Seizure after aneurysmal subarachnoid hemorrhage

Seizure after aneurysmal subarachnoid hemorrhage

Epilepsy is a common and serious complication of subarachnoid hemorrhage (SAH), giving rise to increased morbidity and mortality. It’s difficult to identify patients at high risk of epilepsy and the application of antiepileptic drugs (AEDs) following SAH is a controversial topic. Therefore, it’s pressingly needed to gain a better understanding of the risk factors, underlying mechanisms, and the optimization of therapeutic strategies for epilepsy after SAH. Neuroinflammation, characterized by microglial activation and the release of inflammatory cytokines has drawn growing attention due to its influence on patients with epilepsy after SAH. In a review, Wang et al. discussed the risk factors for epilepsy after SAH and emphasize the critical role of microglia. Then they discussed how various molecules arising from pathophysiological changes after SAH activates specific receptors such as TLR4NLRP3RAGEP2X7R and initiate the downstream inflammatory pathways. Additionally, they focused on the significant responses implicated in epilepsy including neuronal excitotoxicity, the disruption of the blood-brain barrier (BBB), and the change of immune responses. As the application of AEDs for seizure prophylaxis after SAH remains controversial, the regulation of neuroinflammation targeting the key pathological molecules could be a promising therapeutic method. While neuroinflammation appears to contribute to epilepsy after SAH, more comprehensive experiments on their relationships are needed 1).

Literature has reported seizure rates to be as high as 27% in this population 2).

More recently published studies have found seizure rates to be significantly lower than previously described (1–10%) 3) 4).

Seizure activity has been associated with secondary neurologic injury including reduced cerebral blood flow and intracranial hypertension 5).

see Anticonvulsant in aneurysmal subarachnoid hemorrhage.


1)

Wang J, Liang J, Deng J, Liang X, Wang K, Wang H, Qian D, Long H, Yang K, Qi S. Emerging Role of Microglia-Mediated Neuroinflammation in Epilepsy after Subarachnoid Hemorrhage. Mol Neurobiol. 2021 Jan 26. doi: 10.1007/s12035-021-02288-y. Epub ahead of print. PMID: 33501625.
2)

Lin YJ, Chang WN, Chang HW, Ho JT, Lee TC, Wang HC, Tsai NW, Tsai MH, Lu CH. Risk factors and outcome of seizures after spontaneous aneurysmal subarachnoid hemorrhage. Eur J Neurol. 2008 May;15(5):451-7. doi: 10.1111/j.1468-1331.2008.02096.x. Epub 2008 Mar 5. PubMed PMID: 18325027.
3)

Rosengart AJ, Huo JD, Tolentino J, Novakovic RL, Frank JI, Goldenberg FD, Macdonald RL. Outcome in patients with subarachnoid hemorrhage treated with antiepileptic drugs. J Neurosurg. 2007 Aug;107(2):253-60. PubMed PMID: 17695377.
4)

Chumnanvej S, Dunn IF, Kim DH. Three-day phenytoin prophylaxis is adequate after subarachnoid hemorrhage. Neurosurgery. 2007 Jan;60(1):99-102; discussion 102-3. PubMed PMID: 17228257.
5)

Rhoney DH, Tipps LB, Murry KR, Basham MC, Michael DB, Coplin WM. Anticonvulsant prophylaxis and timing of seizures after aneurysmal subarachnoid hemorrhage. Neurology. 2000 Jul 25;55(2):258-65. PubMed PMID: 10908901.

Giant Hypothalamic Hamartoma

Giant Hypothalamic Hamartoma

Giant hypothalamic hamartomas (GHH) are extremely rare lesions in infants and usually intrinsically epileptogenic.

Giant HH is an exceptionally difficult neurological disease. Primary hypofractionated GKRS may be an alternative approach as mono/multitherapy with promising results and minimal complication 1).

