Valmir Passarelli

Multiple endocrine neoplasia type 1 presenting as refractory epilepsy and polyneuropathy — A case report

Hypoglycemia is a well recognized cause of acute symptomatic seizures. The fact that hypoglycemia can cause peripheral neuropathy is less appreciated. We describe a case of insulinoma associated peripheral neuropathy. A 17 year-old previously healthy man was referred for investigation of refractory epilepsy. A history of recurrent seizures, slowly progressive weakness of his feet and hands, and weight gain was obtained. Physical examination showed signs of a chronic sensory-motor polyneuropathy. He was diagnosed with insulinoma and primary hyperparathyroidism, characterizing multiple endocrine neoplasia, type 1 syndrome. Cases of insulinoma associated peripheral neuropathy are very rare. The more characteristic clinical picture appears to be distal weakness, worse in the intrinsic hand and feet muscles, and no or mild sensory signs. Peripheral nervous system symptoms may not completely resolve, despite removal of the cause of hyperinsulinism/hypoglycemia and full reversion of central nervous system symptoms. Mechanisms underlying hypoglycemic neuropathy are still poorly understood.

Gender and age influence in daytime and nighttime seizure occurrence in epilepsy associated with mesial temporal sclerosis

OBJECTIVES The aim of this study was to analyze the daytime and nighttime seizure distribution during video-EEG monitoring in patients with epilepsy associated with unilateral mesial temporal sclerosis (MTS) and the role of gender, age, and lesion side on 24-hour seizure distribution. METHODS We studied 167 consecutive adult (age≥16years) patients with epilepsy associated with unilateral mesial temporal sclerosis that had three or more recorded seizures during continuous video-EEG monitoring with a minimum recording time period of 24h. Seizure onset time was classified according to occurrence in six 4-hour periods. RESULTS Seven hundred thirty-five seizures were evaluated. We observed two higher seizure occurrence periods: 08:01-12:00 (p=0.001) and 16:01-20:00 (p=0.03). Significantly fewer seizures were observed between 0:01 and 4:00 (p=0.01). Nonuniform seizure distribution was noted in women (p<0.0001), in young patients (less than 45years of age) (p<0.0001), and in both patients with left (p=0.03) and patients with right mesial temporal sclerosis (p=0.008). Men presented uniform seizure occurrence distribution (p=0.15). Women had fewer seizures than expected and fewer seizures than men between 0:01-04:00 (p<0.0001 and p=0.0015, respectively) and 04:01-08:00 (p=0.01 and p=0.03, respectively). Young patients (age<45years) had two seizure occurrence peaks, 08:01-12:00 (p=0.016) and 16:01-20:00 (p=0.004). Middle-aged/old patients (≥45years) had only one seizure occurrence peak, 08:01-12:00 (p=0.012). Young patients had more seizures than middle-aged/old patients between 16:01-20:00 (p=0.04). No differences were noted between left and right MTS. SIGNIFICANCE We observed two seizure occurrence peaks: morning and late afternoon/evening. We encountered variations in daytime and nighttime seizure distribution according to gender and age, but not according to side of MTS. Future studies are needed to confirm these findings and to unravel the neurobiological substrate underlying daytime and nighttime variations of seizure occurrence in different age groups and between genders.

Stimulus-induced Rhythmic, Periodic, or Ictal Discharges (SIRPDs) associated with seizures in cefepime neurotoxicity

Departamento de Neurologia, Hospital Santa Marcelina, Sao Paulo SP, Brazil. Correspondence: Valmir Passarelli; Rua Oscar Freire, 1380 / apto 51; 05409-010 Sao Paulo SP, Brasil; E-mail: valmirpas@ig.com.br Received 18 February 2014; Received in final form 02 May 2014; Accepted 22 May 2014. Figure. Initial EEG. Prior to stimulation, slowing of background (A). After sternal rub, bilateral triphasic waves (repeated every 0.5 s) prominent in the fronto-central regions of the two hemispheres, left and median predominated (B). This activity was associated with jerks affecting right upper limb. DOI: 10.1590/0004-282X20140094 IMAGES IN NEUROLOGY

Extratemporal abnormalities in phosphorus magnetic resonance spectroscopy of patients with mesial temporal sclerosis

