Deep Brain Stimulation in Non-motor Symptoms of Neurodegenerative Diseases

Deep brain stimulation (DBS) is a functional neuromodulatory technique that involves the use of a neurostimulator to deliver electrical impulses to the brain. It primarily alleviates the motor symptoms in neurodegenerative diseases; however, it has been found beneficial in a multitude of neurological and psychiatric diseases, such as dystonia, essential tremor, Tourette syndrome, intractable pain, epilepsy, treatment-resistant depression, and obsessive-compulsive disorder. Nonmotor symptoms, such as neurobehavioral disorders, autonomic dysfunction, sleep dysfunction, and somatosensory dysfunction, play an important role in neurodegenerative diseases and have a significant impact on the quality of life. The effects of deep brain stimulation on these symptoms are not yet apparent, although early results are promising and warrant future investigations. The main problem in interpretation is the lack of studies in this field, as most have methodological issues or small sample sizes, which limit the strength of the evidence. However, it is clear that DBS has a promising future in the treatment of neurodegenerative diseases in general and will have a vital role in personalized medicine as functional neuroimaging and our understanding of brain physiology improve

Deep brain stimulation – preoperative preparation and evaluation of patients with Parkinson’s disease

Parkinsonova bolest druga je najučestalija neurodegenerativna bolest. Najčešći simptomi pacijenata koji boluju od Parkinsonove bolesti su tremor u mirovanju, rigidnost, bradikinezija i poremećaj hoda. Liječenje Parkinsonove bolesti složeno je. Razlog tome je kompleksnost bolesti te dugogodišnja progresija. Liječenje može biti medikamentozno ili invazivnim metodama liječenja poput duboke mozgovne stimulacije. Kod medikamentoznog liječenja koristimo tri kategorije lijekova. Prva kategorija lijekova povećava razinu dopamina u mozgu, druga kategorija lijekova, kao što su antikolinergički lijekovi, smanjuje simptome Parkinsonove bolesti i treća kategorija djeluje na nemotoričke simptome Parkinsonove bolesti. Ako medikamentozno liječenje nije dostatno, danas se najviše primjenjuje duboka mozgovna stimulacija. Duboka mozgovna stimulacija je neurokirurška intervencija kojom se ugrađuje elektroda u područje subtalamičke jezgre, ventralne intermedijalne jezgre ili u područje globus pallidus, jer se motoričke komplikacije Parkinsonove bolesti ne mogu adekvatno kupirati lijekovima. Prije same neurokirurške intervencije važna je prijeoperacijska priprema i procjena pacijenata koji su potencijalni kandidati za ovakav zahvat. U prijeoperacijskoj pripremi i procjeni pacijenata treba sudjelovati multidisciplinarni tim stručnjaka koji uključuje psihologe, psihijatre, neurokirurge, a posebnu ulogu imaju kliničari, neurolozi, koji svoje pacijente najbolje poznaju te mogu procijeniti daljnju kvalitetu života pacijenta nakon ugradnje DBS-a.Parkinson\u27s disease is the second most common neurodegenrative disease. The most common symptoms of patients with Parkinson\u27s disease are rest tremor, rigidity, bradykinesia and gait disorder. The treatment of Parkinson\u27s disease is very complex. The reason for this is disease\u27s complexity and yearly progression. It can be medicamentous therapy or invasive methods like applying deep brain stimulation (DBS). There are three categories of medicamentous therapy that are used. The first category of drugs increases the level of dopamine in the brain, the second category of drugs reduces the symptoms of Parkinson\u27s disease, such as anticholinergic drugs and the third category of drugs is used for non-motor symptoms of Parkinson\u27s disease. If medicamentous therapy is not sufficient, deep brain stimulation is used today. Deep brain stimulation is the neurosurgical procedure involving the placement an electrode into the area of the subthalamic nucleus, ventral intermediate nucleus (VIM) in thalamus or globus pallidus because the motor complications of Parkinson\u27s disease can not be adequately eliminated with drugs. Before the neurosurgical procedure, the preoperative preparation and evaluation of the patients, who are potential candidates for this procedure, is very important. A complete medical evaluation is necessary for the preoperative preparation of patients. The preoperative preparation and evaluation of patients should involve a multidisciplinary team of experts including psychologists, psychiatrists, neurosurgeons, with a special role for clinicians, neurologists, who know their patients best and can evaluate the patients\u27 further quality of life after DBS is installed

