William Alves Martins

Variables associated with co-existing epileptic and psychogenic nonepileptic seizures: a systematic review

PURPOSE Epileptic seizures (ES) have many mimickers, perhaps the most relevant being psychogenic nonepileptic seizures (PNES). The picture is even more challenging when PNES are associated with ES in a given patient. The aim of this research paper was to delineate the demographic, epileptological and psychiatric profile of that specific population. METHODS A systematic review was carried out from 2000 to 2015 for articles in English, French, Italian, Spanish and Portuguese in PUBMED and EMBASE. Cohort or case-control studies reporting prospective or retrospective original data comparing patients with co-existing ES and PNES with those who had PNES only and ES only have been included. In retained studies, the presence of PNES was confirmed by video-electroencephalography (V-EEG). Forty-eight abstracts were identified. RESULTS Nine studies were retained. Most showed that female gender predominated in both groups with PNES. Patients with co-existing ES and PNES take higher number of antiepileptic drugs (AEDs) than PNES alone. Two studies showed association of concomitant ES and PNES with earlier age of seizure onset. Localizing EEG features and ES type were evaluated in only two studies and their association with either group was inconclusive. Somatoform, conversion or cluster B personality disorders were more frequent in subjects with PNES than with ES. DISCUSSION Patients with concomitant ES and PNES are highly heterogeneous, challenging differentiation on clinical grounds. A diagnosis of conversion or somatoform, anxiety disorders, and the use of a higher number of AEDs than psychiatric medications may have an association with co-existing ES and PNES. Further studies are warranted to differentiate patients who only have PNES from those with co-existing ES and PNES.

Selective posterior callosotomy for drop attacks: A new approach sparing prefrontal connectivity

Objective: To evaluate a novel approach to control epileptic drop attacks through a selective posterior callosotomy, sparing all prefrontal interconnectivity. Methods: Thirty-six patients with refractory drop attacks had selective posterior callosotomy and prospective follow-up for >4 years. Falls, episodes of aggressive behavior, and IQ were quantified. Autonomy in activities of daily living, axial tonus, and speech generated a functional score ranging from 0 to 13. Subjective effect on patient well-being and caregiver burden was also assessed. Results: Median monthly frequency of drop attacks decreased from 150 to 0.5. Thirty patients (83%) achieved either complete or >90% control of the falls. Need for constant supervision decreased from 90% to 36% of patients. All had estimated IQ below 85. Median functional score increased from 7 to 10 (p = 0.03). No patient had decrease in speech fluency or hemiparesis. Caregivers rated the effect of the procedure as excellent in 40% and as having greatly improved functioning in another 50%. Clinical, EEG, imaging, and cognitive variables did not correlate with outcome. Conclusions: This cohort study with objective outcome assessment suggests that selective posterior callosotomy is safe and effective to control drop attacks, with functional and behavioral gains in patients with intellectual disability. Results are comparable to historical series of total callosotomy and suggest that anterior callosal fibers may be spared. Classification of evidence: This study provides Class III evidence that selective posterior callosotomy reduces falls in patients with epileptic drop attacks.

Posterior Reversible Encephalopathy Syndrome: Clinical Differences in Patients with Exclusive Involvement of Posterior Circulation Compared to Anterior or Global Involvement

INTRODUCTION Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiologic syndrome not yet fully understood and characterized by transient neurologic symptoms in addition to typical radiological findings. There are only a few articles that describe the clinical differences between patients with PRES that involve carotid and vertebrobasilar circulations. Our study aims to further evaluate the differences between predominantly anterior and posterior circulation PRES. METHODS We review 54 patients who had received the diagnosis of PRES from 2009 to 2015. The patients were divided into 2 groups: (1) exclusively in posterior zones; and (2) anterior plus posterior zones or exclusively anterior zones. Several clinical characteristics were evaluated, including the following: age, sex, previous diseases, the neurologic manifestations, the highest blood pressure in the first 48 hours of presentation, highest creatinine level during symptoms, and the neuroimaging alterations in brain magnetic resonance imaging. RESULTS Mean age at diagnosis was 28.5 years old (9 men and 45 women) and mean systolic blood pressure among patients with lesions only in posterior zones was 162.1 mmHg compared to 179.2 mmHg in the anterior circulation. The most common symptoms in the 2 groups were headache and visual disturbances. DISCUSSION PRES may have several radiological features. A higher blood pressure seems to be 1 of the factors responsible for developing widespread PRES, with involvement of carotid vascular territory. This clinical-radiological difference probably occurs because of the larger number of autonomic receptors in the carotid artery in comparison to the vertebral-basilar system.

