West syndrome and multiple sclerosis association
Introduction. West syndrome is a rare and severe infantile epileptic encephalopathy, beginning around the age of six months, characterized by a classic electro-clinical triad. This is a pathology totally different from multiple sclerosis (MS) which is a demyelinating disease of the central nervous s...
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| Format: | Article |
| Language: | English |
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London Academic Publishing
2023-06-01
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| Series: | Romanian Neurosurgery |
| Subjects: | |
| Online Access: | https://journals.lapub.co.uk/index.php/roneurosurgery/article/view/2448 |
| _version_ | 1797802910586765312 |
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| author | Hakim Si Ahmed Megherbi Lilia Daoudi Smail |
| author_facet | Hakim Si Ahmed Megherbi Lilia Daoudi Smail |
| author_sort | Hakim Si Ahmed |
| collection | DOAJ |
| description | Introduction. West syndrome is a rare and severe infantile epileptic encephalopathy, beginning around the age of six months, characterized by a classic electro-clinical triad. This is a pathology totally different from multiple sclerosis (MS) which is a demyelinating disease of the central nervous system caused, affecting young adults, especially females. The association of these two pathologies has never been described.
Observation. We report here an exceptional presentation of MS in a 14-year-old girl with a history of West syndrome. She had normal development until the age of six months, when she began to have flexion spasms. The diagnosis of West syndrome was made with a normal MRI. The infantile spasms disappeared after treatment with vigabatrin and adrenocorticotropic hormone (ACTH). It had generally progressed to Lennox Gastaut encephalopathy, with delayed psychomotor development and epileptic sequelae. At 14, she presented with left hemiparesis within a few days. A cerebral MRI showed multiple nodular hyperintensities of the supra and infratentorial white matter, with the presence of an active lesion, fulfilling the diagnostic criteria for multiple sclerosis. CSF analysis was normal. Anti-AQP4, anti-MOG, anti-NMDA and anti-GABA (AB) antibodies were absent in the blood. Antibodies against HIV and viral hepatitis were. Biotinidase activity and autoimmunity tests were correct. The patient received high doses of methylprednisolone IV (1g/day) for three days with remarkable clinical improvement after 15 days.
Discussion. MS is a complex and heterogeneous central nervous system (CNS) demyelinating disease. It is not uncommon for epilepsy to be the first symptom of multiple sclerosis. Seizures, on the other hand, are more common after disease progression. Although the disease is characterized by inflammatory lesions of the white matter, various neuropathological and radiological studies have shown that the disease also affects the grey matter. Several studies have shown that seizures are three to six times more common in MS patients than in the general population. Even though MS can start with epilepsy and a seizure may be the only symptom of a relapse of MS, it is still not known whether the two diseases coexist or whether MS predisposes to seizures.
Conclusion. The association of these two totally different pathologies can lead us to say that the mechanism of multiple sclerosis may begin in childhood and that the clinical signs appear in adulthood.
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| first_indexed | 2024-03-13T05:13:56Z |
| format | Article |
| id | doaj.art-3d7b26a7c08c4cc685876d7064e92e86 |
| institution | Directory Open Access Journal |
| issn | 1220-8841 2344-4959 |
| language | English |
| last_indexed | 2024-03-13T05:13:56Z |
| publishDate | 2023-06-01 |
| publisher | London Academic Publishing |
| record_format | Article |
| series | Romanian Neurosurgery |
| spelling | doaj.art-3d7b26a7c08c4cc685876d7064e92e862023-06-16T01:16:45ZengLondon Academic PublishingRomanian Neurosurgery1220-88412344-49592023-06-01372West syndrome and multiple sclerosis associationHakim Si Ahmed Megherbi LiliaDaoudi SmailIntroduction. West syndrome is a rare and severe infantile epileptic encephalopathy, beginning around the age of six months, characterized by a classic electro-clinical triad. This is a pathology totally different from multiple sclerosis (MS) which is a demyelinating disease of the central nervous system caused, affecting young adults, especially females. The association of these two pathologies has never been described. Observation. We report here an exceptional presentation of MS in a 14-year-old girl with a history of West syndrome. She had normal development until the age of six months, when she began to have flexion spasms. The diagnosis of West syndrome was made with a normal MRI. The infantile spasms disappeared after treatment with vigabatrin and adrenocorticotropic hormone (ACTH). It had generally progressed to Lennox Gastaut encephalopathy, with delayed psychomotor development and epileptic sequelae. At 14, she presented with left hemiparesis within a few days. A cerebral MRI showed multiple nodular hyperintensities of the supra and infratentorial white matter, with the presence of an active lesion, fulfilling the diagnostic criteria for multiple sclerosis. CSF analysis was normal. Anti-AQP4, anti-MOG, anti-NMDA and anti-GABA (AB) antibodies were absent in the blood. Antibodies against HIV and viral hepatitis were. Biotinidase activity and autoimmunity tests were correct. The patient received high doses of methylprednisolone IV (1g/day) for three days with remarkable clinical improvement after 15 days. Discussion. MS is a complex and heterogeneous central nervous system (CNS) demyelinating disease. It is not uncommon for epilepsy to be the first symptom of multiple sclerosis. Seizures, on the other hand, are more common after disease progression. Although the disease is characterized by inflammatory lesions of the white matter, various neuropathological and radiological studies have shown that the disease also affects the grey matter. Several studies have shown that seizures are three to six times more common in MS patients than in the general population. Even though MS can start with epilepsy and a seizure may be the only symptom of a relapse of MS, it is still not known whether the two diseases coexist or whether MS predisposes to seizures. Conclusion. The association of these two totally different pathologies can lead us to say that the mechanism of multiple sclerosis may begin in childhood and that the clinical signs appear in adulthood. https://journals.lapub.co.uk/index.php/roneurosurgery/article/view/2448West syndromemultiple sclerosisinflammation |
| spellingShingle | Hakim Si Ahmed Megherbi Lilia Daoudi Smail West syndrome and multiple sclerosis association Romanian Neurosurgery West syndrome multiple sclerosis inflammation |
| title | West syndrome and multiple sclerosis association |
| title_full | West syndrome and multiple sclerosis association |
| title_fullStr | West syndrome and multiple sclerosis association |
| title_full_unstemmed | West syndrome and multiple sclerosis association |
| title_short | West syndrome and multiple sclerosis association |
| title_sort | west syndrome and multiple sclerosis association |
| topic | West syndrome multiple sclerosis inflammation |
| url | https://journals.lapub.co.uk/index.php/roneurosurgery/article/view/2448 |
| work_keys_str_mv | AT hakimsiahmed westsyndromeandmultiplesclerosisassociation AT megherbililia westsyndromeandmultiplesclerosisassociation AT daoudismail westsyndromeandmultiplesclerosisassociation |