Second surgery after vertical paramedian hemispherotomy for epilepsy recurrence.

Background: Vertical Paramedian Hemispherotomy (VPH) is considered an effective surgical treatment for drug-resistant epilepsy with 80% of patients experiencing seizure freedom or worthwhile improvement. Identifying persistent connective tracts is challenging in failed VPH. Methods: We reviewed our series of consecutive patients undergoing VPH for hemispheric drug-resistant epilepsy and included cases with recurrent epileptic seizures undergoing second surgery with at least 6 months of postoperative follow-up. The cases were extensively assessed to propose a targeted complementary resection. Results: Two children suffering from seizure recurrence following hemispherotomy leading to second surgery were included. After complete assessment, persisting amygdala residue was suspected responsible for the epilepsy recurrence in both patients. Complementary resection of the amygdala residue led to seizure freedom for both patients (Engel IA/ILAE Class 1) without complication. Different diagnostic tools are used to assess patients after failed hemispherotomy including routine EEG, prolonged video EEG, MRI (particularly DTI sequences), SPECT or PET scans and clinical evaluation. These tools allow to rule out epileptic foci in the contralateral hemisphere and to localize a potentially persisting epileptogenic zone. Assessment of these patients should be as systematic and integrated as the initial workup. Although our two patients suffered from Rasmussen's encephalitis, seizure recurrence after VPH has been described in other pathologies. Conclusion: Lying deep and medially in the surgical corridor of VPH, the amygdala can be incompletely resected and cause recurrent epilepsy. Complementary selective resection of the amygdala residue may safely lead to success in epilepsy control.

Primary cerebral arteritis in a young girl: an unusual cause of acute recurrent neurological deterioration.

BACKGROUND: Primary angiitis of the central nervous system (PACNS) is an idiopathic, usually recurrent vasculitis confined to the brain. PACNS has been reported rarely in children, although the disease is probably underdiagnosed. CLINICAL CASE: : We report the clinical history of a 3-year-old girl who presented subacute neurological deterioration characterised by headache, speech regression, and altered level of consciousness. Brain MRI revealed severe inflammatory lesions involving both grey and white matters. All blood and cerebrospinal fluid (CSF) tests for inflammatory or infectious processes were negative. Over the next 10 years, the patient relapsed eight times. Brain biopsy confirmed lesions suggestive of cerebral vasculitis. Based on histopathological features and due to the absence of systemic vasculitis, the patient was considered to have PACNS. She developed partial epilepsy, and clinical stabilisation was finally achieved via continuous oral corticosteroids and immunosuppressive agents. CONCLUSION: PACNS may be the cause of subacute and relapsing inflammatory encephalopathy in children after excluding other diagnoses, such as multiple sclerosis, sarcoidosis, recurrent acute disseminated encephalomyelitis (ADEM), and primary central nervous system lymphoma. Brain biopsy is necessary to confirm the diagnosis of PACNS and exclude diseases with similar symptoms. Neurological outcome remains poor.