Although the exploration of epileptic activity and the extent of ablation are limited by the number of SEEG electrodes for the complete disconnection. One-stage high-density focal stereo-array SEEG-guided radiofrequency was safe and effective for treating pediatric giant HH patients. It can be an alternative method to treat giant HHs where LITT is unavailable 2).

Stereotactic radiofrequency thermocoagulation (SRT) provided minimal invasiveness and excellent seizure outcomes even in patients with giant HHs 3).

Three pediatric patients (age range 17-65 mo) underwent primary hypofractionated GKRS in 2-3 consecutive days with an interfraction interval of 24 h. All patients had precocious puberty and were on GnRH analog. Frame-based GKRS was done with 8.1-9.2 Gy radiation per fraction at 50% isodose in 2-3 fractions targeting the entire hamartoma volume. The mean target volume was 5.67 cc (4.45-7.39 cc). The authors followed these patients for clinical and endocrinological assessment at every 6 mo interval while the repeat MRI has done at 6 mo and then annually. The seizure outcome analysis was done using the Engel Epilepsy Surgery Outcome Scale.

At a mean follow up of 27 mo (24-30 mo), 2 patients became Engel class 3 while one achieved Engel class 1 control. 2 patients showed halted pubertal growth with no additional hormonal aberration. 2 patients showed significant volumetric reduction (48% and 32%) and patchy necrosis inside the hypothalamic hamartoma (HH). There was no deficit in visual function, memory and cognition. One patient showed reduction in aggressiveness.

Giant HH is an exceptionally difficult neurological disease. Primary hypofractionated GKRS may be an alternative approach as mono/multitherapy with promising results and minimal complication 4)


Wang et al. analyzed the clinical data of six patients with giant HHs (masses with a maximum diameter >30 mm) who underwent stereotactic electrode implantation between November 2017 and April 2019. After a multidisciplinary discussion, we designed a high-density focal stereo-array electrode implantation strategy. SEEG-guided bipolar coagulations were performed between two contiguous contacts of the same electrode, or between two adjacent contacts of different electrodes. Results: Among the six patients, three were male and three were female, with an average age of 5.08 ± 4.73 years (range, 1.4-12 years); the average follow-up duration was 20.17 ± 5.49 months. One patient had previously undergone open surgery. Four patients had gelastic seizures, one had gelastic and tonic seizures, and one had gelastic and generalized tonic-clonic seizures. The number of implanted electrodes ranged from 3 to 7, with an average of 5.33. One patient had transient diabetes insipidus after the operation, and no child had fever or new hormone metabolisms disorder after surgery. Four patients had Engel I classification outcomes (free from disabling seizures), and two patients had Engel II classification outcomes. Conclusion: Although the exploration of epileptic activity and the extent of ablation are limited by the number of SEEG electrodes for the complete disconnection. One-stage high-density focal stereo-array SEEG-guided radiofrequency was safe and effective for treating pediatric giant HH patients. It can be an alternative method to treat giant HHs where LITT is unavailable 5).


Cristobal et al. presented a unique case of an asymptomatic giant hypothalamic hamartoma diagnosed prenatally by fetal magnetic resonance imaging and followed throughout infancy. This case demonstrates the utility of multimetric analysis using difference sequences, including diffuse-weighted imaging, to assess specific properties of intracranial lesions detected in utero and to aid in accurate diagnosis prior to birth 6).


A 10-month-old girl child presenting with drug-resistant seizures and a giant hypothalamic lesion that was confirmed as hamartoma on histopathology. Surgical decompression and disconnection from the hypothalamus was performed with the intent of controlling her seizures. Unfortunately, the patient developed right middle cerebral artery and posterior cerebral artery territory infarction, possibly due to vasospasm or thrombosis of the vessels. The patient had a stormy postoperative course but has recovered well neurologically at the 18-month follow-up. Histopathological examination revealed abnormal clusters of NeuN-positive neurons, which was confirmatory of hypothalamic hamartoma 7).


