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Author Spotlight: Studying Clinical Characters and Epilepsy Outcomes After Frontal Disconnection in Patients with MOGHE
Published on: August 16, 2024
Confusing Onset of MOGAD in the Form of Focal Seizures
Małgorzata Jączak-Goździak1, Barbara Steinborn1
1Department of Developmental Neurology, Poznan University of Medical Sciences, 61-701 Poznań, Poland.
Abstract:
MOGAD is a demyelinating syndrome with the presence of antibodies against myelin oligodendrocyte glycoprotein, which is, next to multiple sclerosis and the neuromyelitis optica spectrum, one of the manifestations of the demyelinating process, more common in the pediatric population. MOGAD can take a variety of clinical forms: acute disseminated encephalomyelitis (ADEM), retrobulbar optic neuritis, often binocular (ON), transverse myelitis (TM), or NMOSD-like course (neuromyelitis optica spectrum disorders), less often encephalopathy. The course may be monophasic (40-50%) or polyphasic (50-60%), especially with persistently positive anti-MOG antibodies. Very rarely, the first manifestation of the disease, preceding the typical symptoms of MOGAD by 8 to 48 months, is focal seizures with secondary generalization, without typical demyelinating changes on MRI of the head. The paper presents a case of a 17-year-old patient whose first symptoms of MOGAD were focal epileptic seizures in the form of turning the head to the right with the elevation of the left upper limb and salivation. Seizures occurred after surgical excision of a tumor of the right adrenal gland (ganglioneuroblastoma). Then, despite a normal MRI of the head and the exclusion of onconeural antibodies in the serum and cerebrospinal fluid after intravenous treatment, a paraneoplastic syndrome was suspected. After intravenous steroid treatment and immunoglobulins, eight plasmapheresis treatments, and the initiation of antiepileptic treatment, the seizures disappeared, and no other neurological symptoms occurred for nine months. Only subsequent relapses of the disease with typical radiological and clinical picture (ADEM, MDEM, recurrent ON) allowed for proper diagnosis and treatment of the patient both during relapses and by initiating supportive treatment. The patient's case allows us to analyze the multi-phase, clinically diverse course of MOGAD and, above all, indicates the need to expand the diagnosis of epilepsy towards demyelinating diseases: determination of anti-MOG and anti-AQP4 antibodies.
Insights
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) can present with initial focal seizures before typical demyelinating symptoms. Early diagnosis requires considering MOGAD in epilepsy evaluations.
Area of Science:
- Neurology
- Immunology
- Demyelinating Diseases
Background:
- Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a demyelinating disorder, distinct from multiple sclerosis and neuromyelitis optica spectrum disorders.
- MOGAD commonly affects the pediatric population and presents with diverse clinical manifestations including acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), and transverse myelitis (TM).
Observation:
- A rare case of MOGAD in a 17-year-old initiated with focal epileptic seizures preceding typical demyelinating signs by months, occurring post-adrenal tumor surgery.
- Initial presentation mimicked paraneoplastic syndrome due to normal brain MRI and negative onconeural antibodies, complicating early diagnosis.
Findings:
- The patient experienced polyphasic MOGAD relapses with classic radiological and clinical features (ADEM, MDEM, recurrent ON) after the initial seizure episode.
- Despite initial misdiagnosis, subsequent relapses confirmed MOGAD, leading to appropriate treatment including steroids, immunoglobulins, and plasmapheresis.
Implications:
- This case highlights the critical need to include MOGAD and neuromyelitis optica spectrum disorder (NMOSD) antibody testing (anti-MOG and anti-AQP4) in the differential diagnosis of new-onset epilepsy, especially in adolescents.
- Recognizing atypical presentations of MOGAD, such as focal seizures, is crucial for timely diagnosis and management of this demyelinating syndrome.
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