hospital, Physical Medicine and Rehabilitation, Istanbul, Turkey;
Istanbul Medeniyet University, Faculty of Medicine, Department of Physical Medicine and Rehabilitation, Turkey
1
2
Abstract
We aimed to share our rehabilitation experience in a patient diagnosed with paraneoplastic Stiff-person syndrome(SPS).
A 45-year-old female patient was admitted to neurology with the complaint of widespread painful contractions. EMG was
evaluated in favor of SPS. Amphiphysin-antibody was +++ in CSF. Patients’ treatment was arranged and transferred to
rehabilitation inpatient-clinic.The patient was included in the rehabilitation program of range of motion,stretching,strength
ening, posture&walking exercises, balance&coordination exercises, 5 days/week for 3 months. The patient was screened
for breast cancer, diagnosed with invasive breast carcinoma and underwent mastectomy. With the rehabilitation, the
patient was mobilized first in the parallel-bar then with tripod-cane in the following months. Significant improvements were
found in functional status and quality of life with control of spasticity and mobilization. Although the primary treatment of
paraneoplastic SPS is cancer treatment, significant gains have been achieved with rehabilitation. It is necessary to raise
awareness of the importance of rehabilitation to physicians who diagnose the disease.
Published under Creative Common License CC BY-NC-SA 4.0 (Attribution-Non Commercial-ShareAlike)
Keywords: Case Report, Paraneoplastic, Rehabilitation, Spasticity, Stiff Person Syndrome
Introduction
Stiff-person syndrome (SPS) is a rare neuroimmunological
disease in the axial muscles causing progressive muscle
stiffness, rigidity, and spasm, leading to a significant limitation
in ambulation1. Nowadays the diagnosis of the disease can be
made more easily by identifying the autoantibodies causing
the disease. The disease is usually associated with higher
rates of antibodies to Glutamic acid decarboxylase (AntiGAD Ab)2. But there is also a variant with antibody against
amphiphysin, which is mostly associated with paraneoplastic
diseases, particularly breast carcinoma. Clinically, these
two types have been shown to differ from each other.
Amphiphysin-associated SPS is more common in women,
more associated with breast ca, which rigidity and spasm are
more common, neck or arm involvement is higher, and BDZ
response is higher3.
SPS patients have difficulties in their daily activities and
their quality of life deteriorates due to axial muscle spasms
that also spread to the appendicular areas in some cases,
decreased range of motion and ambulation restrictions.
There are many options in the medical treatment of the
disease such as benzodiazepine, baclofen, steroid, IVIG,
plasmapheresis, rituximab. However, the literature on the
rehabilitation of the disease is quite limited. We aimed to
share our rehabilitation experience in a patient diagnosed
with paraneoplastic SPS.
Case presentation
The authors have no conflict of interest.
Corresponding author: Bilinc Dogruoz Karatekin, M.D., Merdivenköy
mahallesi Göztepe Eğitim ve Araştırma Hastanesi Merdivenköy Poliklinikleri
G Blok Fizik Tedavi Ünitesi , 34730 Kadıköy, İstanbul, Turkey
E-mail: bilincdogruoz@hotmail.com
Edited by: G. Lyritis
Accepted 11 November 2020
A 45-year-old female patient without a known history of
chronic disease was admitted to our hospital’s neurology
clinic with the complaint of widespread painful muscle
contractions. The contractions were triggered by emotional
stress. Contractions were progressively increased and
within 1 month the patient became bed-dependent due to
the spasms in lower back and both ankles. In the neurology
clinic, EMG performed for etiological research, the presence
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B.D. Karatekin et al.: Rehabilitation in paraneoplastic stiff-person syndrome – Case Report
Figure 1. Patients ankle plantar flexed and inverted due to spasticity of the gastrocnemius and soleus muscles.
Figure 2. Balance and coordination exercises on parallel bar (weight bearing, weight shifting and stepping).
of continuous MUAP activity at rest accompanied by spasm
in the right lower extremity was evaluated in favor of stiffperson syndrome. Amphiphysin antibody was detected as
+++ in paraneoplastic panel search in CSF. Patients’ treatment
regime was arranged as 1000mg pulse methylprednisolone
for 5 days, followed by methylprednisolone 64 mg/day,
IVIG/5 days, diazepam 10 mg/day and baclofen 40 mg/day
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and patient was transferred to rehabilitation inpatient clinic.
The neurological examination of the patient on admission
to rehabilitation inpatient clinic; Patient was conscious,
oriented, and cooperative. Muscle strength evaluated via
manual muscle test with standard technique (score from 0 to
5 /5)4: Left pectoralis major 4/5, biceps brachii 4/5, triceps
5/5, brachialis 5/5, wrist flexors and extensors 5/5, upper
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B.D. Karatekin et al.: Rehabilitation in paraneoplastic stiff-person syndrome – Case Report
Figure 3. Posture of the patient before discharge.
right 5/5 throughout, lower right 3/5, lower left 3/5. Lower
extremity selective muscle strength examination could
not be performed due to spasticity. Sensory examination:
Superficial and deep sense intact. The patient’s bilateral
hip flexion (R: 100°, L: 95°), bilateral knee flexion (R: 135°,
L: 95°), bilateral ankle dorsiflexion (R: 5°, L: 15°), right
shoulder abduction (45°) and left shoulder flexion (90°),
extension (40°) and abduction (90°) movements were
limited. The patient had sitting balance partially and was
immobile out of bed due to lumbar extensor spasticity and
balance-coordination disorder. In the spasticity evaluation,
in addition to the lumbar extensor muscles, there was MAS
2, Tardieu 3 spasticity in the bilateral gastrocnemius and
soleus muscles, and MAS 1+ spasticitiy in the left hip flexor
muscles (Figure 1).
