Session Title: AA 2021 Virtual Posters - Neurological Rehabilitation
Session Time: None. Available on demand.
Disclosures: Carolyn Cao, MD: No financial relationships or conflicts of interest
Case Diagnosis: A 58-year-old female with linear scleroderma “en coup de sabre”
Case Description: A 58-year-old female presented to the emergency department after being found unresponsive. Upon arrival, the patient had 2 generalized tonic-clonic seizures associated with left gaze preference. Post-ictally, she was noted to have left hemiparesis, aphasia, and dysarthria. She was admitted to the neurocritical care unit, where she developed status epilepticus requiring intubation. MRI brain and CT head showed right cerebral hemispheric swelling with right frontal dural enhancement. After titrating anti-epileptic drugs, her seizure activity decreased, and her mental status improved. She was admitted to acute inpatient rehabilitation, where she was noted to have an atrophic scar on her right forehead with associated alopecia.
Setting: Acute inpatient rehabilitation hospitalAssessment/
Results: “En coup de sabre” is a rare subset of linear scleroderma involving the frontoparietal area of the head and associated with neurologic abnormalities such as ipsilateral intracranial lesions and epilepsy. The characteristic skin lesion is described as band-like sclerosis resembling the stroke of a saber. The mean age of onset of “en coup de sabre” is 13 years old, and the majority of patients with linear scleroderma are diagnosed before the age of 18.
Discussion: Rheumatology and dermatology were consulted, and the patient was clinically diagnosed with linear scleroderma “en coup de sabre.” She underwent a punch biopsy with resulting pathology that was nonspecific, which can be seen in morphea. Given the severity of her seizures and imaging findings, she was recommended to start empiric treatment with methotrexate.
Conclusion: This is a rare diagnosis of linear scleroderma in an adult with neurologic manifestation of new onset status epilepticus. While the patient had evidence of cutaneous findings early in life, she had no neurologic complications until this presentation.
Level of Evidence: Level V
To cite this abstract in AMA style:Cao C, Kearse T. A Rare Diagnosis of Linear Scleroderma “en Coup De Sabre” in an Adult with New Onset Status Epilepticus: A Case Report [abstract]. PM R. 2021; 13(S1)(suppl 1). https://pmrjabstracts.org/abstract/a-rare-diagnosis-of-linear-scleroderma-en-coup-de-sabre-in-an-adult-with-new-onset-status-epilepticus-a-case-report/. Accessed December 3, 2023.
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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/a-rare-diagnosis-of-linear-scleroderma-en-coup-de-sabre-in-an-adult-with-new-onset-status-epilepticus-a-case-report/