Pediatric stiff-person syndrome with renal failure

Kumar, M Veerendra; Savida, P

doi:10.4103/0976-3147.165352

Cited by 8 publications

(4 citation statements)

References 6 publications

(19 reference statements)

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“…2 This is the third report of a rituximab trial in a child with SPS, with a positive response in both prior cases. 4,6 Our patient presented with primarily asymmetric leg involvement, with less prominent trunk symptoms, which while less typical of classic SPS, it is described. 2,8 One reason for this difference may also be that our patient was diagnosed relatively early, after less than a month of symptoms.…”

mentioning

confidence: 76%

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Adolescent Stiff Person Syndrome: Long-Term Symptom Remission on Immunomodulatory Therapy

Ogilvie

Kolski

2019

Can. J. Neurol. Sci.

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confidence: 76%

“…1 Childhood SPS with GAD seropositivity is rarely reported, with 11 GAD seropositive cases in total in the literature. [2][3][4][5][6] Three of these demonstrated glycine receptor alpha-1 subunit autoantibodies, though this testing was not readily available. 2 Our patient has had an essentially complete response to immunotherapy.…”

mentioning

confidence: 99%

Adolescent Stiff Person Syndrome: Long-Term Symptom Remission on Immunomodulatory Therapy

Ogilvie

Kolski

2019

Can. J. Neurol. Sci.

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“…Published studies regularly report the prevalence of SPS to range from 0.5 to 2 cases per million habitants [ 3 , 8 , 14 , 15 ]; however, to date, few population-based studies have actually been conducted to estimate the true burden of SPS [ 8 , 13 , 14 ]. Between 2000 and 2015, the British Neurological Surveillance Unit identified 119 individuals with SPS from the United Kingdom, suggesting a disease prevalence of 1–2 cases per million people [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Inpatient care for stiff person syndrome in the United States: a nationwide readmission study

et al. 2018

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BackgroundStiff person syndrome (SPS) is a progressive neurological disorder characterized by axial muscle rigidity and involuntary spasms. Autoimmune and neoplastic diseases are associated with SPS. Our study objectives were to describe inpatient care for SPS in the United States and characterize 30-day readmissions.MethodsWe queried the 2014 Nationwide Readmission Database for hospitalizations where a diagnosis of SPS was recorded. For readmission analyses, we excluded encounters with missing length of stay, hospitalization deaths, and out-of-state and December discharges. National estimates of index hospitalizations and 30-day readmissions were computed using survey weighting methods. Unconditional logistic regression was used to examine associations between demographic, clinical, and hospital characteristics and readmission.ResultsThere were 836 patients with a recorded diagnosis of SPS during a 2014 hospitalization. After exclusions, 703 patients remained, 9.4% of which were readmitted within 30 days. Frequent reasons for index hospitalization were SPS (27.8%) and diabetes with complications (5.1%). Similarly, readmissions were predominantly for diabetes complications (24.2%) and SPS. Most readmissions attributed to diabetes complications (87.5%) were to different hospitals. Female sex (OR, 3.29; CI: 1.22–8.87) and routine discharge (OR, 0.26; CI: 0.10–0.64) were associated with readmission, while routine discharge (OR, 0.18; CI: 0.04–0.89) and care at for-profit hospitals (OR, 10.87; CI: 2.03–58.25) were associated with readmission to a different hospital.ConclusionsReadmissions in SPS may result from disease complications or comorbid conditions. Readmissions to different hospitals may reflect specialty care, gaps in discharge planning, or medical emergencies. Studies are required to determine if readmissions in SPS are preventable.

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“…In some cases, continuous long‐lasting or frequent attacks of spasms (spasmodic storm) may compromise respiration and result in myoglobinuria with renal failure . It may be accompanied by severe dysautonomia with hypertensive crises and tachycardia.…”

Section: Cautions and Caveats In The Management Of Spsdmentioning

confidence: 99%

Pragmatic Treatment of Stiff Person Spectrum Disorders

Balint

Meinck

2018

Movement Disord Clin Pract

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Background Stiff person spectrum disorders (SPSD) are a group of rare conditions clinically characterized by stiffness, spasms, and heightened stimulus sensitivity. They also share a spectrum of antibodies. Methods We reviewed the literature and our own experience with the aim of providing a practical approach to the treatment of SPSD. Results Because of the rarity of SPSD, there is little evidence to guide treatment decisions. The treatment of SPSD is based on the triad of symptomatic treatment, immunotherapy, and tumor treatment where appropriate. Moreover, the management involves continuous and appropriate monitoring of the symptoms of the disease, its autoimmune associations, and potential treatment side effects. Conclusions Here we delineated a pragmatic treatment approach to SPSD, based on our experience and existing literature. We also highlighted how our understanding of neuronal antibodies and their implications reflects on management considerations.

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Pediatric stiff-person syndrome with renal failure

Cited by 8 publications

References 6 publications

Adolescent Stiff Person Syndrome: Long-Term Symptom Remission on Immunomodulatory Therapy

Adolescent Stiff Person Syndrome: Long-Term Symptom Remission on Immunomodulatory Therapy

Inpatient care for stiff person syndrome in the United States: a nationwide readmission study

Pragmatic Treatment of Stiff Person Spectrum Disorders

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