Hippocampal dysgenesis associated with temporal lobe hypoplasia and arachnoid cyst of the middle cranial fossa

We describe two children with left hippocampal dysgenesis in association with temporal lobe hypoplasia and arachnoid cyst of the middle cranial fossa. The hippocampus showed an abnormal globular shape and blurred internal structure in both patients. One of the patients had juvenile myoclonic epilepsy without evidence of seizure onset in the abnormal temporal region. The other patient did not have epilepsy; however, he showed developmental language disorder with a probable relationship to the left temporal abnormalities.     

Temporal lobe epilepsy associated with cystic lesion in the temporal lobe and middle cranial fossa]

Four patients out of 52 patients with temporal lobe epilepsy (TLE), who underwent epilepsy surgery in our hospital since September of 1994, had cystic lesions in the temporal lobe and middle cranial fossa. Case 1 had old hematoma cavity in the inferior temporal gyrus and chronic subdural electrode recording revealed the ictal onset zone to be localized in the ipsilateral medial temporal region. Case 2 had cystic ganglioglioma in the temporal tip, and intraoperative electrocorticography demonstrated independent paroxysmal activities from medial temporal region and temporal tip near the cyst. Both area were resected and the patients became seizure free. Case 3 and 4 had arachnoid cysts in the middle cranial fossa. Chronic subdural electrode recording revealed that the ictal onset zone was localized in the ipsilateral inferior temporal gyrus (that had microdysgenesis) in Case 3 and contralateral medial temporal region (that had hippocampal sclerosis) in Case 4, respectively. These finding suggest that co-existence of extra-axial cyst such as Case 3 and 4 is incidental and that arachnoid cyst is less epileptogenic. However, intra-axial cyst such as Case 1 and 2 is epileptogenic and complicated physiological mechanism such as kindling phenomenon or secondary epileptogenesis may effect on the hippocampus. Comprehensive presurgical evaluation including electrocorticography is needed in the surgical treatment of TLE with cystic lesion.     

Extradural hematoma complicating middle fossa arachnoid cyst

Two cases of post-traumatic extradural hematoma complicating an arachnoid cyst of the middle cranial fossa in children are described. While subdural and intracystic hemorrhages are well-known complications from this malformation, the association with extradural hematoma has never been previously reported in the literature. The pathogenetic mechanisms are discussed and the particular vulnerability of intracranial arachnoid cysts is stressed.     

Temporal lobe epilepsy due to hippocampal sclerosis in pediatric candidates for epilepsy surgery.

OBJECTIVE: To characterize the clinical, EEG, MRI, and histopathologic features and explore seizure outcome in pediatric candidates for epilepsy surgery who have temporal lobe epilepsy (TLE) caused by hippocampal sclerosis (HS). METHODS: The authors studied 17 children (4 to 12 years of age) and 17 adolescents (13 to 20 years of age) who had anteromesial temporal resection between 1990 and 1998. RESULTS: All patients had seizures characterized by decreased awareness and responsiveness. Automatisms were typically mild to moderate in children and moderate to marked in adolescents. Among adolescents, interictal spikes were almost exclusively unilateral anterior temporal, as opposed to children in whom anterior temporal spikes were associated with mid/posterior temporal, bilateral temporal, extratemporal, or generalized spikes in 60% of cases. MRI showed hippocampal sclerosis on the side of EEG seizure onset in all patients. Fifty-four percent of children and 56% of adolescents had significant asymmetry of total hippocampal volumes, whereas the remaining patients had only focal atrophy of the hippocampal head or body. Subtle MRI abnormalities of ipsilateral temporal neocortex were seen in all children and 60% of adolescents studied with FLAIR images. On histopathology, there was an unexpectedly high frequency of dual pathology with mild to moderate cortical dysplasia as well as HS, seen in 79% of children and adolescents. Seventy-eight percent of patients were free of seizures at follow-up (mean, 2.6 years). A tendency for lower seizure-free outcome was observed in patients with bilateral temporal interictal sharp waves or bilateral HS on MRI. The presence of dual pathology did not portend poor postsurgical outcome. CONCLUSIONS: TLE caused by HS similar to those in adults were seen in children as young as 4 years of age. Focal hippocampal atrophy seen on MRI often was not reflected in total hippocampal volumetry. Children may have an especially high frequency of dual pathology, with mild to moderate cortical dysplasia as well as HS, and MRI usually, but not always, predicts this finding. Postsurgical seizure outcome is similar to that in adult series.     

