Beta-propeller protein-associated neurodegeneration presenting Rett-like features: A case report and literature review.

Several patients with beta-propeller protein-associated neurodegeneration (BPAN)/static encephalopathy with neurodegeneration in adulthood have been reported to present Rett syndrome (RTT)-like features. This report presents an individual with BPAN showing clinical features of RTT. Psychomotor delay and epilepsy onset were noted at 1 year, and regression began at 4 years. Screening of the methyl-CpG binding protein 2 (MECP2) did not show variants. At 22 years, basal ganglia iron deposits were found on magnetic resonance imaging (MRI), and the WD-domain repeat 45 gene (WDR45) variant was identified. Review of the literature showed that BPAN with RTT-like features is associated with more epileptic seizures and less deceleration of head growth, breathing irregularities, and cold extremities than classic RTT with MECP2 variants. These clinical presentations may provide clues for differentiating between these two disorders. However, both WDR45 and MECP2 should be screened in patients presenting a clinical picture of RTT without specific MRI findings of BPAN.

Long-Term Epilepsy-Associated Tumors (LEATs): A Single-Center, Retrospective Series and Review of Literature on Factors Affecting the Seizure Outcome.

BACKGROUND: Tumors presenting with drug-resistant seizures are termed as long-term epilepsy-associated tumors (LEATs). LEATs are more common in the temporal lobe, occur predominantly in pediatric age, and focal neurological deficits are rare. In this article, we aim to highlight our surgical experience in terms of seizure outcome among LEATs and discuss the factors affecting outcome. METHODOLOGY: We have retrospectively analyzed all the operated cases of intra-axial brain tumors with seizures (2015-2019). The clinical and radiographic data were collected from the hospital record system. For comparison, 2 groups were made (group 1 with good seizure control, i.e., Engel 1; and group 2 poor seizure outcome, i.e., Engel 2 and 3). RESULTS: A total of 51 cases were included; the temporal lobe was the most common location (n = 27); 23 patients had seizure frequency of "more than 1 seizure per week." Focal unaware seizures/complex partial seizures were the most common type of seizures encountered (n = 28). At a mean follow-up of 39.60 months, 38 patients had Engel 1 (78.5%) outcome (35 cases [71.05%] had the seizure duration of ≤2 years). The median duration of symptoms (group 1, 25 months vs. group 2, 65 months) was significantly different (P = 0.002). On comparing patients with seizure duration, we found a statistically significant difference (P < 0.00001). CONCLUSION: A shorter duration of symptoms, younger age of the patient, partial/focal seizures, and gross total excision were predictors of a good seizure outcome. Histopathology of the tumor does not affect the outcome when one compares glioneuronal tumors with non-glioneuronal tumors.

Ictal Asystole Induced by Right Posterior Quadrant Epilepsy: Report of a Radically Treated Case.

BACKGROUND: Ictal asystole (IA) and ictal bradycardia (IB) are mainly seen with temporal or frontal lobe epilepsy. Many patients with these conditions undergo cardiac pacemaker therapy but not epilepsy surgery. CASE DESCRIPTION: We report the case of a 15-year-old boy with IA and IB secondary to right posterior quadrant epilepsy (PoQE) who underwent right posterior quadrant disconnection, but not cardiac pacemaker implantation. He has remained free from daily epileptic seizures, IA, and IB for more than 6 months postoperatively. This is the first report of a radically treated case with IA and IB caused by PoQE. CONCLUSIONS: Both temporofrontal lobe epilepsy and PoQE caused the IA and IB. Because a cardiac pacemaker only addresses arrhythmia, not epileptic seizures, radical treatment for both epilepsy and arrhythmia may be warranted for patients with medically intractable epilepsy.

Crisis parciales complejas y lesión estructural cerebelosa / Complex partial seizures and cerebellar structural damage

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Complex partial seizure with severe depression and conduct disorder in a 15 year old female adolescent: a case report.

Complex partial seizure complicated by psychiatric comorbidities like depression and conduct disorder presents management challenges for both the physician and parents. The etiology of such psychiatric comorbidities may be related to the seizure or to several other unrelated risk factors. The psychiatric comorbidities and the seizure affects the child's activities of daily living and are often a source of worry to parents and school authorities A high index of suspicion and a multidisciplinary approach are essential in the management of the affected adolescents.

Prosthodontic treatment of an edentulous epileptic patient with an implant-retained overdenture. A case report.

This report describes the treatment of a completely edentulous, mentally retarded patient with epilepsy with an implant-retained overdenture, anchored by a locator system. The intractable epileptic condition of the patient with frequent grand mal seizures and complex partial seizures required a stable, well retained prosthesis, however the maxilla and the mandible were severely atrophied. The bone volume of the maxilla allowed the placement of only two implants it the region of the canines, compared with the generally required four. On long time recall, after one year, patient's chewing ability was satisfactory.

