Neurofibromatoza tipa 1: klinička, patologijska i radiologijska korelacija

Type 1 neurofibromatosis is a phacomatosis inherited as an autosomal dominant disorder. It is characterized by the occurrence of hamartomas and tumors on the body, particularly on the nervous system and skin. The clinical criteria for its diagnosis include the following findings: six or more cafe-au-lait spots of 35 mm in diameter on the skin, two or more neurofibromas, spots in the axillary or inguinal region, optic nerve gliomas, two or more hamartomas of the iris, and characteristic changes of bones and brain. The pathologist\u27s finding is predominated by the occurrence of neurofibromas along peripheral and cranial nerves, optical gliomas, policystic astrocytomas of the brain, and hamartomas of the brain and iris. During a 15-year period, 166 children with type 1 neurofibromatosis were examined and radiologically evaluated. Classical radiological bone x-rays were made, along with brain and spine CT and MR. Dysplasia of the greater wing of the sphenoid bone was detected on bone tissue accompanied by deformation of the orbit and middle cranial fossa, wedge-formed vertebrae, expanded intervertebral foramina, and cystic masses in other bones. In the brain parenchyma, hamartomas, policystic astrocytomas and optic nerve gliomas were detected along with neurinomas of the cranial and spinal nerves. Based on the clinical, laboratory and radiologic follow-up of the patients with type 1 neurofibromatosis, the need is stressed for a multiple approach to the diagnosis and treatment of neurofibromatosis.Neurofibromatoza tipa 1 je fakomatoza koja se nasljeđuje autosomno dominantno, a obilježena je pojavom hamartoma i tumora po tijelu, poglavito živčanog sustava i kože. Klinički kriteriji za postavljanje dijagnoze temelje se na nalazima: šest ili više pjega boje bijele kave promjera većeg od 5 mm na koži, dva ili više neurofibroma, pjegavosti pazušnih i ingvinalnih regija, optičkog glioma, dva ili više hamartoma šarenice i znakovitim promjenama na kostima i mozgu. U nalazu patologa dominira pojava neurofibroma duž perifernih i kranijskih živaca, optičkih glioma, policističnog astrocitoma mozga te hamartoma mozga i šarenice. U tijeku 15-godišnjeg razdoblja pregledano je i radiološki obrađeno 166 djece s neurofibromatozom tipa 1. Primijenjene su klasične radiološke metode snimanja kostiju, CT mozga i kralješnice te MR mozga i kralješnice. Na koštanom tkivu utvrđena je displazija velikog krila sfenoida praćena deformacijom orbite i srednje lubanjske jame, klinasto oblikovani kralješci, prošireni intervertebralni otvori te cistične tvorbe u dugim kostima. U parenhimu mozga utvrđeni su hamartomi, policistieniastrocitomi i gliomi optikusa te neurinomi kranijskih i spinalnih živaca. Na temelju kliničkog, laboratorijskog i radiologijskog praćenja bolesnika s neurofibromatozom tipa 1 ukazano je na potrebu višestrukog pristupa u dijagnostici i liječenju neurofibromatoze

Bilateral MR Volumetry of the Amygdala in Chronic PTSD Patients

Posttraumatic stress disorder (PTSD) patients experience symptoms which implicate dysfunction of emotional memory circuits, and possible damage of the amygdala. Laterality differences in activity of the amygdala have been reported in PTSD patients, with presumed adaptive plasticity in the hippocampus and the amygdala. The aim of this study was to investigate possible interhemispheric differences of amygdalar volume in chronic PTSD patients, with calculation of right-to-left volume ratios. Bilateral magnetic resonance (MR) volumetry was applied in 11 chronic PTSD patients. The mean right amygdalar volume of our patients was significantly smaller than the left one (p=0.031), with the right-to-left volume ratio of 0.96±0.06. This tendency towards smaller right amygdala may be an acquired effect as a result of stress-induced plasticity, however we can not exclude the possibility of a predisposing condition

Bilateral MR volumetry of the amygdala in chronic PTSD patients

Posttraumatic stress disorder (PTSD) patients experience symptoms which implicate dysfunction of emotional memory circuits, and possible damage of the amygdala. Laterality differences in activity of the amygdala have been reported in PTSD patients, with presumed adaptive plasticity in the hippocampus and the amygdala. The aim of this study was to investigate possible interhemispheric differences of amygdalar volume in chronic PTSD patients, with calculation of right-to-left volume ratios. Bilateral magnetic resonance (MR) volumetry was applied in 11 chronic PTSD patients. The mean right amygdalar volume of our patients was significantly smaller than the left one (p = 0.031), with the right-to-left volume ratio of 0.96 +/- 0.06. This tendency towards smaller right amygdala may be an acquired effect as a result of stress-induced plasticity, however we can not exclude the possibility of a predisposing condition

Minor head trauma and isolated unilateral internuclear ophthalmoplegia

Internuclear ophthalmoplegia is a syndrome that develops due to a lesion of the medial longitudinal fasciculus. This lesion is mostly caused by multiple sclerosis (usually bilaterally), and only rarely by head injury. A case is presented of unilateral internuclear ophthalmoplegia as an isolated sequel of minor head trauma, which eventually resolved. A 40-year-old woman with isolated internuclear ophthalmoplegia secondary to closed head trauma with anatomical lesions of the mesencephalon in the region of medial longitudinal fasciculus is described. A minor contusion was detected by magnetic resonance imaging. Diplopia resolved in 5 months. In conclusion, internuclear ophthalmoplegia should be considered in the differential diagnosis in patients with recent head injuries showing adduction impairment. The connection between the clinical picture and anatomical lesions is visualized by magnetic resonance imaging