Corpora amylacea in temporal lobe epilepsy associated with hippocampal sclerosis

Hippocampal sclerosis (HS) is the commonest pathology in epileptic patients undergoing temporal lobe epilepsy surgery. Beside, there are an increased density of corpora amylacea (CA) founded in 6 to 63% of those cases. OBJECTIVE: verify the presence of CA and the clinical correlates of their occurrence in a consecutive series of patients undergoing temporal surgery with diagnosis of HS. METHOD: We reviewed 72 hippocampus specimens from January 1997 to July 2000. Student's t test for independent, samples, ANOVA and Tukey test were performed for statistical analysis. RESULTS: CA were found in 35 patients (49%), whose mean epilepsy duration (28.7 years) was significantly longer than that group of patients without CA (19.5 years, p=0.001). Besides, when CA were found, duration was also significantly correlated with distribution within hippocampus: 28.7 years with diffuse distribution of CA, 15.4 with exclusively subpial and 17.4 years with distribution subpial plus perivascular (p=0.001). CONCLUSION: Our findings corroborate the presence of CA in patients with HS and suggest that a longer duration of epilepsy correlate with a more distribution of CA in hippocampus.     

Temporal lobe developmental malformations and hippocampal sclerosis: epilepsy surgical outcome

BACKGROUND: Temporal lobe developmental malformations coexist with mesial temporal sclerosis in the form of dual pathology with a high frequency of bilateral amygdala or hippocampal abnormalities. OBJECTIVE: The aim of this study was to correlate and compare the MRI findings and the surgical outcome in patients with temporal lobe developmental malformations (n = 20) and isolated mesial temporal sclerosis (n = 36). METHODS: MRI-based normalized volumetry of the amygdala and hippocampal formation in patients with unilateral temporal lobe developmental malformations and isolated mesial temporal sclerosis who underwent temporal lobe resections was performed. Seizure outcome was compared between groups at follow-up. RESULTS: The frequency of bilateral hippocampal or amygdala atrophy (p < 0.04) and combined hippocampal-amygdala atrophy (p < 0.02) was higher in patients with temporal lobe developmental malformations. Although no significant difference in postsurgical seizure-free status was found between the temporal lobe developmental malformations and isolated mesial temporal sclerosis groups (70% versus 91%), patients with temporal lobe developmental malformations and bilateral amygdala or hippocampal-amygdala atrophy had a significantly worse outcome (p < 0.02). CONCLUSION: Bilateral hippocampal atrophy is frequent in patients with temporal lobe developmental malformations. However, it is the presence of bilateral amygdala or amygdalo-hippocampal atrophy that is associated with a higher risk of seizure recurrence.     

Semantic memory impairment in temporal lobe epilepsy associated with hippocampal sclerosis

Episodic memory impairment is a well-recognized feature of mesial temporal lobe epilepsy. Semantic memory has received much less attention in this patient population. In this study, semantic memory aspects (word-picture matching, word definition, confrontation and responsive naming, and word list generation) in 19 patients with left and right temporal lobe epilepsy secondary to mesial temporal sclerosis (MTS) were compared with those of normal controls. Patients with LMTS showed impaired performance in word definition (compared to controls and RMTS) and in responsive naming (compared to controls). RMTS and LMTS patients performed worse than controls in word-picture matching. Both patients with left and right mesial temporal lobe epilepsy performed worse than controls in word list generation and in confrontation naming tests. Attentional-executive dysfunction may have contributed to these deficits. We conclude that patients with left and right MTS display impaired aspects of semantic knowledge. A better understanding of semantic processing difficulties in these patients will provide better insight into the difficulties with activities of daily living in this patient population.     

