Clinical and neurophysiological changes in patients with pineal region expansions [Kliničke i neurofiziološke značajke u bolesnika s ekspanzivnim tvorbama pinealne regije]

Hajnšek, Sanja and Paladino, Josip and Petelin Gadže, Željka and Nanković, Sibila and Mrak, Goran and Lupret, Velimir (2013) Clinical and neurophysiological changes in patients with pineal region expansions [Kliničke i neurofiziološke značajke u bolesnika s ekspanzivnim tvorbama pinealne regije]. Collegium Antropologicum, 37 (1). pp. 35-40. ISSN 0350-6134

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Abstract

In the last 20 years neurological and neurosurgical follow up of our patients with pineal region expansions (118 patients) pointed to certain clinical and neurophysiological regularities. We performed retrospective study which included 84 patients with pineal region expansions in the period from 1992 to 2009. The study included 55 women and 29 men, mean age 30.08 +/- 13.93 years, with positive brain magnetic resonance imaging (MRI)--70 patients (83.4%) had simple pineal gland cysts, and 14 patients (16.67%) had expansive process in pineal region with compressive effect. All patients had headache, while 32 patients (38%) had epileptic phenomena--primary generalized seizures. Patients had common electroencephalography (EEG) pattern with paroxysmal discharges of 3Hz (or more than 3 Hz) spike-and-wave complexes. Operation with supracerebellar infratentorial approach was performed in 70 patients. In most of our patients indication for the operation was established based on the size of the cyst (15 mm or more), with the signs of compression on the quadrigeminal plate and compression of the surrounding veins, which could result in seizures and EEG changes verified in our group of patients. Pathohistological analysis revealed pineocytomas in 11 cases (15.71%), pinealoblastomas in 2 cases (2.86%), one case of teratoma (1.43%), while 56 patients had pineal gland cysts (80%). Following surgery clinical condition improved in all patients--patients became seizure-free and headaches significantly decreased. Other symptoms including diplopiae, nausea, vomiting, vertigo as well as blurred vision also disappeared. There were no complications after surgical procedures. This study points to often appearance of seizures that clinically and neurophysiologically present as primary generalized epilepsy in patients with pineal region expansions. Our hypotheses are that mass effect on the surrounding veins that affects normal perfusion, compressive effect on the quadrigeminal plate and the aqueduct of the midbrain, hemosiderin deposists, as well as secretion disturbances of anticonvulsive agent melatonin can be involved in the pathogenesis of seizures. We suggest to perform high resolution brain MRI with special demonstration of pineal region in all young patients that have seizures and specific EEG changes.

Abstract in Croatian

U posljednjih 20-tak godina neurološko i neurokirurško praćenje naših bolesnika s ekspanzivnim tvorbama pinealne regije (118 bolesnika) ukazalo je na određene kliničke i neurofiziološke značajke. Stoga smo se odlučili učiniti retrospektivnu studiju koja je uključila 84 bolesnika s ekspanzivnim tvorbama pinealne regije u periodu od 1992. do 2009. godine. Studija je uključila 55 žena i 29 muškaraca, srednje životne dobi 30,08±13,93 godina, s pozitivnim nalazom magnetske rezonance (MR) mozga – 70 bolesnika (83,4%) imalo je jednostavne ciste pinealne žlijezde, dok je 14 bolesnika (16,67%) imalo ekspanzivni proces pinealne regije s kompresivnim učinkom. Svi su bolesnici imali glavobolje, dok je 32 bolesnika (38%) imalo primarno generalizirane epileptičke napadaje. Bolesnici su imali uobičajen elektroencefalografski (EEG) uzorak s paroksizmalnim izbijanjima šiljak-val kompleksa frekvencije 3Hz (ili više od 3Hz). Operativni zahvat sa supracerebelarnim infratentorijskim pristupom izvršen je u 70 bolesnika. U većine bolesnika indikacija za operaciju postavljena je temeljem veličine pinealne ciste (15 mm ili više), uz znakove kompresije lamine kvadrigemine i okolnih vena, što može rezultirati epileptičkim napadajima i EEG promjenama koje su verificirane u našoj grupi bolesnika. Patohistološka analiza otkrila je da se u 11 slučajeva (15,71%) radilo o pineocitomima, u 2 slučaja (2,86%) o pineoblastomima, u jednom slučaju o teratomu (1,43%), dok je 56 (80%) bolesnika imalo cistu pinealne žlijezde. Nakon operacije došlo je do poboljšanja kliničkog stanja u svih bolesnika – bolesnici više nisu imali epileptičke napadaje, a glavobolje su se značajno smanjile. Drugi simptomi uključujući dvoslike, mučninu, povraćanje, vrtoglavicu te zamagljenje vida su također nestali. U postoperativnom tijeku nisu zabilježene komplikacije. Ova studija ukazuje na učestalu pojavu epileptičkih napadaja, koji se klinički i neurofiziološki prezentiraju kao primarno generalizirana epilepsija, u bolesnika s ekspanzivnim tvorbama pinealne regije. Naše hipoteze su da u patogenezu epileptičkih napadaja mogu biti uključeni: kompresivni učinak ekspanzivnih tvorbi pinealne regije na okolne vene čime se remeti uredna perfuzija, kompresivni učinak na laminu kvadrigeminu i mezencefalički akvedukt, hemosiderinski depoziti, kao i smetnje izlučivanja melatonina koji ima antikonvulzivni učinak. Preporučamo da se u svakog bolesnika mlađe životne dobe sa specifičnim EEG promjenama učini visokorezolucijski MR mozga sa specijalnim prikazom pinealne regije.

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