Functional status of the patients was evaluated with
Funtional Independance Measure (FIM). Total score of the
patient was 66. Quality of life of the patient was evaluated
with SF-36. SF-36 provides a self-reported health status
profile consisting of eight dimensions. Results of the patient:
Physical functioning was 15%, role limitations due to physical
problems was 75%, bodily pain was 22.5%, general health
perceptions was 40% , vitality was 5%, social functioning
was 25%, role limitations due to emotional problems was
66.7%, and mental health was 28%.
Since the patient was using 64 mg/day methylprednisolone,
the patient’s bone mineral density measurement with Dual
energy x-ray absorptiometry (DEXA) and laboratory tests
were performed. Bone mineral density measurement was
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within normal range, Ca: 9,1 mg/dL, P:2,9 mg/dL, 25-OHVitD:
7,4 ng/mL, PTH: 150,9 pg/mL. 1000 mg/day calcium and
600 IU/day vitamin D supplement was given to the patient.
The patient was included in the rehabilitation program
of active range of motion of shoulder, hip, knee and ankle,
gastrosoleus and hip stretching (iliopsoas/hamstring),
strengthening of abdominal muscles (knee to chest,
isometric abdominal exercises, pelvic tilt), posture and gait
retraining (symmetrical weight bearing, weight shifting,
stepping, single leg standing, push off – calf rise) balance and
coordination exercises (heel rise, side stepping, single leg
standing, backwards walking) 5 days a week for 2 months
(Figure 2).
Meanwhile, the patient was screened for breast cancer
with a prediagnosis of paraneoplastic stiff person due to the
antibody positivity to amphiphysin. The patient was diagnosed
with invasive breast carcinoma by breast ultrasound and
subsequent breast biopsy. The patient underwent simple
mastectomy, in consultation with general surgery, and was
transferred back to our inpatient clinic. The rehabilitation
program continued for another month in the same order. With
the rehabilitation program, the patient was mobilized in the
parallel bar at the end of the first month, and subsequently
with a walker and a tripod cane in the following 2 months.
The neurological examination of the patient on discharge
from rehabilitation inpatient clinic; The patient was
counscious, cooperative and oriented. Muscle strength: upper
right 5/5, upper left 5/5, lower right 5/5, lower left 5/5.
Sensory examination: Superficial and deep sense intact. The
patient had no limitation in range of motion and no spasticity
was detected (Figure 3). FIM total score of the patient was
88. SF-36 results of the patient: Physical functioning was
65%, role limitations due to physical problems were 0%,
bodily pain was 22%, general health perceptions were
50% , vitality was 55%, social functioning was 37.5%, role
limitations due to emotional problems were 0%, and mental
health was 84%.
The patient’s drug doses were reduced gradually,
and she was discharged with baclofen 10 mg/day and
methylprednisolone 48 mg/day. Control EMG performed
after discharge has been interpreted as; Compared to
baseline EMG, there was no pathological spontaneous
MUAP activity, and this has been interpreted as noticeably
improving condition.
Discussion
Paraneoplastic SPS accounts for approximately 10% of all
SPS patients and is highly associated with breast cancer, lung
cancer and Hodgkin lymphoma5.
The disease usually begins insidiously. As the disease
progresses, spasticity develops in the lumbar spinal muscles
characteristic of the disease and causes hyperlordosis6.
Rigidity can spread to the hips and proximal extremities.
However, in our case, as the hyperlordotic posture regressed
early with oral treatments and rehabilitation, spasticity in the
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B.D. Karatekin et al.: Rehabilitation in paraneoplastic stiff-person syndrome – Case Report
gastrocnemius and soleus muscles was more resistant and
took longer to decrease.
Since SPS is a rare disease, the literature is very limited.
Case reports in the literature mostly focused on diagnosis
and medical treatment of the disease. Due to rarity of the
disease, there is no specific protocol for rehabilitation and
physical therapy. In previous cases, massage, electrotherapy,
hydrotherapy, relaxation and stretching techniques have
been applied at different frequencies and times. In addition,
balance and flexibility exercises were performed in some
cases, and in some cases casting, walker and orthotic
applications were performed7. Also in our case, range of
motion, stretching, strengthening, posture and walking,
balance and coordination exercises were included in the
rehabilitation program of the patient, and the program was
found to be extremely beneficial. Improvements in rigidity,
pain and gait have been demonstrated with Botulinum toxin
administration in patients who do not respond to medical
treatment8. Botulinum toxin application was not deemed
necessary as our patient benefited from cancer treatment,
oral antispasticity treatment and rehabilitation program.
Spasticity and disturbance of posture and gait caused by
SPS affects the functional status and quality of life of the
patients. In the literature, the negative effects of spasticity
on functional status and quality of life have been shown in
studies conducted on many diseases characterized with
spasticity9-11. Functional status and quality of life in our patient
were evaluated with FIM and SF-36 scales respectively,
and significant improvement was found in both scales by
controlling spasticity and mobilization of the patient.
Conclusion
Although the primary treatment of paraneoplastic SPS
is cancer treatment, significant gains have been achieved
with rehabilitation even before cancer treatment. For this
reason, it is necessary to raise awareness of the importance
of rehabilitation to physicians who diagnose the disease,
especially who deals with spasticity patients in clinical
practice. The best results will be obtained by conducting the
treatment of these patients with a multidisciplinary approach
from the moment of diagnosis.
Acknowledgements
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Written consent was obtained from the patient.
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