Puthenparampil Marco, Poggiali Davide, Causin Francesco, Rolma Giuseppe, Rinaldi Francesca, Perini Paola, Gallo Paolo
Department of Neurosciences NPSRR, Multiple Sclerosis Centre Veneto Region, First Neurology Clinic, University of Padova, Italy
The Multiple Sclerosis Centre of the Veneto Region of Italy (CeSMuV), Padova, Italy.
Mult Scler. 2016 Aug;22(9):1184-91. doi: 10.1177/1352458514564483. Epub 2015 Mar 19.
Multiple sclerosis (MS) is a white and grey matter disease of the central nervous system (CNS). It is recognized that cortical damage (i.e. focal lesions and atrophy) plays a role in determining the accumulation of physical and cognitive disability that is observed in patients with progressive MS. To date, an association of cortical lesions with clinical relapses has not been described.
We report clinical and magnetic resonance imaging (MRI) findings of five relapsing-remitting MS (RRMS) patients who had clinical relapses characterized by the acute appearance of cortical symptoms, due to the development of large, snake-like, cortical inflammatory lesions. Symptoms were: acute Wernicke's aphasia mimicking stroke; agraphia with acalculia, not associated to a motor deficit nor linguistic disturbance; hyposthenia of the left arm, followed by muscle twitching of the hand, spreading to arm and face; acute onset of left lower limb paroxysmal hypertonia; and temporal lobe status epilepticus, with psychotic symptoms.
Cortical relapses may occur in MS. MRI examination in MS should include sequences, such as double inversion recovery (DIR) or phase sensitive inversion recovery (PSIR), that are aimed at visualizing cortical lesions, especially in the presence of symptoms of cortical dysfunction. Our observation further stresses and extends the clinical relevance of cortical pathology in MS.
多发性硬化症(MS)是一种中枢神经系统(CNS)的白质和灰质疾病。人们认识到,皮质损伤(即局灶性病变和萎缩)在决定进展型MS患者中出现的身体和认知残疾的累积方面发挥作用。迄今为止,尚未描述皮质病变与临床复发之间的关联。
我们报告了5例复发缓解型MS(RRMS)患者的临床和磁共振成像(MRI)结果,这些患者的临床复发表现为皮质症状急性出现,原因是出现了大的、蛇形的皮质炎性病变。症状包括:类似中风的急性韦尼克失语症;失写症伴失算症,与运动缺陷或语言障碍无关;左臂肌力减退,随后手部肌肉抽搐,并蔓延至手臂和面部;左下肢急性发作阵发性张力亢进;以及伴有精神症状的颞叶癫痫持续状态。
MS可能发生皮质复发。MS的MRI检查应包括旨在显示皮质病变的序列,如双反转恢复(DIR)或相位敏感反转恢复(PSIR),特别是在存在皮质功能障碍症状的情况下。我们的观察进一步强调并扩展了MS中皮质病理的临床相关性。