1) , 4)

Tripathi M, Maskara P, Sankhyan N, Sahu JK, Kumar R, Kumar N, Ahuja CK, Kaur P, Kaur R, Batish A, Mohindra S. Safety and Efficacy of Primary Hypofractionated Gamma Knife Radiosurgery for Giant Hypothalamic Hamartoma. Indian J Pediatr. 2021 Jan 27. doi: 10.1007/s12098-020-03637-w. Epub ahead of print. PMID: 33501606.
2) , 5)

Wang M, Zhou Y, Zhang Y, Shi W, Zhou S, Wang Y, Li H, Zhao R. One-Stage High-Density Focal Stereo-Array SEEG-Guided Radiofrequency Thermocoagulation for the Treatment of Pediatric Giant Hypothalamic Hamartomas. Front Neurol. 2020 Sep 2;11:965. doi: 10.3389/fneur.2020.00965. PMID: 32982954; PMCID: PMC7493627.
3)

Shirozu H, Masuda H, Ito Y, Sonoda M, Kameyama S. Stereotactic radiofrequency thermocoagulation for giant hypothalamic hamartoma. J Neurosurg. 2016 Oct;125(4):812-821. doi: 10.3171/2015.6.JNS15200. Epub 2016 Jan 1. PMID: 26722850.
6)

Cristobal A, Vorona G, Ritter A, Lanni S, Urbine J. Pre- and postnatal MR imaging of an asymptomatic giant hypothalamic hamartoma. Radiol Case Rep. 2020 Jun 16;15(8):1250-1255. doi: 10.1016/j.radcr.2020.05.041. PMID: 32577141; PMCID: PMC7303913.
7)

Kandregula S, Savardekar AR, Nandeesh BN, Arivazhagan A, Rao MB. Giant Hypothalamic Hamartoma in an Infant: A Case Report and Review of the Literature. Pediatr Neurosurg. 2017;52(1):55-61. doi: 10.1159/000448738. Epub 2016 Oct 26. PMID: 27780163.

Choroid plexus tumor

Choroid plexus tumor

Choroid plexus tumors are rare intraventricular papillary neoplasms derived from choroid plexus epithelium.

They account for approximately 2% to 4% of intracranial tumors in children and

Choroid plexus tumors occur more frequently in children, comprising approximately 4% of all pediatric brain tumors. Up to 20% of these tumors occur during the first year of life.

They account for 0.5% of intracranial tumors in adults.

Choroid plexus tumors most commonly arise from the lateral ventricles (50%), followed by the fourth (40%) and the third ventricle (5%). Other locations are rare, including the cerebellopontine angle, supresellar region, brain parenchyma and the spine.

They include three histologies, choroid plexus papilloma (WHO grade I), atypical choroid plexus papilloma (WHO grade II) and choroid plexus carcinoma (WHO grade III). All together, they account for 0.4-0.6% of all brain tumors 1) 2).

Results support the role of aggresome as a novel prognostic molecular marker for pediatric choroid plexus tumors (CPTs) that was comparable to the molecular classification in segregating samples into two distinct subgroups, and to the pathological stratification in the prediction of patients’ outcomes. Moreover, the proteogenomic signature of CPTs displayed altered protein homeostasis, manifested by enrichment in processes related to protein quality control 3).

see Choroid plexus metastases.

On CT, choroid plexus tumors appear heterogeneous and isodense with calcifications and necrosis.

Amer et al. examined the presence of aggresomes in 42 patient-derived tumor tissues by immunohistochemistry and we identified their impact on patients’ outcomes. We then investigated the proteogenomics signature associated with aggresomes using whole-genome DNA methylation and proteomic analysis to define their role in the pathogenesis of pediatric CPTs.

Aggresomes were detected in 64.2% of samples and were distributed among different pathological and molecular subgroups. The presence of aggresomes with different percentages was correlated with patients’ outcomes. The ≥ 25% cutoff had the most significant impact on overall and event-free survival (p-value < 0.001) compared to the pathological and the molecular stratifications.

