Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich.

We present the case of a 7-month-old baby with Cushing’s disease due to an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma combined with cells producing thyreotropin-secreting hormone (TSH). In MRI scans, a contrast-enhancing lesion was seen inside the pituitary fossa, and it extended into the suprasellar region. On the assumption of a pituitary adenoma, surgery was performed. Corresponding with biochemical findings, histopathological evaluation revealed an ACTH- and TSH-producing tumor. Genetic analysis did not demonstrate an alteration at codon 201 (Arg) and 227 (Glu). To our knowledge, this is the first case described in a child of this age. / Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich.

Objetivos: Malformação de Chiari do tipo I (MC-I) é a principal doença malformativa congênita da junção craniovertebral, manifestando-se com ampla variedade de sinais e sintomas neurológicos. A melhor técnica cirúrgica a ser empregada no tratamento dos pacientes com malformação de Chiari do tipo I é ainda controversa. A descompressão das estruturas da fossa craniana posterior com plástica de ampliação dural é considerada procedimento padrão. Embora efetiva e de baixa morbidade, a craniectomia occipital isolada, sem abertura e ampliação dural, implica maior taxa de recidiva dos sintomas. Métodos que selecionam os pacientes quanto a necessidade da duroplastia não foram estabelecidos. O presente trabalho avalia a eficácia da mensuração intraoperatória da velocidade do fluxo do líquido cefalorraquidiano através da ultrassonografia (USG) na seleção da técnica cirúrgica a ser utilizada. Métodos: Foram analisados de forma prospectiva 49 pacientes submetidos à cirurgia para MC-I. A indicação de craniectomia da fossa posterior associada ou não à plástica de ampliação da dura-máter baseou-se na velocidade do fluxo do líquido cefalorraquidiano, mensurada pela ultrassonografia intraoperatória. Dor cervical, cefaleia e qualidade de vida foram avaliadas antes e após o tratamento cirúrgico. Resultados: Dos 49 pacientes incluídos, 36 pacientes (73%) apresentavam fluxo do líquido cefalorraquidiano superior a 3 cm/s e não foram submetidos a duroplastia ampliadora. Nos 13 (27%) pacientes com fluxo inicial inferior a 3 cm/s, indicou-se craniectomia occipital com duroplastia de ampliação. Não houve diferença significativa entre os dois grupos com relação aos parâmetros estudados. Conclusão: A ultrassonografia intraoperatória com avaliação da dinâmica e velocidade do fluxo do líquido cefalorraquidiano da junção craniovertebral auxilia a indicação de duroplastia durante descompressão da fossa craniana posterior em pacientes adultos com MC-I / Objectives: Chiari malformation Type I (CM-I) is the main congenital malformation disease of the craniovertebral junction, and may be responsible for a variety of neurological symptoms. The ideal surgical technique used to treat patients with CM-I is still controversial. Invasive procedures that enters CSF space and are associated with dural repair, are considered the gold standard. Although effective and less morbidity, isolated bone decompression without dural opening, implies greater recurrence of symptoms. Objective parameters to select patients, who need or not to have a duroplasty performed, have not been established. Our study evaluates the efficacy of intra-operative CSF flow measurement through the use of ultrasonography (USG) as a determining parameter in the selection of these patients. Methods: We analyzed prospectively 49 posterior fossa surgeries for patients with CM-I. Patients underwent decompressive surgery with or without opening of the dura mater after conducting USG intra-operatively with measured flow rate, being adopted 3cm/s flow rate as a determining value. The quality of life before and after surgery and the improvement of neck pain and headache were the parameters evaluated. Results: Of the 49 patients enrolled, 36 patients (73%) had adequate CSF flow above 3 cm / s and have not undergone duroplasty. In 13 (27%) patients with initial flow < 3 cm / s an opening in dura mater was performed together with duroplasty. There was no significant difference between the two groups regarding the parameters studied. Conclusion: Intraoperative ultrasound with measurement of CSF flow, having a flow of 3 cm / s as cut-off, allows the proper selection of patients with CM-I that can have a less invasive surgery with bone decompression without duroplasty