Odgođeni učinak VNS-a na interiktalna epileptiformna izbijanja i farmakorezistentnost u bolesnice s refraktornom perinatalnom postishemijskom epilepsijom

A 20-year-old female with refractory perinatal postischemic catastrophic epilepsy and frequent daily generalized atonic, tonic, tonic-clonic and focal seizures was hospitalized in the progressive phase of illness. The diagnosis was confirmed by semiology, interictal electroencephalogram (EEG), long-term video EEG monitoring, and brain magnetic resonance imaging. Repeated interictal EEG findings showed generalized spike and slow wave complexes with a 2-3 Hz frequency. Interictal EEG showed evidence of electroclinical epileptic status on several occasions. She was treated with various antiepileptic drugs without improvement. After verification of her incompetence for normal autonomous living, which resulted in very low quality of life, this patient with refractory epilepsy underwent implantation of vagus nerve stimulator (VNS). In this case report, we present delayed effect of VNS on interictal epileptiform discharges and pharmacoresistance.Mlada žena u dobi od 20 godina s farmakorezistentnom perinatalnom postishemijskom epilepsijom i svakodnevnim učestalim generaliziranim atoničkim, toničkim, toničko-kloničkim te žarišnim napadima hospitalizirana je u pogoršanoj fazi bolesti. Dijagnoza je potvrđena kliničkom slikom, interiktalnim elektroencefalogramom (EEG), video EEG monitoriranjem te magnetskom rezonancom (MR) mozga. Ponavljani interiktalni nalazi EEG-a pokazali su generalizirana izbijanja šiljakval kompleksa frekvencije 2-3 Hz. U više navrata u interiktalnom EEG-u je evidentiran i elektroklinički epileptički status. Bolesnica je liječena različitim antiepilepticima, ali bez poboljšanja stanja. Zbog učestalih napada bolesnica nije bila sposobna za samostalan život, što je rezultiralo vrlo niskom kvalitetom života te je bolesnica podvrgnuta ugradnji vagusnog stimulatora (VNS). U ovom prikazu slučaja opisujemo odgođeni učinak VNS-a na interiktalna epileptiformna izbijanja i farmakorezistentnost

Extracranial propagation of glioblastoma with extension to pterygomaxillar fossa

<p>Abstract</p> <p>Background</p> <p>Glioblastoma multiforme is a highly malignant primary brain tumor that shows marked local aggressiveness, but extracranial spread is not a common occurrence. We present an unusual case of recurrent glioblastoma in 54-year old male that spread through the scull base to the ethmoid and sphenoid sinuses, to the orbita, pterygomaxillar fossa, and to the neck.</p> <p>Methods</p> <p>A 54-year old male underwent left temporal resection because of brain tumor of his left temporal lobe. Operation was followed by external beam radiation combined with temozolomide. The tumor recurred eight months after first surgery. The patient developed swelling of left temporal region, difficult swallowing and headache. MRI of head showed recurrent tumor, which invaded orbita, ethmoid and sphenoid sinuses, nasal cavity, pterygomaxillar fossa.</p> <p>Results</p> <p>The patient died ten months after initial diagnosis of glioblastoma multiforme, and two months after his second operation.</p> <p>Conclusions</p> <p>The aggressive surgical operation helped to downsize the tumor mass as much as possible, but did not prolonged significantly the life or improved the life quality of the patient. The current literature is reviewed, and the diagnostic approaches as well as therapeutic options are discussed.</p

GPi DBS treatment outcome in children with monogenic dystonia: a case series and review of the literature