Endometriosis of the sciatic nerve

, manifesting as cyclic sciatica, parestesis and pa-resis with loss of deep tendon reflex. We cite a case of sci-atica endometriosis and make a review of the literature.CASEA 26-year-old Caucasian woman exhibits intense pain and weakness in the lower left limb, which had pro-gressed over the past nine years. She had no previous pregnancy and symptoms were worse during the men-strual period. During examination, a dorsiflexion paresis of the left foot was notable. Magnetic resonance imagining (MRI) revealed an expansive process in the left sciatic nerve to-pography at gluteal topography.The patient was referred for microsurgery, pro-ceeding exeresis of a cystic lesion in the left sciatic nerve. Neuropathology analysis showed an endometrioma (Figure). The patient had no complications during the post-operative period.During initial follow-up, dorsiflexion paresis in the left foot persisted. Post-operative MRI showed a cica-tricial process, with no residual tumor. The eletroneu-romiography (ENMG) displayed signs of a subacute de-nervatory process.DISCUSSIONEndometriosis is a chronic recurrent pathology, char-acterized by proliferation of endometrial tissue outside the uterine cavity

PRES with asymptomatic spinal cord involvement. Is this scenario more common than we know

Introduction:Posterior reversible encephalopathy syndrome (PRES) is an entity characterized by neurologic symptoms such as headaches, altered mental status, seizures and visual changes, and it is associated with white matter vasogenic edema predominantly affecting the posterior occipital and parietal lobes of the brain.Case report:A 19-year-old patient developed PRES after the use of chemotherapy for a testicular teratocarcinoma and after the development of a blood pressure elevation.Discussion:Few cases described the involvement of the spinal cord in this syndrome. In the majority of these cases, the spinal cord involvement was asymptomatic or with few symptoms of spinal cord disease.

Malignant Posterior Reversible Encephalopathy Syndrome: A Case of Posterior Irreversible Encephalopathy Syndrome

Dear Editor, Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic syndrome characterized by predominant parietal and occipital lobe edema that is mainly reversible within a few days.1,2 However, many atypical patterns have been identified.3 Reversibility is not always achieved,3 which exposes a contradiction in this supposedly benign entity. We report the case of a 14-year-old patient who presented with classical signs of PRES, but whose condition evolved into a malignant phenotype. A 14-year-old patient received a renal transplant from a cadaveric donor and took tacrolimus, corticosteroids, and antithymocyte immunoglobulin. On the third day, his renal function had not improved and hemodialysis was started. He then immediately developed headache, vomiting, and experienced a tonic-clonic seizure. His blood pressure (BP) peaked at 240/150 mm Hg. Brain CT revealed bilateral edema in the parieto-occipital regions associated with a small hemorrhagic transformation, representing PRES (Fig. 1A, B, and C). Laboratory tests revealed thrombocytopenia (66,000/µL) and elevated serum creatinine (2.52 mg/dL). Tacrolimus was suspended and sodium nitroprusside was administered, which fully controlled his hypertension. Fig. 1 Brain CT in the first presentation. A: Subcortical edema extends to the parietal and frontal region. B and C: Bilateral occipital edema is demonstrated, with minor hemorrhagic transformation, suggesting posterior reversible encephalopathy syndrome. D, ... On the following day his BP was 147/90 mm Hg but his neurological function had deteriorated. Follow-up brain CT revealed extensive cortical and white-matter edema associated with parenchymatous, subarachnoid, and intraventricular hemorrhage (Fig. 1D, E, and F). Brain death was later confirmed, leading to the suspension of life support. PRES pathophysiology remains a mystery even after almost 20 years since its initial description.1 There are two prevailing hypotheses: cytotoxic and vasogenic.4,5 Eclampsia, renal failure, autoimmune diseases, and chemotherapy treatment and other factors may trigger PRES.1,5 Despite its classically favorable outcome, PRES is associated with direct mortality in 5-15% of cases.6,7 Important prognostic factors within PRES have been suggested, such as the anatomical distribution of edema, hemorrhage, and cytotoxic edema in diffusion-weighted imaging sequences, but they have yet to be proven.7,8 Alhilali et al.7 further analyzed the prognostic factors in PRES and found that hemorrhage, even if minor, is the most relevant factor associated with a poor outcome, which may indicate more extensive endothelial dysfunction. Hefzy et al.6 found that hemorrhagic transformation occurred in 17% of PRES cases, mainly small or petechial hemorrhages (<5 mm) and hematomas, but also subarachnoid and intraventricular bleeding. The rates found in other studies have varied from 6.4% to 19.4%, with mortality reaching 26-29% in this group.6,7 The precipitating illness is usually severe, and PRES may be only a marker of such severity, but novel reports unveil the malignant evolution of this condition.7,8 Although many terms have been used to describe this event,7 malignant PRES seems to best encompass the permanent neurological damage or even death that sometimes accompanies this endothelial breakdown in the brain. The optimal management of PRES has yet to be elucidated.8 Better outcomes in malignant PRES have been attributed to aggressive neurointensive care, decompressive craniectomy, and intracranial pressure management.8 These results are promising, but larger studies are needed to confirm the efficacy of this approach. PRES is usually a benign entity; however, it represents a small outlier in the clinical spectrum of this syndrome. A wider range of neurological disabilities encompasses this syndrome and leads to a malignant evolution, suggesting that a good prognosis cannot be assumed with PRES, but rather its occurrence should prompt immediate action to avoid disability.

