Pure epileptic headache and related manifestations: a video-EEG report and discussion of terminology.

We present the first video-EEG recording of episodes of "epileptic headache". The case reported is that of a 9-year-old girl with brief episodes (of a few minutes) of severe frontal headache, which corresponded to the presence of concurrent spikes and slow waves, starting in the right temporal area. A dysplastic lesion of the right temporal lobe was observed by MRI and the patient received surgery, with subsequent disappearance of headaches. This case highlights ictal EEG as the main diagnostic tool for epileptic headache. We discuss the terminology regarding this type of manifestation and believe that cases without subsequent epileptic manifestations, as in the present case, should be more appropriately referred to as "pure ictal epileptic headache" or simply "pure epileptic headache". [Published with video sequences].

Conservative treatment of an atlantoaxial degenerative articular cyst: case report.

BACKGROUND: Atlantoaxial degenerative articular cysts are rare lesions that can cause extradural compression of the cervicomedullary junction. When symptomatic, they usually require surgical treatment. We report an unusual case of spontaneous regression of an atlantoaxial degenerative articular cyst after conservative treatment with an external cervical brace along with a systemic therapy with nonsteroidal anti-inflammatory drugs (NSAIDs) and steroids. We also discuss the potential pathogenetic mechanisms involved. PURPOSE: To describe a case of significant volume reduction of an atlantoaxial articular degenerative cyst in a patient treated with a Philadelphia collar and anti-inflammatory drugs. STUDY DESIGN: Case report with analysis of the literature. METHODS: A 80-year-old patient was admitted to our institution with a history of progressive tetraparesis, ataxic gait, and cervical pain. A cervical spine magnetic resonance imaging (MRI) scan showed an extradural mass lesion behind the dens of C2 causing significant compression of the cervicomedullary junction, suggesting the diagnosis of atlantoaxial degenerative articular cyst. The patient refused surgery in favour of a conservative treatment with a Philadelphia collar and a short-term course of NSAIDs and corticosteroids. RESULTS: After 6 weeks, the patient's neurological condition improved, and a 6-month follow-up cervical spine MRI scan revealed an almost complete regression of the atlantoaxial cystic lesion. At a 1-year follow-up, his clinical condition was further improved. CONCLUSIONS: Atlantoaxial articular degenerative cysts are rare lesions that should be included in the differential diagnosis of those extradural lesions that can cause a ventral or ventrolateral compression of the cervicomedullary junction. They most commonly occur in elderly female patients affected by diffuse arthrosic degeneration of the cervical spine, with or without clear radiological signs of atlantoaxial instability, and have a typical appearance on MRI imaging. Surgery, with direct excision of the cyst and/or a C1-C2 fusion, is the first treatment of choice. Nevertheless, our report points out the possibility of a significant spontaneous regression of these lesions following a simple conservative strategy based on the use of an external cervical brace together with a systemic anti-inflammatory therapy.

Demyelinating syndrome in SLE encompasses different subtypes: Do we need new classification criteria? Pooled results from systematic literature review and monocentric cohort analysis.

OBJECTIVE: To describe features of demyelinating syndrome (DS) in systemic lupus erythematosus (SLE). METHODS: A systematic review using a combination of Mesh terms in PubMed and a retrospective analysis of 343 adult patients with SLE were carried out to identify patients with DS. Retrieved cases were classified as affected with DS according to 1999 ACR nomenclature and attributed to SLE by applying the 2015 algorithm. DS defined according to the clinical but not temporal 1999 ACR criteria was classified as clinically isolated syndrome (CIS). RESULTS: Estimated prevalence of DS (including CIS) in the SLE cohort was 1.3% and incidence rate was 1.5 cases per 1000 patient-years. Overall, 100 cases from literature review and 4 from SLE cohort were identified and are presented as a whole: 49 (47.1%) were classified as neuromyelitis optica spectrum disorders (NMOSD), 29 (27.9%) as CIS, 14 (13.5%) as NMO, 7 (6.7%) as DS prominently involving the brainstem and 5 (4.8%) as DS prominently involving the brain. DS was the SLE onset manifestation in 41 (39.4%) patients. Longitudinally extensive transverse myelitis was the most frequent manifestations being present in 73 (70.2%) patients (37 NMOSD, 21 CIS, 14 NMO, 1 DSB). Methylprednisolone (79.8%) and cyclophosphamide (55.8%) pulses, but also plasma-exchange (16.3%) and rituximab (7.6%) in relapsing-refractory cases, were mostly prescribed. Complete recovery rate ranged between 62% in CIS to 7% in NMO. CONCLUSION: DS in SLE is rare (1%) and encompasses different subtypes including CIS. Timely diagnosis and early treatment are recommended to minimize complications.

Twenty-year brain magnetic resonance imaging follow-up study in Systemic Lupus Erythematosus: Factors associated with accrual of damage and central nervous system involvement.

To evaluate the long-term progression of cerebral MRI abnormalities in patients with longstanding SLE, 30 patients (age 53.5 ± 11.3) underwent brain MRI at baseline (b-MRI) and after 19.4 ± 3.7 years of follow-up (fu-MRI). Two neuroradiologists visually analyzed the MRIs comparing: 1) white matter hyperintensities (WMHIs), 2) cerebral volume, and 3) parenchymal defects; these outcomes were also built in a modified MRI scoring system (mMSS) to estimate the cumulative parenchymal damage. The independent risk factors for accrual of MRI brain damage, as well as the association between MRI abnormalities and the development of new neuropsychiatric (NP) manifestations classified according to the 1999 ACR case definition were also analyzed. Twenty-three patients (76.7%) showed worsening of mMSS; 19 (63.3%) had increased number and volume of WMHIs, 8 (26.7%) had significant cerebral volume loss, and 6 (20%) showed new ischemic parenchymal lesions. Only 6 patients had normal MRI. Antimalarial agents (p=0.006; OR 0.08) were protective against worsening of WMHIs. High cumulative dose of corticosteroids (p=0.026; OR 8.8) and dyslipidemia (p=0.044; OR 10.1) were associated with increased mMSS and cerebral volume loss, respectively. Higher mMSS score at baseline was independently associated with worsening of WMHIs (p=0.001; OR 5.7) and development of new NP events (p=0.019; OR 2.0); higher load of deep WMHIs at b-MRI (p=0.018; OR 2.0) was independently associated with stroke risk. This study shows that MRI brain damage in SLE patients progresses independently from NP involvement as effect of potentially modifiable risk factors and it is associated with increased risk of new NP events.