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Título

Dysarthria-clumsy hand syndrome: An atypical presentation of multiple sclerosis relapse

RESUMO

Case presentation:
A 39-years-old woman with a history of hypothyroidism presented in August 2020 with progressive slurred speech. The following day she noticed weakness in her right hand and was evaluated at the emergency room. She was three weeks pregnant according to obstetric ultrasound.
Neurological examination revealed mild weakness of the right upper limb and discrete dysarthria. The remainder of the examination was normal.
Brain magnetic resonance imaging (MRI) revealed a tumefactive lesion on the right corona radiata and rare foci of T2/FLAIR high signal lesions in the white matter, suggesting a demyelinating disease. Cerebrospinal fluid was normal except for the presence of type 2 oligoclonal bands.
Due to her pregnancy and improvement of symptoms, she did not receive any treatment initially, and she had an incomplete recovery. Six months after delivery, the MRI was repeated and showed a new mass lesion involving the left internal capsule. She decided to breastfeed, and a DMD was initiated a year later.
Discussion:
Multiple Sclerosis (MS) is the most common demyelinating disease of the central nervous system, and its typical presentations include optic neuritis, internuclear ophthalmoplegia, and myelitis. Stroke-like presentation of MS is uncommon, with a few cases described in the literature.
Dysarthria-clumsy hand syndrome (DCHS) is typically a lacunar syndrome characterized by unilateral pyramidal signs and speech disturbance. The most frequently described sites of lesions are the internal capsule, corona radiata and pons.
Our patient also had a tumefactive demyelinating lesion (TDL), a rare form of MS relapse. Typical pathological findings of TDLs include abundant foamy macrophages containing myelin debris, reactive astrogliosis, relative axonal preservation, and variable perivascular and parenchymal lymphocytic inflammation.
The TDL could be explained by a procoagulant state that represents an increased innate immune response, probably needful to compensate for reduced cell-mediated immunity inherent to the pregnant state.
Final comments:
There is no description in literature of MS relapse causing DCHS, but the lesion's location seen on MRI and the patient's symptoms are compatible with the syndrome.
This atypical case shows that MS can present in various forms. In this context, the systematic approach is fundamental to diagnosing and expanding the knowledge about this disease.