Amygdalohippocampectomy for epilepsy in a patient with prior ipsilateral deep brain stimulator lead placement.

In light of failed medical therapy for movement disorders, the use of deep brain stimulation (DBS) has increased the last two decades. Many complications may transpire; however, to our knowledge, the literature does not mention the phenomena of brain shift from a second unrelated neurosurgical procedure and its theoretical effect on lead displacement and lead function. We present a patient with a left sided DBS for essential tremor and subsequent left amygdalohippocampectomy for temporal lobe epilepsy with minimal radiographic distortion of the DBS lead and without clinical or functional complications. A 47-year-old woman presented with bitemporal epilepsy secondary to a brain injury acquired in childhood in addition to a comorbid bilateral essential tremor, both refractory to medical intervention. A successful left-sided DBS placement was performed with satisfactory resolution of her essential tremor. The patient subsequently developed deterioration of seizure control, becoming refractory to anti-epileptic medications, requiring surgical intervention. A left-sided selective amygdalohippocampectomy and techniques to minimize brain shift were performed without complications. Postoperative imaging suggested minimal distortion of the DBS lead. This did not correspond with reemergence of her essential tremor, implying that the lead maintained functional utility. Brain shift secondary to a craniotomy may cause DBS lead displacement. This phenomenon should be considered when planning operative approaches and can be limited by selective resections. With the growing propensity for placement of DBS leads and the risk of lead displacement, it is important to consider operative approaches to minimize brain shift.

Psychosis induced by zonisamide: a case report and review of the literature.

Zonisamide is an anti-seizure medication that is indicated for adjunctive therapy in the treatment of partial seizures. This medication is rarely used in the United States. An infrequent adverse effect of psychosis occurs in about 2% of patients taking zonisamide. This is a case report of a 34-year-old male on phenytoin who presented with psychosis symptoms approximately 10 months after starting adjunctive zonisamide.

A case of adult onset medulloblastoma during maintenance chemotherapy for anaplastic astrocytoma one year after radiotherapy.

Multiple primitive intracranial tumors with different histological characteristics are uncommon. Although coexistence of a medulloblastoma with glial tumors has been reported in children, medulloblastoma is rarely found in adults, especially those older than 40 years of age. We present an extremely rare case of a medulloblastoma developing in a 40-year-old male undergoing maintenance chemotherapy for anaplastic astrocytoma for 21 months after radiotherapy. Initially, he complained of intractable epilepsy characterized by complex partial seizures. Magnetic resonance imaging (MRI) revealed a slightly enhanced mass lesion in the left insula region. He underwent subtotal removal of the tumor and it was histologically diagnosed as anaplastic astrocytoma. After 19 months of treatment with temozolomide (TMZ) and radiotherapy, he presented with vertigo and headache. A homogeneously enhanced mass had developed in the left cerebellar hemisphere. He received gross total resection of the second tumor, pathologically diagnosed as medulloblastoma. In conclusion, this is the first case report of an adult medulloblastoma coexisting with anaplastic astrocytoma.

Postictal psychosis: implications for nursing.

Postictal psychosis (PIP) is a serious outcome of epilepsy, which may be preventable. Once the symptoms are identified, rapid diagnosis and treatment will prevent further deterioration in the patient's condition. The nurse plays a critical role in this process. The specific presentation of PIP, along with clarification of potential causes and strategies to prevent the occurrence of this complication, will be discussed. Through a case study format and emphasis on the role of the nurse, the specifics of case finding and rapid intervention will be described.

Asystole following complex partial seizures.

A case is presented of a patient with a long history of epilepsy who presents with recurrent seizures and develops a period of asystole. The case highlights the need to consider the potential arrhythmic complications of seizures and the clinical characteristics that may be present in those with epilepsy that may warrant evaluation for arrhythmias.

A retrospective diagnosis of epilepsy in three historical figures: St Paul, Joan of Arc and Socrates.

It has been suggested that undiagnosed epilepsy profoundly influenced the lives of several key figures in history. Historical sources recounting strange voices and visions may in fact have been describing manifestations of epileptic seizures rather than more supernatural phenomena. Well-documented accounts of such experiences exist for three individuals in particular: Socrates, St Paul and Joan of Arc. The great philosopher Socrates described a 'daimonion' that would visit him throughout his life. This daimonion may have represented recurrent simple partial seizures, while the peculiar periods of motionlessness for which Socrates was well known may have been the result of co-existing complex partial seizures. St Paul's religious conversion on the Road to Damascus may have followed a temporal lobe seizure which would account for the lights, voices, blindness and even the religious ecstasy he described. Finally, Joan of Arc gave a detailed narrative on the voices she heard from childhood during her Trial of Condemnation. Her auditory hallucinations appear to follow sudden acoustic stimuli in a way reminiscent of idiopathic partial epilepsy with auditory features. By analysing passages from historical texts, it is possible to argue that Socrates, St Paul and Joan of Arc each had epilepsy.

