Susac syndrome and pregnancy: a review of published cases and considerations for patient management

Willekens, Barbara; Kleffner, Ilka

doi:10.1177/1756286420981352

Cited by 8 publications

(6 citation statements)

References 77 publications

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“…Although harmful effects cannot be ruled out definitively, rituximab may be considered in severe cases. 3…”

Section: Discussionmentioning

confidence: 99%

“…Given its rarity, the true incidence and prevalence are not well known, but occurrence during pregnancy or postpartum has been described in about 5% of female patients. 3 As the classical triad is very rarely noticed on initial presentation, in as few as 13% of patients, 1 diagnosis is often challenging. This occurs because eye and ear involvement may be subclinical, and encephalopathy is the presenting symptom in as many as two thirds of cases, 1 precluding the overt expression of the other symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…Given its rarity, the true incidence and prevalence are not well known, but occurrence during pregnancy or postpartum has been described in about 5% of female patients. 3…”

Section: Discussionmentioning

confidence: 99%

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Clinical Reasoning: A 24-Year-Old Pregnant Woman With Headache and Behavioral Change Progressing to Coma

Bernardes,

Pires,

Farinha

et al. 2023

Neurology

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The broad differential diagnosis associated with progressive subacute encephalopathy can be intimidating, especially in a young, pregnant woman. In this case, a 24-year-old woman at 21 weeks of gestation presented with persistent, drug-resistant fronto-parietal headache, with subsequent progressive development of psychomotor lentification and inappropriate behavior. Physical examination was normal, as were routine laboratory parameters and CT findings, and these symptoms were initially interpreted in the context of chronic depression. Later, the patient developed generalized dystonia and fever, with rapid clinical deterioration, depression of consciousness and, eventually, progression to coma. This case emphasizes the complexity and challenges involved in the diagnostic approach to a patient with progressive subacute encephalopathy framed by worsening CNS symptoms. It highlights the clinical considerations and complementary investigation of various etiologies, in a step-by-step approach, ultimately leading to the final diagnosis. Early recognition and appropriate treatment of these conditions can lead to more favorable outcomes, particularly in gestating patients, where prompt intervention is crucial, and where critical decisions may have to be made regarding pregnancy and the safety of treatment options.

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“…Although harmful effects cannot be ruled out definitively, rituximab may be considered in severe cases. 3…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Clinical Reasoning: A 24-Year-Old Pregnant Woman With Headache and Behavioral Change Progressing to Coma

Bernardes,

Pires,

Farinha

et al. 2023

Neurology

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“…Up until now, approximately 500 cases have been reported in the literature [5]. However, the true prevalence and incidence of SuS remain unknown, likely because many cases are misdiagnosed [6].…”

Section: Introductionmentioning

confidence: 99%

Cognitive outcomes in Susac syndrome: A 2‐year neuropsychological follow‐up study

Van Vrekhem,

Miatton,

Hemelsoet

et al. 2024

Euro J of Neurology

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Background and purposeSusac syndrome (SuS) is a rare, autoimmune, neurological disease characterized by a clinical triad of branch retinal artery occlusion, sensorineural hearing loss and encephalopathy. Neuropsychological functioning in SuS is little researched and the prevalence, nature, and evolution over time of cognitive deficits in SuS remain unclear. This study aimed to better understand the long‐term neuropsychological outcomes of patients with SuS.MethodsThirteen patients with SuS (mean [SD] age 39.5 [11.1] years) were enrolled at the Ghent University Hospital by their treating neurologist. The cognitive functioning and emotional well‐being of each patient was evaluated by means of a thorough neuropsychological test battery at baseline and after 2 years. Follow‐up testing after 2 years was performed in 11 patients (mean [SD] age 42.2 [11.5] years).ResultsPatients showed normal neuropsychological test results at a group level, both at baseline and follow‐up testing. Significant improvements over time were found for information processing speed, verbal recognition, and semantic and phonological fluency. Individual test results showed interindividual variability at baseline, with most impairments being in attention, executive functioning and language, which improved after a 2‐year period. In addition, patients reported significantly lower mental and physical well‐being, both at baseline and follow‐up testing.ConclusionsOur results suggest that neuropsychological dysfunction in SuS is limited at a group level and improves over time. Nonetheless, individual test results reveal interindividual variability, making cognitive screening essential. Furthermore, a high psycho‐emotional burden of the disease was reported, for which screening and follow‐up are necessary.

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“…Significant knowledge gaps exist about pregnancy and the course of other less common demyelinating conditions affecting the central nervous system, such as acute demyelinating encephalomyelitis (ADEM), Susac syndrome, and Behçet disease ( table 1-1 8-18 ).…”

Section: Introductionmentioning

confidence: 99%

Pregnancy Management in Multiple Sclerosis and Other Demyelinating Diseases

Bove¹,

Houtchens²

2022

CONTINUUM: Lifelong Learning in Neurology

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PURPOSE OF REVIEW:Multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSDs) are chronic autoimmune demyelinating conditions of the central nervous system often diagnosed in women of childbearing age. Therefore, safe family planning, pregnancy, and postpartum management are important considerations for many patients with MS or NMOSD. RECENT FINDINGS:Many patients with MS can safely become pregnant and remain well throughout pregnancy and the postpartum period with guidance from specialists on treatment planning. During pregnancy, women with NMOSD may face some increased risk of both neurologic and obstetric complications. Recent attention has focused on evaluating the safety of pharmacologic agents during pregnancy and breastfeeding. Unfortunately, care disparities remain common in both MS and NMOSD, and recovery of function is often not optimally managed in the postpartum period.

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Susac syndrome and pregnancy: a review of published cases and considerations for patient management

Cited by 8 publications

References 77 publications

Clinical Reasoning: A 24-Year-Old Pregnant Woman With Headache and Behavioral Change Progressing to Coma

Clinical Reasoning: A 24-Year-Old Pregnant Woman With Headache and Behavioral Change Progressing to Coma

Cognitive outcomes in Susac syndrome: A 2‐year neuropsychological follow‐up study

Pregnancy Management in Multiple Sclerosis and Other Demyelinating Diseases

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