Objetivos: A dor neuropática crônica origina-se em consequência direta de uma lesão ou doença que afete o sistema somato-sensitivo. Pacientes que são refratários ao tratamento conservador são considerados candidatos a procedimentos invasivos, principalmente de ordem neuro-modulatória. A estimulação do gânglio da raiz dorsal é uma técnica recente de neuromodulação utilizada para o tratamento das dores neuropáticas crônicas de diferentes etiologias. Apesar do sucesso clínico da estimulação do gânglio da raiz dorsal no tratamento de dor neuropática já ter sido relatado em diversos trabalhos da literatura, os mecanismos neurofisiológicos responsáveis pelo alivio da dor ainda permanecem pouco esclarecidos. O presente trabalho avalia o efeito da estimulação do gânglio da raiz dorsal no processamento cortical da dor através do método de potenciais evocados a laser (PELs). Métodos: Avaliamos prospectivamente por 3 anos 34 doentes com dor inguinal (grupo 1), 62 doentes com dores neuropáticas diversas (grupo 2) que foram submetidos a estimulação do gânglio da raiz dorsal. Nestes 2 grupos foram analisadas variáveis relacionadas a intensidade da dor, incapacidade relacionada a dor, níveis de depressão e pensamentos catastróficos relacionados a dor. Adicionalmente, um subgrupo de 12 pacientes submetidos à cirurgia de estimulação do gânglio da raiz dorsal para tratamento de dor neuropática crônica unilateral da região inguinal, joelho ou perna por lesão direta de nervo periférico após procedimento cirúrgico, síndrome do insucesso da cirurgia espinhal ou síndrome dolorosa regional complexa tipo II foi avaliado de forma prospectiva (grupo 3). O lado normal foi utilizado como controle. PELs foram evocados por meio de estimulação a laser de CO2 na área desaferentada e normal. Latências e amplitudes dos componentes N2 e P2 e amplitudes do complexo N2-P2 foram correlacionados à intensidade da dor antes da terapia e após 1 e 6 meses de tratamento com estimulação do gânglio da raiz dorsal. Testes sensitivos quantitativos, escalas de intensidade da dor, incapacidade relacionada a dor, qualidade de vida, e depressão foram avaliadas. Resultados: Houve aumento significante das amplitudes do complexo N2-P2, igualando-se ao lado normal, e em paralelo diminuição significativa da intensidade de dor e na incapacidade relacionada à dor após 1 e 6 meses de tratamento em comparação com o estado pré-tratamento. Houve melhora significativa em 2 dos 8 itens de qualidade de vida avaliados. Não houve alteração significativa nos testes sensitivos quantitativos e na escala de depressão. Conclusão: A estimulação do gânglio da raiz dorsal restabeleceu as amplitudes dos PELs oriundos do giro do cíngulo anterior, ínsula e áreas temporais mediais, restaurando o processamento cortical fisiológico de dor em pacientes com dor neuropática crônica / Objectives: Chronic neuropathic pain originates as a direct consequence of an injury or disease that affects the somatosensory system. Patients who are refractory to conservative treatment are considered candidates for invasive procedures, mainly of neuro-modulatory order. Dorsal root ganglion stimulation is a recent neuromodulation technique used for the treatment of chronic neuropathic pain of different etiologies. Although the clinical success of dorsal root ganglion stimulation in the treatment of neuropathic pain has already been reported in several studies in the literature, the neurophysiological mechanisms responsible for pain relief remain unclear. The present study evaluates the effect of dorsal root ganglion stimulation on cortical pain processing through the use of laser evoked potentials (LEP). Methods: We evaluated prospectively during 3 years 34 patients with groin pain (group 1), 62 patients with various forms of neuropathic pain (group 2) who underwent dorsal root ganglion stimulation. In these 2 groups, variables related to pain intensity, pain-related disability, depression levels and painrelated catastrophic thoughts were analyzed. In addition, we prospectively analyzed a subgroup of 12 patients treated with dorsal root ganglion stimulation for treatment of chronic unilateral neuropathic pain of the groin region, knee or leg caused by direct injury of the peripheral nerve after surgical procedure, failed back surgery syndrome or complex regional pain syndrome type II (group 3). The healthy side was used as control. LEPs were evoked by means of CO2 laser stimulation in the deafferented and