1Lilia Megherbi, 1Hakim Si Ahmed, 1Smail Daoudi
1Tizi Ouzou University Hospital, Tizi Ouzou, Algeria
Introduction:
Multiple sclerosis (MS) is an autoimmune demyelinating disease of the central nervous system (CNS). Several associations with MS have been described, including epilepsy but not a particular epileptic syndrome, such as Lennox-Gastaut syndrome. We report an exceptional presentation of MS in a 14-year-old girl child with a childhood history of Lennox-Gastaut syndrome.
Material(s) and Method(s):
A 14-year-old female child presented with a rapid onset left hemiparesis. She was born into a second-degree consanguineous marriage and was diagnosed with West syndrome at the age of 8 months which then progressed to Lennox Gastaut encephalopathy. A brain MRI at the age of four was without abnormalities.
Her epilepsy was relatively stable under treatment with motor and cognitive sequelae.
At the age of 14, she presented a left hemiparesis worsening over few days. Her brain MRI showed figure 1. CSF analysis showed neither hyperproteinorachia nor the oligoclonal band. Antibodies to HIV and viral hepatitis, anti AQP4, anti-MOG, anti NMDA, anti-GABA were negative. The biotinidase activity and autoimmunity tests were correct.
The patient received IV methylprednisolone (1g/day) for three days with a remarkable clinical improvement. Five months later, the left hemiparesis reoccurred. A brain and spinal cord MRI showed a significant increase in the number of lesions with the presence of active lesions (figure 2).
The patient had another relapse after six months, made of left arm weakness with an exacerbation of her seizures. Brain MRI showed the presence of multiple active lesions (figure 3).
Our patient’s clinical and radiological presentation fulfilled the Mc Donald 2017 Criteria of RRMS. Therefore, the diagnosis of MS was retained after ruling out other differential diagnoses, mainly MOGopathy.
Result(s):
MS is a CNS demyelinating disease that is both complex and heterogeneous. Although the disease is characterized by inflammatory lesions in the white matter, various neuropathological and radiological studies demonstrated that it also affects grey matter. Several studies showed that seizures are three to six times more common among MS patients than in the general population. Even though MS can start with epilepsy and that seizure may be the only symptom of an MS relapse, it is still unclear whether the two diseases coexist or whether MS predisposes to seizures or vice versa.
Our patient had a history of classic West syndrome with no acquired aetiology, which progressed to a typical Lennox-Gastaut syndrome, with a normal brain MRI and absence of extra neurological abnormalities. The MRI and clinical presentation at the age of 14 were highly indicative of MS. Despite the fact that these two diseases are utterly different in terms of onset age and clinical and para-clinical manifestations, their co-occurrence in one patient is perplexing, as it is unclear whether this is a coincidence or if there are any common predisposing genetic factors, especially considering the parents’ consanguinity.
Conclusion(s):
MS and Lennox-Gastaut syndrome are two fundamentally different neurological diseases with regard to their physiopathological, clinical, and paraclinical features. Yet genetic factors are frequently reported in these two conditions, opening up new prospects for research in this field.