OBJECTIVE We evaluated extratemporal metabolic changes with phosphorus magnetic resonance spectroscopy (31P-MRS) in patients with unilateral mesial temporal sclerosis (MTS). METHOD 31P-MRS of 33 patients with unilateral MTS was compared with 31 controls. The voxels were selected in the anterior, posterior insula-basal ganglia (AIBG, PIBG) and frontal lobes (FL). Relative values of phosphodiesters- PDE, phosphomonoesters-PME, inorganic phosphate - Pi, phosphocreatine- PCr, total adenosine triphosphate [ATPt = γ- + a- + b-ATP] and the ratios PCr/ATPt, PCr/γ-ATP, PCr/Pi and PME/PDE were obtained. RESULTS We found energetic abnormalities in the MTS patients compared to the controls with Pi reduction bilaterally in the AIBG and ipsilaterally in the PIBG and the contralateral FL; there was also decreased PCr/γ-ATP in the ipsilateral AIBG and PIBG. Increased ATPT in the contralateral AIBG and increased γ-ATP in the ipsilateral PIBG were detected. CONCLUSION Widespread energy dysfunction was detected in patients with unilateral MTS.

The intracarotid etomidate Wada test: A 54-patient series

Hemispheric language dominance and isolated hemispheric memory function evaluation can be undertaken with the intracarotid injection of a general anesthetic agent (Wada test). Amobarbital has been traditionally used as the anesthetic agent, but legal and commercial constraints limit its use. We evaluated the use of etomidate as an alternative agent for the Wada test in a series of 54 consecutive adult patients with mesial temporal sclerosis undergoing presurgical evaluation for epilepsy surgery. Language lateralization and hemispheric memory function evaluation were successfully achieved in all cases. Side effects (somnolence, tremor, and dystonia) were infrequent, minor, and transient and did not require interruption of the procedure. Etomidate appears to be a safe and effective alternative agent to amobarbital in the Wada test. Similarly to the amobarbital Wada test, the ability of the etomidate Wada test to predict postoperative memory decline remains unclear.

Corpus callosum diffusion abnormalities in refractory epilepsy associated with hippocampal sclerosis

OBJECTIVES To detect by diffusion tensor imaging (DTI) the extent of microstructural integrity changes of the corpus callosum (CC) in patients with hippocampal sclerosis (HS) and to evaluate possible association with clinical characteristics. METHODS Fourty-two patients with temporal lobe epilepsy (TLE) and HS and 30 control subjects were studied with DTI. We grouped patients according to lesion side (left or right) HS. Mean diffusivity (MD), fractional anisotropy (FA), radial (RD) and axial diffusivity (AD) were extracted from five segments in CC midsagittal section obtained by automatic segmentation. CC DTI findings were compared between groups. We also evaluated association of DTI changes and clinical characteristics. RESULTS HS patients displayed decreased FA and increased MD and RD in the anterior, mid-posterior and posterior CC segments, compared to controls. No differences were observed in AD. Patients reporting febrile seizure as the initial precipitating event presented more intense diffusion changes. No differences were seen comparing left and right HS. Age at epilepsy onset, disease duration and seizure frequency were not associated with DTI findings. CONCLUSIONS This is one of the largest series of TLE-HS patients evaluating CC white matter fiber integrity by DTI, which allowed us to study how some clinical characteristics, such as seizure frequency, disease duration and lesion side, are related to CC integrity. Occurrence of febrile seizure was the only factor that had significant impact on tract integrity. Diffusion changes were not restricted to the posterior part of the CC; we observed the same changes for the anterior part of the CC. Diffusion changes were characterized by an increase in RD, while the AD remained intact for all regions of the CC.

Folate use in women with epilepsy: Predictors of adherence in a specialized tertiary outclinic

PURPOSE OF THE STUDY The aims of this study were to determine the percentage of women with epilepsy (WWE) taking folic acid (FA) in an outpatient epilepsy tertiary hospital at São Paulo, Brazil, and to identify the possible predictors of adherence. METHOD Five hundred seventy-six medical records of patients followed in our service were reviewed. Sixty-six WWE using antiepileptic drugs with age between 16 and 52, sexually active but not in menopausal period, not pregnant, and not sterilized or with vasectomized partners underwent direct interviews with a prestructured questionnaire. Logistic regression with clinical and demographic variables using a stepwise method was performed to identify predictable variables of folate use. RESULTS We found 42.40% of FA intake. Hormonal contraceptive method use was an independent variable negatively associated with adherence to folate. Additionally, educational level was an independent variable positively associated with adherence to folate in WWE above 35years of age. CONCLUSION We found low folate adherence. Improved planning pre-conception for WWE should improve rates of effective folate supplementation.