Suicide risk, temperament traits, and sleep quality in patients with refractory epilepsy

OBJECTIVES The objective of the study was to access the suicide risk (SR) in patients with refractory epilepsy and its association with temperament and sleep quality. METHODS A total of 50 consecutive patients referred for epilepsy surgery evaluation in the Porto Alegre Epilepsy Surgery Program were included. All patients had a detailed neurologic and psychiatric evaluation, including video-electroencephalogram (VEEG), high-resolution magnetic ressonance imaging (MRI), and neuropsychologic assessment. In addition, structured questionnaires were applied: module C of the MINI-plus (International Neuropsychiatric Interview-Brazilian version 5.0.0), Affective and Emotional Composite Temperament Scale (AFECTS), and Pittsburgh Sleep Quality Index (PSQI). RESULTS Ten patients (20.0%) showed an increased SR. The most frequent location of the epileptic focus was in the temporal lobe (50%; n=25). Final diagnosis on VEEG comprised epilepsy in 74.0% (n=37), psychogenic nonepileptic seizures (PNES) in 8.0% (n=4), and both in 12%. Thirty patients (60.0%) received surgery indication. Mood disorders were the main psychiatric diagnosis, found in 19 subjects (70.4%), with major depressive disorder (MDD) encountered in 15 patients (55.6%). In the group, SR was more frequent in patients with sleep disorders (p=0.001) and elevated scores of high emotional sensitivity (p=0.003). CONCLUSION In this cohort of patients with highly refractory epilepsy, there was a significant association between SR, sleep disorders, and high emotional sensitivity. Careful evaluation of these factors should be performed in these patients to fully access SR.

Periodic lateralized epileptiform discharges (PLEDs) in herpetic encephalitis

An 86-year-old male presented with confusion, fever and tonic-clonic seizures. CSF analysis showed lymphocytic pleocytosis and high protein count, leading to initiation of acyclovir. EEG revealed PLEDs in the left hemisphere (figure 1C) and MRI demonstrat-ed left hippocampal and insular hyperintensities (figure 1A, 1B). Herpes simplex virus (HSV) encephalitis was confirmed by PCR.PLEDs represent periodic sharp discharges lateralized to one hemisphere usually due to acute neuronal injury

“Mirror EPC” Epilepsia partialis continua shifting sides after rolandic resection in dysplasia

Background: Epilepsia partialis continua (EPC) is a life-threatening condition often caused by focal cortical dysplasia (FCD). Resection of the motor cortex is contemplated in the hope that the trade-off between a severe motor deficit and complete seizure control justifies the procedure. Methods: Report of 3 patients with EPC due to histologically confirmed FCD, who underwent resection of the motor cortex under acute electrocorticography. Results: All had re-emergence of medically intractable EPC in the other side of the body after rolandic resection. Two patients died and the third continues with refractory attacks. Conclusion: In some instances, EPC due to FCD may shift sides and re-emerge in the contralateral, previously asymptomatic, hemibody. A mechanism of disinhibition by surgery of a suppressed contralateral and homologous epileptogenic zone is speculated.

Neuropsychiatric features of the coexistence of epilepsy and psychogenic nonepileptic seizures

OBJECTIVE To investigate demographic, epidemiologic and psychiatric features suggestive of the coexistence epilepsy (ES) and psychogenic nonepileptic seizures (PNES) that may contribute to precocious suspicion of the association. METHODS In this exploratory study, all patients older than 16 years admitted to prolonged video-electroencephalogram monitoring were evaluated about demographic, epileptological and psychiatric features. Detailed psychiatric assessment using M.I.N.I.-plus 5.0, Beck Anxiety Inventory, Beck Depression Inventory and the Childhood Trauma Questionnaire (CTQ) was performed. Data were collected previous to the final diagnosis and patients with ES-only, PNES-only or coexistence of ES/PNES were compared. RESULTS Of 122 patients admitted to epilepsy monitoring unit, 86 patients were included and 25 (29%) had PNES. Twelve (14%) had PNES-only, 13 (15%) had ES/PNES and the remaining 61 (71%) had only ES. A coexistence of ES and PNES was associated with clinical report of more than one seizure type (p˂0.001), nonspecific white matter hyperintensities on MRI (p < .001) and a past of psychotic disorder (p = .005). In addition, these patients had significantly more emotional abuse and neglect (p < .002 and 0.001, respectively). Somatization (including conversion disorder) was the most common diagnosis in patients with PNES- only (83%) and co-existing of PNES and ES (69.2%), differentiating both from ES-only patients (p < .001). CONCLUSION The high prevalence of this coexistence ES/PNES in this study reinforces a need to properly investigate PNES, especially in patients with confirmed ES who become refractory to medical treatment with antiepileptic drugs. The neuropsychiatric assessment may help to diagnostic suspicion and in the planning of therapeutic interventions.