Neurocognitive profiles in children with epilepsy.

PURPOSE: The presence of specific neurocognitive deficits may help explain why school achievement and psychosocial functioning are often worse in children with epilepsy than would be predicted by their global intellectual functioning. This study compared children with two forms of epilepsy: localization-related epilepsy with complex partial seizures (CPS) and childhood absence epilepsy (CAE), to determine whether they display distinct neurocognitive profiles. METHODS: Fifty-one children with CPS, 31 children with CAE, and 51 controls underwent neuropsychological testing assessing verbal memory, visual memory, and executive functioning. Groups were compared in these cognitive domains. Within-group analyses were also conducted to examine seizure-related factors that may be related to neuropsychological test performance. KEY FINDINGS: When compared to controls, children with CPS showed a mild generalized cognitive deficit, whereas children with CAE did not. When we controlled for intelligent quotient (IQ), both epilepsy groups showed poorer performance relative to controls in the domain of verbal memory. When the epilepsy groups were compared to one another, the CPS group performed significantly poorer than the CAE group on a test of generalized cognitive functioning. However, in the specific domains of executive functioning, verbal memory, and visual memory the epilepsy groups did not differ when compared to one another. SIGNIFICANCE: Neurocognitive deficits present in the context of grossly intact global intellectual functioning highlight the importance of neuropsychological screening in both children with CPS and children with CAE.

Testing for minimal consciousness in complex partial and generalized tonic-clonic seizures.

Impaired consciousness in epilepsy has a major negative impact on quality of life. Prior work suggests that complex partial seizures (CPS) and generalized tonic-clonic seizures (GTCS), which both cause loss of consciousness, affect similar frontoparietal networks. Milder involvement in CPS than in GTCS may spare some simple behavioral responses, resembling the minimally conscious state. However, this difference in responses has not been rigorously tested previously. During video-electroencephalography (EEG) monitoring, we administered a standardized prospective testing battery including responses to questions and commands, as well as tests for reaching/grasping a ball and visual tracking in 27 CPS (in 14 patients) and 7 GTCS (in six patients). Behavioral results were analyzed in the ictal and postictal periods based on video review. During both CPS and GTCS, patients were unable to respond to questions or commands. However, during CPS, patients often retained minimally conscious ball grasping and visual tracking responses. Patients were able to successfully grasp a ball in 60% or to visually track in 58% of CPS, and could carry out both activities in 52% of CPS. In contrast, during GTCS, preserved ball grasp (10%), visual tracking (11%), or both (7%), were all significantly less than in CPS. Postictal ball grasping and visual tracking were also somewhat better following CPS than GTCS. These findings suggest that impaired consciousness in CPS is more similar to minimally conscious state than to coma. Further work may elucidate the specific brain networks underlying relatively spared functions in CPS, ultimately leading to improved treatments aimed at preventing impaired consciousness.

Central apnea at complex partial seizure onset.

Sudden Unexpected Death in Epilepsy (SUDEP) is the most common cause of epilepsy related mortality in treatment resistant epilepsy. Most SUDEPs occur after one or more seizure(s) during sleep. Nocturnal seizures may go unrecognized. Respiratory depression in the peri-ictal period is one of the primary potential causes of SUDEP. Ictal and postictal apnea is often overlooked because it is not routinely assessed, but appears common and has been a recent focus of SUDEP research. We report a 37 year-old man who had central apnea as the initial manifestation of partial complex seizures associated with oxygen desaturation. This important pathophysiological consequence of a nocturnal complex seizure was identified by respiratory monitoring during a combined video EEG and sleep study. Diagnostic and therapeutic implications are discussed.

Levetiracetam-associated loss of libido and anhedonia.

The relationship between the older antiepileptic drugs (AEDs) and sexual dysfunction has long been known and it is likely to be related to sexual hormonal changes. Instead, rare reports on sexual disorders related to new AEDs suggest the possibility of complex and poorly understood mechanisms, mainly involving central nervous system neurotransmitters such as glutamate, serotonin, and dopamine. Herein, we describe two young men with epilepsy who experienced severe loss of libido and anhedonia after levetiracetam intake.