normal areas. Latencies and amplitudes of the N2 and P2 components and amplitudes of the N2-P2 complex were correlated to the pain intensity before therapy and after 1 and 6 months of treatment with dorsal root ganglion stimulation. Quantitative sensory testing, pain intensity scales, pain-related disability, quality of life, and depression were assessed. Results: There was a significant increase in N2-P2 complex amplitudes, matching the normal side, and in parallel a significant decrease in pain intensity and pain-related disability after 1 and 6 months of treatment compared to the pre-treatment state. There was a significant improvement in 2 out of 8 quality of life domains evaluated. There was no significant change in quantitative sensory testing and depression levels. Conclusion: Stimulation of the dorsal root ganglion reestablished the amplitudes of the LEPs originated from the anterior cingulate gyrus, insula, and medial temporal areas, restoring physiological cortical pain processing in patients with chronic neuropathic pain

Objetivos: Malformação de Chiari do tipo I (MC-I) é a principal doença malformativa congênita da junção craniovertebral, manifestando-se com ampla variedade de sinais e sintomas neurológicos. A melhor técnica cirúrgica a ser empregada no tratamento dos pacientes com malformação de Chiari do tipo I é ainda controversa. A descompressão das estruturas da fossa craniana posterior com plástica de ampliação dural é considerada procedimento padrão. Embora efetiva e de baixa morbidade, a craniectomia occipital isolada, sem abertura e ampliação dural, implica maior taxa de recidiva dos sintomas. Métodos que selecionam os pacientes quanto a necessidade da duroplastia não foram estabelecidos. O presente trabalho avalia a eficácia da mensuração intraoperatória da velocidade do fluxo do líquido cefalorraquidiano através da ultrassonografia (USG) na seleção da técnica cirúrgica a ser utilizada. Métodos: Foram analisados de forma prospectiva 49 pacientes submetidos à cirurgia para MC-I. A indicação de craniectomia da fossa posterior associada ou não à plástica de ampliação da dura-máter baseou-se na velocidade do fluxo do líquido cefalorraquidiano, mensurada pela ultrassonografia intraoperatória. Dor cervical, cefaleia e qualidade de vida foram avaliadas antes e após o tratamento cirúrgico. Resultados: Dos 49 pacientes incluídos, 36 pacientes (73%) apresentavam fluxo do líquido cefalorraquidiano superior a 3 cm/s e não foram submetidos a duroplastia ampliadora. Nos 13 (27%) pacientes com fluxo inicial inferior a 3 cm/s, indicou-se craniectomia occipital com duroplastia de ampliação. Não houve diferença significativa entre os dois grupos com relação aos parâmetros estudados. Conclusão: A ultrassonografia intraoperatória com avaliação da dinâmica e velocidade do fluxo do líquido cefalorraquidiano da junção craniovertebral auxilia a indicação de duroplastia durante descompressão da fossa craniana posterior em pacientes adultos com MC-I / Objectives: Chiari malformation Type I (CM-I) is the main congenital malformation disease of the craniovertebral junction, and may be responsible for a variety of neurological symptoms. The ideal surgical technique used to treat patients with CM-I is still controversial. Invasive procedures that enters CSF space and are associated with dural repair, are considered the gold standard. Although effective and less morbidity, isolated bone decompression without dural opening, implies greater recurrence of symptoms. Objective parameters to select patients, who need or not to have a duroplasty performed, have not been established. Our study evaluates the efficacy of intra-operative CSF flow measurement through the use of ultrasonography (USG) as a determining parameter in the selection of these patients. Methods: We analyzed prospectively 49 posterior fossa surgeries for patients with CM-I. Patients underwent decompressive surgery with or without opening of the dura mater after conducting USG intra-operatively with measured flow rate, being adopted 3cm/s flow rate as a determining value. The quality of life before and after surgery and the improvement of neck pain and headache were the parameters evaluated. Results: Of the 49 patients enrolled, 36 patients (73%) had adequate CSF flow above 3 cm / s and have not undergone duroplasty. In 13 (27%) patients with initial flow < 3 cm / s an opening in dura mater was performed together with duroplasty. There was no significant difference between the two groups regarding the parameters studied. Conclusion: Intraoperative ultrasound with measurement of CSF flow, having a flow of 3 cm / s as cut-off, allows the proper selection of patients with CM-I that can have a less invasive surgery with bone decompression without duroplasty

