Dacrystic seizures reconsidered

Ictal lachrymation has not been reported in children as a main seizure manifestation. We report an infant with tuberous sclerosis who presented with seizures characterised by lachrymation without apparent emotional change associated with a left posterior temporal epileptic focus. We discuss the pathophysiological implications, and propose a clinically orientated classification of crying seizures.     

Tension arachnoid cyst causing uncal herniation in a 60 year old: a rare presentation

Arachnoid cysts are congenital benign cysts accounting for approximately 1% of all intracranial mass lesions. Uncal herniation due to arachnoid cyst is a rare mode of presentation. It is hypothesized that only tension arachnoid cyst could cause the life-threatening condition that results from a progressive deterioration and worsening of a simple and usually congenital arachnoid cyst, associated with the formation of a "ball valve" at the point of an opening on the cyst wall. To-date only one case of an arachnoid cyst causing Uncal herniation has been reported to the best of our knowledge. We present a rare case of uncal herniation in a 60-year-old lady caused by a giant left temporal arachnoid cyst. She presented to us in emergency room after experiencing headaches since last one week followed by vomiting, seizures, and altered state of consciousness. She was operated immediately and marsupialization of the arachnoid cyst was performed. She showed good recovery. Although arachnoid cyst is a benign slowly growing pathology, it can lead to Uncal herniation as a "tension" arachnoid cyst, possible due to "ball-valve" mechanism. Elective treatment should be considered to prevent progressive significant enlargement of cyst.     

Epilepsy surgery for children with tuberous sclerosis complex

Tuberous sclerosis complex is associated with medically refractory seizures and developmental delay in children. These epilepsies are often resistant to antiepileptic drugs, can be quite severe, and usually have a negative impact on the child's neurologic and cognitive development. It is believed that functional outcome is improved if seizures can be controlled at an early age. The surgical treatment of intractable epilepsy in children and adults with tuberous sclerosis complex has gained significant interest in recent years. Previously published studies have shown a potential benefit from resection of single tubers, with most of the results noted in relatively older children. All of these reports support the idea that if a single primary epileptogenic tuber or region can be identified, then a surgical approach is appropriate. However, most children with tuberous sclerosis complex have multiple potentially epileptogenic tubers, rendering localization challenging, and they are therefore rejected as possible surgical candidates. We have used a novel surgical approach using invasive intracranial monitoring, which is typically multistaged and bilateral. This multistage surgical approach has been useful in identifying both primary and secondary epileptogenic zones in patients with tuberous sclerosis complex with multiple tubers. Multiple or bilateral seizure foci are not necessarily a contraindication to surgery in selected patients. Long-term follow-up will determine whether this approach has durable effects. We await better methods for identifying the epileptogenic zone, both noninvasive and invasive.     

Gelastic seizures in tuberous sclerosis complex: case report and literature review

Gelastic seizures are typically associated with hypothalamic hamartoma. Given the rarity of gelastic seizures, pathways for the motor and emotional aspects of laughter have been hypothesized but remain unclear. The authors perform a literature review to discuss what is known about these pathways. They also report a child who presented with tuberous sclerosis complex initially without cutaneous stigmata, who later developed gelastic seizures. Only 2 case reports of patients with tuberous sclerosis complex who subsequently developed gelastic epilepsy have previously been reported. In discussing his case, the authors postulate additional etiologies for gelastic seizures.     

Autism and tuberous sclerosis

The co-occurrence of autism spectrum disorder and tuberous sclerosis complex has been recognized for decades. The prevalence of tuberous sclerosis complex in the autism spectrum disorder population is 1 to 4%, whereas features of autism spectrum disorder are present in 25 to 50% of individuals with tuberous sclerosis complex. The underlying reason for this association might be a nonspecific disruption of brain function owing to tuberous sclerosis complex, including tuber location, seizures and their effect on brain development, cognitive impairment, a disturbance in brain development in regions associated with autism spectrum disorder, or, less likely, a linkage between a TSC gene and an autism susceptibility gene. Awareness of the relationship between autism spectrum disorder and tuberous sclerosis complex is important during the evaluation of individuals with either disorder. Better delineation of the association and its causative factors is needed for the development of possible interventions.     