Autoimmune limbic encephalitis: A manifestation of systemic lupus erythematosus in the central nervous system

Autoimmune limbic encephalitis (ALE) associated with systemic lupus erythematosus (SLE) is a rare entity with few reports in the literature to date. In general, ALE associated with SLE has a satisfactory response to immunosuppressive treatment (RIT), but the pathogenesis of this association is poorly understood and may include an autoimmunity component. We report a case study describing the diagnosis and management of limbic encephalitis in a patient with active Systemic Lupus Erythematosus disease (SLE) and past medical history of cancer (endometrial adenocarcinoma in 2004 and papillary urothelial carcinoma in 2011 with curative treatment), followed over a one-year period. We discuss the possible association between limbic encephalitis and all past neoplastic and immune-mediated conditions of this patient. In this particularly case, autoimmunity was the most relevant factor associated with limbic encephalitis given negative neoplastic screening. Moreover, a good response was observed to immunotherapy, not seen with paraneoplastic limbic encephalitis, which is associated with poor response. In this case, the association of ALE with SLE is possible, since laboratory testing disclosed lupic activity and the patient had involvement of other systems (such as hematologic) during the period. However, the presence of other surface membrane antibodies are possible in the search for alternative etiologies.

Juvenile myoclonic epilepsy mimicking focal epilepsy

Neurologist at Santa Marcelina Hospital, Sao Paulo SP, Brazil.Correspondence: Valmir Passarelli; Rua Oscar Freire 1.380 / apto. 51; 05409-010 Sao Paulo SP - Brasil; E-mail: valmirpas@ig.com.brConflict of interest: There is no conflict of interest to declare.Received 15 January 2012; Received in final form 27 April 2012; Accepted 07 May 2012

S132. Ipsilateral cerebellar atrophy and crossed ictal cerebellar diaschisis in a case of Rasmussen encephalitis

Introduction Rasmussen encephalitis is a rare hemispheric inflammatory disease with progressive course leading to intractable focal seizures and neurological deficits, which can evolve to epilepsia partialis continua, hemiplegia, aphasia and other symptoms. Neuroimaging classically reveals progressive hemispheric atrophy, based on sequential computed tomography (CT) and magnetic resonance imaging (MRI). Contralateral injury to the cerebellum is expected, represented either by atrophy in anatomical images or by hypometabolism in functional studies as positron emission tomography-computed tomography (PET-CT) or single-photon emission computed tomography (SPECT). The postulated mechanism is interruption to the cerebropontine-cerebellar pathway, with resultant involvement of cerebellum ipsilateral to motor deficits. Methods We describe a case of Rasmussen encephalitis with unexpected cerebellar involvement, evidenced in MRI and ictal SPECT performed during preoperative evaluation. Results A 4-year-old boy had onset of focal clonic motor seizures involving right face and tongue seizures, which evolved to epilepsia partialis continua, progressive right hemiparesis and aphasia. Neuroimaging studies showed progressive left cerebral hemisphere and left cerebellum atrophy. Ictal spect disclosed right cerebellar diaschisis. The patient underwent left functional hemispherectomy. Pathology findings were compatible with Rasmussen’s encephalitis. Clinical follow-up reveals a patient that remains with no more seizures. Conclusion Considering cerebral hemisphere and cerebellum fiber connections, one would expect cerebellar atrophy contralateral to the involved cerebral hemisphere, as previously demonstrated. In our case, functional assessment with ictal SPECT showed contralateral diaschisis, but MRI demonstrated ipsilateral atrophy of cerebellum. These findings did not compromise clinical and surgical prognosis and anatomopathological studies confirmed the diagnosis of Rasmussen encephalitis. The reason for ipsilateral cerebellar involvement in our patient is unclear, but it appears to be more an anatomical variation of neuronal circuits between cerebral cortex and cerebellum than a marker of different clinical course.