Effective shunt-independent treatment for primary middle fossa arachnoid cyst

The results of a prospective study on excision of the outer and inner membrane for symptomatic primary middle fossa arachnoid cysts in children are presented. During the period 1982–1989, among 48 cases of cyst in various locations, 18 symptomatic patients were treated by excision of both the outer and inner membranes with an opening to the basal cistern. The mean patient age was 3.2 years. All patients were evaluated by examinations, including magnetic resonance imaging (MRI), X-ray computed tomography (CT), quantitative CT cisternography (CTC), digital substraction angiography (DSA), N-isopropyl-p[123]iodoamphetamine single-photon emission CT (IMP-SPECT) and IQ. All showed abnormal cerebrospinal fluid flow dynamics. Significant complications included massive subdural effusion in two patients and transient pulmonary edema in one. There were no recurrences during the follow-up period (mean 4.7 years). Two morphological types were noted: type I, the classical anteromedial type where the cysts are attached directly to the adjacent parasellar cisterns, and type II, the anterolateral type, where the diagonally concave anterior temporal lobe covers the adjacent cisterns, making wide opening difficult. Type I accounted for 78% of all cases, and the reduction of the cyst volume with clinical improvement was remarkable within 6 months after surgery. Angiographically, 22% of cases showed tapering and retrograde filling of the superficial middle cerebral vein. This pattern is not included in Hacker's normal variations [18] and suggest mild but chronic compression of the developing brain. Even in patients with hemispheric cyst, the reconstituted brain showed sufficient cerebral perfusion on SPECT, suggesting that the nature of this disease entity is reversible developmental arrest. These results confirm that excision is a safe, effective shunt-independent procedure for middle-fossa arachnoid cysts, especially for those of type I.     

Mesial temporal lobe epilepsy with hippocampal sclerosis is infrequently associated with neuronal autoantibodies

Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE‐HS) is characterized by its well‐defined clinical profile. Limbic encephalitis is increasingly recognized as a possible etiology of adult‐onset MTLE‐HS, and neuronal autoantibodies have been detected in patients even without previous signs of encephalitis. The aim of this study is to analyze the frequency of specific autoantibodies in patients with MTLE‐HS. A case‐control study was carried out with 100 patients with MTLE‐HS and 50 healthy controls. Sera samples from subjects were tested by indirect immunofluorescence assay for detection of anti‐N‐methyl‐d ‐aspartate receptor (NMDA‐R), anti‐contactin‐associated protein‐like 2 (CASPR2), anti‐leucine‐rich glioma inactivated 1 (LGI1), anti‐gamma aminobutyric acid B receptor (GABA‐B‐R), anti‐alpha‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid 1 and 2 receptors (AMPA‐1‐R and AMPA‐2‐R), and enzyme‐linked immunosorbent assay for detection of anti‐glutamic acid decarboxylase 65 (GAD65). Mean age of patients and controls was 41.2 vs 42 years, and 55% vs 56% were female. Mean duration of epilepsy was 27.2 years. No neuronal autoantibodies were found in either group, except for anti‐GAD65 in 3 patients and 2 controls. This study adds to the mounting evidence that, in Brazilian patients, MTLE‐HS without signs and symptoms of autoimmune encephalitis may be infrequently associated with these autoantibodies. Differences regarding accuracy of used methodologies for autoantibody detection and genetic and environmental characteristics are discussed. Further works with different methodologies tested simultaneously in different populations may help clarify the incongruent study results about autoantibodies in MTLE‐HS.     