These results support the role of aggresome as a novel prognostic molecular marker for pediatric CPTs that was comparable to the molecular classification in segregating samples into two distinct subgroups, and to the pathological stratification in the prediction of patients’ outcomes. Moreover, the proteogenomic signature of CPTs displayed altered protein homeostasis, manifested by enrichment in processes related to protein quality control 4).

2015

A total of 349 patients with CPTs were identified (120 CPCs, 26 aCPPs, and 203 CPPs). Patients with CPC presented at a younger age (median 3, mean 14.8 years) relative to CPP (median 25, mean 28.4 years; p < 0.0001). Histology was a significant predictor of OS, with 5-year OS rates of 90, 77, and 58 % for CPP, aCPP, and CPC, respectively. Older age and male sex were prognostic for worse OS and CSS for CPP. Only extent of surgery had a significant impact on survival for CPC. The use of adjuvant RT in patients with CPC undergoing surgery was not associated with a significantly improved OS (p = 0.17). For patients undergoing GTR without RT as part of an initial course of therapy, estimated 5- and 10-year OS were 70 % (±7 %) and 67 % (±8 %), respectively. Prospective data are required to define the optimal combination of surgery with adjuvant therapies, including chemotherapy 5).


Seventeen childhood patients were recorded with CPT. Cases were distributed so that 9 cases were choroid plexus-papilloma (CPP) (52.9%), 2 cases atypical CPP (11.7%) and 6 cases choroid plexus-carcinoma (CPC) (35.2%). Age at diagnosis was less than 2 years in 14 of the 17 patients (82.3%) and the incidence was higher in males (82.3% of the cases). Gross total resection was performed in 16 patients (94.1%). Adjuvant treatment was used in 6 patients (all this cases with CPC) (35.2%). Two of the 17 patients died (11.7%), showing an incidence density of 0.01 deaths/year.

The case series is consistent with previous published in scientific literature regarding epidemiology, tumor grade, clinical presentation, radiological features and therapeutic approach. Gross total resection is considered the therapeutic gold standard for choroid plexus tumors. Chemotherapy and radiotherapy should be used as adjuvant treatment in CPC and recurrent or remaining atypical CPP 6).


1)

Fuller CE, Narendra S, Tolocica I. Choroid plexus neoplasm. Adesina AM, Tihan T, Fuller C, Young Poussaint T, editors. , Atlas of pediatric brain tumors. New York: Springer Publication; 2010. pp 269-279
2)

Gopal P, Parker JR, Debski R, Parker JC., Jr. Choroid plexus carcinoma. Arch Pathol Lab Med 2008;132:1350-4
3) , 4)

Amer N, Taha H, Hesham D, Al-Shehaby N, Mosaab A, Soudy M, Osama A, Mahmoud N, Elayadi M, Youssef A, Elbeltagy M, Zaghloul MS, Magdeldin S, Sayed AA, El-Naggar S. Aggresomes predict poor outcomes and implicate proteostasis in the pathogenesis of pediatric choroid plexus tumors. J Neurooncol. 2021 Jan 26. doi: 10.1007/s11060-020-03694-3. Epub ahead of print. PMID: 33501605.
5)

Cannon DM, Mohindra P, Gondi V, Kruser TJ, Kozak KR. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol. 2015 Jan;121(1):151-7. doi: 10.1007/s11060-014-1616-x. Epub 2014 Oct 1. PubMed PMID: 25270349.
6)

Cuervo-Arango I, Reimunde P, Gutiérrez JC, Aransay A, Rivero B, Pérez C, Budke M, Villarejo F. [Choroid plexus tumour treatment at Hospital Infantil Niño Jesús in Madrid: Our experience over the last three decades.]. Neurocirugia (Astur). 2015 Feb 24. pii: S1130-1473(15)00005-6. doi: 10.1016/j.neucir.2015.01.001. [Epub ahead of print] Spanish. PubMed PMID: 25724620.
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