Σκοπός της παρούσης εργασίας είναι η βιβλιογραφική αναζήτηση, παράθεση και επιβεβαίωση παλαιότερων τεχνικών που έχουν χρησιμοποιηθεί κατά καιρούς για τη βέλτιστη στόχευση και ηλεκτρικό ερεθισμό δομών των βασικών γαγγλίων και του θαλάμου, αλλά και η αναζήτηση νέων τεχνικών που θα μπορούσαν να χρησιμοποιηθούν τόσο κατά τη διάρκεια του χειρουργείου όσο και μετά από αυτό. Το κύριο μέρος της εργασίας είναι η εύρεση νέων συνδυαστικών τεχνικών οι οποίες επηρεάζουν τη στόχευση των εν τω βάθει πυρήνων, και πιο συγκεκριμένα την διεγχειρητική στόχευση του υποθαλάμιου πυρήνα κατά την διάρκεια στερεοτακτικής και λειτουργικής νευροχειρουργικής Παρκινσονικών ασθενών, καθώς και ο καθορισμός συγκεκριμένων τροχιών μικρο/μακροηλεκτροδίων οι οποίες να μπορούν να εγγυηθούν μακροχρόνια θετικά κλινικά αποτελέσματα. Προτού αναφέρουμε λεπτομερώς αυτές τις τεχνικές, αναλύουμε τον τρόπο λειτουργίας του εν τω βάθει εγκεφαλικού διεγέρτη, αναφέρουμε κάποια ιστορικά ορόσημα στην ανάπτυξη της στερεοτακτικής και λειτουργικής νευροχειρουργικής και αναφέρουμε τους διαφόρους πυρήνες που έχουν διεγερθεί κατά καιρούς για την αντιμετώπιση των συμπτωμάτων διαφόρων νευρολογικών παθήσεων. Επίσης αναφέρουμε αρκετά ανατομικά στοιχεία (συνοδεία σχεδίων) των υπό διέγερση περιοχών καθώς και τη φυσιολογία που εμπλέκεται έτσι ώστε να έχουμε μείωση ή και εξάλειψη των διαφόρων νευρολογικών/ψυχιατρικών συμπτωμάτων. Αναφερόμαστε στη γενικότερη εφαρμογή του ηλεκτρισμού στην ιατρική καλύπτοντας τόσο το κεντρικό όσο και το περιφερειακό νευρικό σύστημα, σε ασθένειες οι οποίες παρουσιάζουν νευρολογικές και ψυχιατρικές εκφάνσεις, ενώ αναλύουμε και διεξοδικά τον μηχανισμό δράσης νευρολογικών ασθενειών από μοριακό επίπεδο μέχρι των αλλαγών που παρατηρούνται στα μετρήσιμα ηλεκτρικά πεδία τόσο των εν τω βάθει δομών όσο και του φλοιού. Στο τελευταίο κεφάλαιο αυτής της εργασίας αναλύουμε τις μετρήσεις μας που ελήφθησαν από 7 Παρκινσονικούς ασθενείς κατά τη διάρκεια χειρουργείου για την στόχευση και ερεθισμό του υποθαλάμιου πυρήνα, και αναφέρουμε πως με τη χρήση μη γραμμικής δυναμικής και χάους μπορούμε να επιτύχουμε το βέλτιστο κλινικό αποτέλεσμα. / The objective of the present thesis is the bibliographical research, instantiation and confirmation of various techniques that have been occasionally used for the most optimal targeting and electric stimulation of basal ganglia nuclei and thalamus, as well as the finding of new innovative techniques that can be used so much intraoperatively as much postoperatively. The main part of this thesis is the finding of new combined techniques that influence the targeting of deep brain nuclei, and more specifically the targeting of subthalamic nucleus during functional neurosurgery in parkinsonian patients, as well as the determination of specific trajectories of micro/macroelectrodes which can guarantee long-lasting positive clinical results. Before we report in detail these techniques, we analyze the function of the deep brain stimulator, we report certain historical landmarks in the growth of stereotactic and functional neurosurgery and we report the various nuclei that they occasionally have been stimulated for the amelioration of symptoms of various neurological diseases. Also we report many anatomical information (accompanied by drawings) of the areas under stimulation as well as the physiology that is involved so as to induce amelioration of various neurological/psychiatric symptoms. We have analyzed the more general application of electricity in the medicine covering so much the central as much the peripheral nervous system, the symptoms of diseases that present neurological and psychiatric manifestations, while we have analyzed in depth the mechanism of action of neurological diseases from molecular level up to the changes that are observed in the measurable electric fields from both deep brain nuclei and the cerebral cortex. In the last chapter of this thesis we analyze the electric activity that was measured intraoperatively from scalp and deep brain electrodes of 7 parkinsonian patients during targeting and stimulation of the subthalamic nucleus, and we report that with the use of non linear dynamics and chaos we can achieve the most optimal clinical result.