Familial intracranial arachnoid cysts

Three siblings with intracranial arachnoid cysts are described, two males and one female. One of the males has symmetric, bilateral, temporoparietal convexity cysts, and the others have singular, unilateral cysts. Three additional siblings in the family and other known relatives are clinically unaffected. As far as we know, this is the second reported case of familial intracranial arachnoid cysts and the first involving three siblings. The significance of these cysts and a review of the literature are presented.     

Epilepsy surgery for tuberous sclerosis

Tuberous sclerosis complex is often associated with medically refractory epilepsy secondary to cortical tubers. Previous studies have identified an association between early seizure onset, greater seizure burden, and mental retardation in childhood. Early effective seizure control could therefore significantly reduce the adverse developmental effects of chronic epilepsy in tuberous sclerosis complex. In medically intractable epileptic patients, surgical treatment has been demonstrated to reduce or abolish seizures and the associated burden for the child and its environment. Epilepsy surgery has not been widely used in patients with tuberous sclerosis complex thus far, mostly because of the concern that the multifocal nature of the cortical lesions could be associated with the development of independent epileptogenic zones. Advances in electroencephalographic techniques, functional neuroimaging, and invasive cortical mapping are changing this view and allowing an increased number of tuberous sclerosis complex patients to be evaluated for resective surgery. Additional techniques emerging in the neurosurgical field may add further treatment options to the current state of the art.     

Subdural hygroma in association with middle fossa arachnoid cyst: acetazolamide therapy

Intracranial arachnoid cysts are cerebrospinal fluid-filled collections between arachnoid layers. While many are silent, arachnoid cysts can become symptomatic if there is sudden expansion, haemorrhage or rupture with the development of subdural hygroma or subdural hematoma. Several studies have demonstrated the association of arachnoid cysts with subdural hygroma and subdural hematoma. We describe a 9-year-old girl with a moderate-sized middle-fossa arachnoid cyst and bilateral frontal subdural hygroma presenting with raised intracranial pressure. She was treated with acetazolamide which resulted in resolution of the subdural hygroma and relief of symptomatology.     

A case of neonatal tuberous sclerosis with large calcified lesions in the brain parenchyma]

A patient was reported, who was diagnosed as having tuberous sclerosis in neonatal period. He began to have complex partial seizures at 3 weeks of age. At 27 days of age, brain CT revealed a large high density lesion from the left temporal cortex to the left posterior horn, in addition to periventricular nodules. These lesions were not enhanced after injection of contrast medium. Brain MRI demonstrated more nodular lesions, the intensity pattern of which was different form those in previously reported cases. Ictal EEG showed secondary generalization from left temporal focus, which was compatible with complex partial seizures. In English literature, neonatal cases of tuberous sclerosis with large parenchymal lesions on brain CT were reported by only a few authors, and only two cases of neonatal MRI findings were documented. The neonate with tuberous sclerosis should be carefully evaluated because of the atypical appearance of MRI in rare cases.     

Temporal lobe epilepsy associated with hippocampal sclerosis and a contralateral middle fossa arachnoid cyst.

We report on a 13-year-old boy with temporal lobe epilepsy associated with left hippocampal sclerosis and a contralateral arachnoid cyst in the middle cranial fossa (ACMCF). Chronic intracranial recording from subdural grid electrodes showed the left medial temporal lobe to be the ictal onset zone. After left anterior temporal lobectomy with hippocampectomy, seizure control was improved. ACMCF was not considered the direct cause of epilepsy; instead the seizures were attributed to hippocampal sclerosis.     

Subependymal giant cell astrocytoma associated with tuberous sclerosis presenting with intratumoral bleeding. Case report and review of literature

The authors describe a rare case of subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis complex that presented with intratumoral bleeding with extension to the ventricles. The literature regarding intracranial hemorrhage of SEGA is reviewed and only five cases have been reported in the literature. We have not identified a histological feature associated with bleeding.     

Arachnoid cyst of the middle cranial fossa — aplasia of temporal lobe?