颞叶蛛网膜囊肿继发癫痫的外科治疗

目的：探讨颞叶蛛网膜囊肿继发癫痫的临床特点及手术治疗效果。方法所有患者均以癫痫发作起病,其中蛛网膜囊肿在左颞有12例,右颞有13例。应用皮层脑电监测对这些患者行手术治疗,术后随访1年以上观察手术的疗效。结果所有患者在切除蛛网膜囊肿后皮层脑电图监测均见颞叶皮层有异常放电,其中15例患者加行了前颞叶切除术（包括大部分海马及杏仁核）,另外10例加行皮层热灼术。术后随访发现囊肿消失18例,囊肿明显减小7例。癫痫发作随访发现Engel Ⅰ级13例,Ⅱ级9例,Ⅲ级2例,Ⅳ级1例,手术总有效率88％。结论对于颞叶蛛网膜囊肿继发癫痫的患者,在手术切除蛛网膜囊肿的同时,应在皮层脑电图监测将癫痫灶一并切除,且手术治疗的效果较理想。     

Development of a middle fossa arachnoid cyst A theory on its pathogenesis

The progression of congenital arachnoid cysts has seldom been documented. We report the case of a child who was diagnosed with arrested hydrocephalus at the age of 13 months. Neuroimaging studies performed when the girl was 22 months old showed the appearance of an arachnoid cyst in the right middle fossa, while the previously enlarged ventricles seemed to have decreased in size. To the best of our knowledge, the paradoxical expansion of an arachnoid pouch following a reduction in the size of the ventricular system has not previously been documented. We advance the hypothesis that the development of some cases of arachnoid cyst might be pathogenically related to impaired CSF dynamics associated with pre-existing hydrocephalus. We also briefly review the pertinent literature on the formation and evolution of congenital cerebral arachnoid cysts. Received: 4 September 1998     

Spontaneous disappearance of middle fossa arachnoid cyst after head injury

A case of middle fossa arachnoid cyst is presented, which disappeared after head injury. Five days after the trauma, CT scan revealed subdural fluid collection in addition to an arachnoid cyst in the middle fossa on the same side. As the subdural fluid resorbed, the cyst became smaller and disappeared on the follow-up CT scans without surgical intervention.     

Chronic subdural hematoma associated with the middle fossa arachnoid cyst: pathogenesis and review of its management

Background

The pathogenesis of chronic subdural hematoma (CSDH) associated with an arachnoid cyst (AC) is still not clear. We propose an origin of initial bleeding of CSDH in patients with AC based on our experience and discuss the management of this disease.

Materials and methods

The total number of operations included in this study was 23. Eleven cases were indicated because of associated CSDH (group 1), and the other cases were due to different reasons (group 2). The relationship of the AC and dura was evaluated in patients who did not have CSDH (group 2) because patients with CSDH would have had structural changes of AC due to hematoma.

Results

In group 1, the age distribution was from 8 to 61 years (mean 23.0). The development of CSDH was not related with Galassi types. The hematomas were located outside of the outer cyst membrane in six patients and both inside and outside in three patients. In two patients, the location could not be reviewed. After suspicion of the outer cyst membrane as the origin of the bleeding site, we found small bridging vessels between the dura and outer cyst membrane in three of five consecutive patients in group 2.

Conclusions

Based on our surgical experience of AC cyst, we found small bridging vessels between the dura and outer membrane of the AC. We suggested that these small vessels are the source of initial bleeding leading to CSDH in AC.     

Intraoperative subdural hematoma in a patient with arachnoid cyst in the middle cranial fossa

A case of arachnoid cyst in the middle cranial fossa complicated by intraoperative subdural hemorrhage is presented. Accumulation of cerebrospinal fluid (CSF) outside the outer wall of the arachnoid cyst was found to induce the rupture of well-developed, fragile leptomeningeal vessels at the base of the middle cranial fossa. This phenomenon is noteworthy as a pitfall in surgery for intracranial arachnoid cyst and suggest the bleeding source of subdural hematoma occasionally associated with this lesion.     

ILAE Commission Report. Mesial temporal lobe epilepsy with hippocampal sclerosis

Experts discussed the definition, natural history, pathologic features, pathogenesis, electroclinical, neurophysiological, neuropsychological, structural and functional imaging findings, as well as surgical outcome in mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). After a long-lasting consensus process the ILAE Commission Neurosurgery of epilepsy accepted the resulting conclusions as state-of-the art report on MTLE-HS. The majority of contributors considered MTLE-HS to represent a sufficient cluster of signs and symptoms to make up a syndromic diagnostic entity.     

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.