scholarly journals Stiff-person syndrome with sensorimotor polyneuropathy – case report

2021 ◽  
Vol 21 (1) ◽  
pp. 47-55
Author(s):  
Karol Jastrzębski ◽  
◽  
Nina Romanowska ◽  
Marta Rek-Pacześ ◽  
Łukasz Kępczyński ◽  
...  

Stiff-person syndrome (SPS) is a rare disorder with an estimated prevalence in the general population of 1–2 cases/1,000,000. It is 2–3 times more common in females, with symptom onset at the age of 20–50 years in most cases. Although stiff-person syndrome is associated with antibodies against glutamic acid decarboxylase and amphiphysin, their presence is not necessary for the diagnosis. The treatment should be multidirectional and include immunomodulation, symptomatic treatment as well as monitoring and treatment of overlapping autoimmune, and surgery. We present a case report of a patient diagnosed with stiff-person syndrome overlapping with axonal and demyelinating sensorimotor polyneuropathy. The diagnostic workup indicated diabetes-related polyneuropathy. About 30% of patients diagnosed with stiff-person syndrome also have diabetes. Polyneuropathy alone is rarely reported to overlap with the disorder. In our opinion, polyneuropathy may have a beneficial effect on the clinical presentation of stiff-person syndrome.

2016 ◽  
Vol 7 (01) ◽  
pp. 147-149 ◽  
Author(s):  
M. Veerendra Kumar ◽  
P. Savida

ABSTRACTStiff-person syndrome (SPS) is an autoimmune neuronitis with progressive myoclonus and stiffness. It is a rare but treatable disorder with few case reports in children. SPS is due to autoantibodies against the enzyme glutamic acid decarboxylase which is present in neuronal and nonneuronal tissues. This is the case report of an 8-year-old boy with clinical and investigational features suggestive of SPS with associated myoglobin-induced renal failure, who completely recovered with treatment.


2019 ◽  
Vol 11 (2) ◽  
pp. 217-221
Author(s):  
Kok Pin Yong ◽  
Yew Long Lo

Stiff person syndrome (SPS) is a rare and disabling neurological disorder of autoimmune origin, characterized by progressive stiffness and muscle spasms affecting the axial and limb muscles, most frequently associated with antibodies against glutamic acid decarboxylase. We describe a patient who presented initially with compartment syndrome and was later diagnosed with SPS.This is the first case report of SPS possibly presenting initially with compartment syndrome. This case illustrates the importance of recognizing that patients with SPS may present with varied manifestations, including compartment syndrome, which by itself is a medical emergency.


2020 ◽  
Vol 7 (3) ◽  
pp. e684 ◽  
Author(s):  
Megan E. Esch ◽  
Scott D. Newsome

ObjectiveTo describe 2 cases from a single academic institution of improvement in stiff-person syndrome (SPS) symptoms during pregnancy and to review the clinical outcomes of SPS in 6 additional pregnancies described in the literature.MethodsEvaluation of clinical symptoms and treatment changes of disease state during pregnancy.ResultsSeven patients with 9 pregnancies are described in women with a diagnosis of SPS. Six of 7 (86%) women were positive for glutamic acid decarboxylase (GAD65) antibody. In 5 of 9 (56%) pregnancies, symptomatic medications (antispasmodics) were significantly reduced with stabilization or improvement in symptoms through pregnancy. Nine live, healthy pregnancies resulted. All 7 (100%) women experienced worsening of symptoms after the birth of their children, and symptomatic therapies were resumed and/or increased.ConclusionsThe immune pathogenesis of SPS continues to be explored. Immunomodulatory shifts during pregnancy may influence changes of clinical SPS symptoms and provide insight into the unique pathogenesis of SPS. Some women with SPS may be able to reduce symptomatic medications related to clinical improvement during pregnancy. Women with SPS may safely carry pregnancies to term, delivering healthy and unaffected babies.


2019 ◽  
Vol 8 (1) ◽  
pp. 61-64 ◽  
Author(s):  
Joana Jesus‐Ribeiro ◽  
Alireza Bozorgi ◽  
Modhi Alkhaldi ◽  
Mahmoud Shaqfeh ◽  
Guadalupe Fernandez‐Baca Vaca ◽  
...  

2019 ◽  
Vol 40 (6) ◽  
pp. 1303-1306 ◽  
Author(s):  
Silvia Maria Villa ◽  
Alessandra Rufa ◽  
Alessandro Malandrini ◽  
Alfonso Cerase ◽  
Francesca Rosini ◽  
...  

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