In 11 cases of intracranial, temporal arachnoid cysts the etiology, clinical and radiographic findings, surgical treatment, and outcome are reviewed in respect of post-traumatic subdural hematoma. Cysts of the middle cranial fossa are susceptible to trauma, which may cause bleeding either into the cyst or into the subdural space. Signs and symptoms of increasing intracranial pressure (ICP), local neurological deficits, and sometimes epileptic seizures may lead to hospitalization. CT or MRI scans are diagnostic in these cases. In cases of intracranial mass lesion with displacement of the midline structures and increasing ICP, osteoplastic craniotomy is performed and the lateral wall of the cyst is resected down to the tentorial notch by a microsurgical procedure, with opening into the basal cisterns. There were no operative or postoperative complications in 11 consecutive cases. However, one boy required a cystoperitoneal shunt 3 months later as a result of hydrocephalus following subdural hematoma. Asymptomatic arachnoid cysts are discussed with respect to brain function and social behavior.Paper presented at the 10th Annual Meeting of the European Society for Paediatric Neurosurgery, Aalborg 1986     

Arachnoid cysts in tuberous sclerosis complex

Objective: Some clinical findings in tuberous sclerosis complex (TSC), such as hypomelanotic macules or angiofibromas are related to problems in development of the neural crest, which is also the origin of cranial leptomeninges. Arachnoid cysts have been reported in two TSC patients to date. The purpose of this study was to assess the prevalence and characteristics of arachnoid cysts in a large cohort of TSC. Materials and method: We performed a review of brain MRIs of 220 TSC patients searching for arachnoid cysts. Results: Arachnoid cysts were found in 12 (5.5%) (general population: 0.5%), including ten males (83.3%). Four patients (33.3%) had also autosomal dominant polycystic kidney disease (ADPKD) due to a contiguous deletion of the TSC2–PKD1 genes. Three patients (25%) had two or more arachnoid cysts, of whom two also had ADPKD. One patient with an arachnoid cyst did not have tubers, subependymal nodules or white matter migration lines. Conclusion: Our study suggests that arachnoid cysts are part of the clinical spectrum of TSC and may be also present in TSC patients without other typical TSC brain lesions.     

脑电监测颅内蛛网膜囊肿继发癫(癎)的显微外科治疗

目的:探讨颅内蛛网膜囊肿(IAC)与癫癎的关系以及显微外科治疗方法。方法:应用脑电监测对颅内IAC伴癫癎的26例患者进行显微手术治疗。均行颅内IAC切除,致灶局限于脑非功能区者行致灶切除;致灶局限于脑功能区行灶区皮质热灼术。结果:26例患者中完全不发作者11例,显著改善者10例,无变化者5例。结论:颅内IAC可引起癫癎,显微手术切除IAC的同时,应用脑电监测行致灶切除和(或)皮质热灼术,对IAC伴癫癎患者的治疗较为理想。     

Magnetic resonance imaging of intracranial lesions of tuberous sclerosis

Six patients with tuberous sclerosis were evaluated with computed cranial tomography (CCT) and magnetic resonance imaging (MRI). The results were assessed in comparison with the clinical severity of the disease. The brain lesions were shown by MRI as low-signal areas on IR images (T1 weighted sequences) and high-signal areas on SE images (T2 weighted sequences) Three patients, who had severe psychomotor retardation (DQ less than 70) and intractable epileptic seizures following infantile spasms, had many cortical and subcortical lesions. In the other three patients, intelligence was normal or slightly retarded (DQ or IQ greater than 70) and epileptic seizures were well controlled, and small subependymal lesions were observed. Cortical lesions were rare. These results indicate that MRI can detect more precisely intracranial lesions in tuberous sclerosis.     

Experimental Models of Temporal Lobe Epilepsy: New Insights from the Study of Kindling and Synaptic Reorganization

Summary: Temporal lobe epilepsy is a common localization-related epileptic syndrome characterized by complex partial seizures, ictal and interictal epileptic discharges arising from limbic structures of the temporal lobe, and association with hippocampal sclerosis. Temporal lobe epilepsy may follow perinatal injury and febrile convulsions, may be progressive, and frequently becomes refractory to standard antiepileptic therapy. The neurobiology that underlies these features of temporal lobe epilepsy is not known. Recent studies in experimental models have provided new insights that may help clarify the relationship of seizures, hippocampal sclerosis, and temporal lobe epilepsy. Observations from the study of the hippocampus with kainic acid-induced lesions, the kindling model, and other experimental models of epilepsy have demonstrated that seizures induce structural and electrophysiologic alterations in hippocampal pathways that may lead to increased excitability and could play a role in the development and progression of temporal lobe epilepsy. These alterations include mossy fiber synaptic reorganization, induction of NMDA-mediated synaptic transmission, and progressive hippocampal neuronal loss induced by brief kindled seizures. Some of the structural alterations induced by kindling have also been observed in the human epileptic temporal lobe, raising the possibility that mechanisms operative in kindling may play a role in the pathogenesis of hippocampal sclerosis and in the syndrome of human temporal lobe epilepsy.     

Anévrismes intracrâniens et sclérose tubéreuse de Bourneville : une association rare

Introduction

Tuberous sclerosis is an autosomal dominant inherited phakomatosis. It is associated with a wide variety of central nervous system abnormalities, but intracranial aneurysms are rare.

Case report

We report a 34-year-old patient fulfilling the diagnostic criteria of tuberous sclerosis in association with intracranial aneurysm.

Discussion

This association has been reported in only 17 other cases of tuberous sclerosis. We discuss the etiopathogenic mechanisms, preferential localizations and the various therapeutic propositions.     

Outcome after temporal lobectomy in bilateral temporal lobe epilepsy

We determined how noninvasive presurgical data relate to prognosis after temporal lobectomy in patients with independent bilateral temporal lobe (IBTL) complex partial seizures on the intracranial electroencephalogram (EEG). Between 1986 and 1994, 28 patients had IBTL seizures on intracranial EEG. Fifteen of these 28 patients underwent temporal lobectomy and 13 were not offered surgery. Of the 15 patients who had surgery, 10 patients became seizure-free. Magnetic resonance imaging (MRI) and the Wada test were the only variables associated with a seizure-free outcome. Seven of 10 seizure-free patients had a lateralized Wada result or the presence of unilateral hippocampal sclerosis, whereas none of the patients with persistent seizures had either of these findings. Variables not found to be predictive of a seizure-free outcome included location of scalp interictal spikes, degree of seizure-onset laterality, presence of early epilepsy risk factor, duration of epilepsy, and full-scale intelligence quotient. We conclude that MRI and the Wada test provide information of prognostic value in patients with bilateral temporal seizures independent of intracranial EEG data.     

Cervicothoracic extradural arachnoid cyst: possible association with obstetric brachial plexus palsy

The association of cervicothoracic extradural arachnoid cysts and obstetric brachial plexus palsy has not previously been reported. We report two patients with this association. The first patient is a 9-month-old boy with left obstetric brachial plexus palsy that developed bilateral leg weakness at 6 months of age owing to compression of the spinal cord by a C6 to T8 left cervicothoracic extradural arachnoid cyst. The second patient is a 3-year-old girl with bilateral brachial plexus palsy and spastic paraparesis who had magnetic resonance imaging at 3 days of age that showed intraspinal cord injury and a cervicothoracic extradural arachnoid cyst compressing the spinal cord. We believe that the association of cervicothoracic epidural arachnoid cysts and obstetric brachial plexus palsy in these patients was causal and recommend that the possibility of a cervicothoracic epidural arachnoid cyst be considered in patients with brachial plexus palsy and evidence of spinal cord injury.     

Klüver–Bucy syndrome after unilateral frontotemporal resection in a child with tuberous sclerosis

Klüver–Bucy syndrome (KBS) is a behavioral phenotype that appears most often after bilateral temporal damage. The main features of KBS are compulsion to examine objects orally, increased sexual activity, placidity, hypermetamorphosis (irresistible impulse to notice and react to everything within sight), visual agnosia, and problems with memory. It is more rarely reported in children than in adults. We present a case of KBS in a 2-year-old boy with tuberous sclerosis complex (TSC) after left frontotemporal resection for refractory epilepsy. This is the first KBS after unilateral temporal resection in a child, although it has already been reported in two adult cases. It also is the first case reported in a TSC patient.