IntroductionDystonia is the third most common pediatric movement disorder and is often difficult to treat. Deep brain stimulation (DBS) of the internal pallidum (GPi) has been demonstrated as a safe and effective treatment for genetic dystonia in adolescents and adults. The results of DBS in children are limited to individual cases or case series, although it has been proven to be an effective procedure in carefully selected pediatric cohorts. The aim of our study was to present the treatment outcome for 7- to 9-year-old pediatric patients with disabling monogenic isolated generalized DYT-THAP1 and DYT-KMT2B dystonia after bilateral GPi-DBS.Patients and resultsWe present three boys aged &lt;10 years; two siblings with disabling generalized DYT-THAP1 dystonia and a boy with monogenic-complex DYT-KMT2B. Dystonia onset occurred between the ages of 3 and 6. Significantly disabled children were mostly dependent on their parents. Pharmacotherapy was inefficient and patients underwent bilateral GPi-DBS. Clinical signs of dystonia improved significantly in the first month after the implantation and continued to maintain improved motor functions, which were found to have improved further at follow-up. These patients were ambulant without support and included in everyday activities. All patients had significantly lower Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS) values, indicating &gt;25% improvement over the first 15 months. However, there was a decline in speech and upper limb function, manifesting with bradylalia, bradykinesia, and dysphonia, which decreased after treatment with trihexyphenidyl.ConclusionAlthough reports of patients with monogenic dystonia, particularly DYT-THAP1, treated with DBS are still scarce, DBS should be considered as an efficient treatment approach in children with pharmacoresistent dystonia, especially with generalized monogenic dystonia and to prevent severe and disabling symptoms that reduce the quality of life, including emotional and social aspects. Patients require an individual approach and parents should be properly informed about expectations and possible outcomes, including relapses and impairments, in addition to DBS responsiveness and related improvements. Furthermore, early genetic diagnosis and the provision of appropriate treatments, including DBS, are mandatory for preventing severe neurologic impairments

Potential Error in Ventriculocisternal Perfusion Method for Determination of Cerebrospinal Fluid Formation Rate in Cats

The cerebrospinal fluid (CSF) formation rate (Vf) has been extensively studied by the ventriculocisternal perfusion, a method still regarded as the most precise one. This method as well as the equation for the calculation of the CSF formation rate (Vf) was established by Heisey et al4 on indicator dilution in perfusate. They assumed that the dilution of the indicator substance in perfusion is a consequence of newly formed CSF i.e. a higher CSF formation rate would result in a higher degree of dilution of the indicator substance. Therefore, such method is indirect and any mistake in the interpretation of the degree of indicator dilution would lead to questionable and often contradictory results regarding CSF formation rates. According to Heisey’s equation, Vf shoud not depend on the rate of ventriculocisternal perfusion. However it has been shown that Vf is perfusion dependt value10, and also that during perfusion the indicator substance is partially absorbed into surrounding tissue. It is possible that obtained Vf dependence on perfusion rate was caused by observed absorption of indicator substances. For that reason, in anaesthetised cats ventriculocisternal perfusion was performed at higher (252.0 mL/min) and at lower perfusion rate (65.5 mL/min) and Vf was calculated at both experimental and corrected (just for absorbed amount) values of indicator substance. Since (inspite of the correction) the difference of 12.4 mL/min between lower (15.0 mL/min) and higher perfusion rate (27.4 mL/min) was obtained, it is obvious that ventriculocisternal perfusion method cannot be considered reliable for measuring CSF formation rate

Long term tracking of pallidal deep brain stimulation used in generalized dystonia – case report

Cilj: Generalizirana distonija karakterizirana je nevoljnim kontrakcijama mišića koje dovode do pojave abnormalnog držanja i ponavljajućih pokreta. Duboka stimulacija mozga (engl. Deep Brain Stimulation, DBS) učinkovita je u distoniji otpornoj na lijekove, međutim, postoji manjak radova o dugoročnom učinku. Cilj rada je prezentirati dugoročni učinak primjene DBS-a kod pacijenata s generaliziranom distonijom. Prikaz slučaja: Pacijent je 29-godišnji muškarac s generaliziranom distonijom. Nakon rođenja primijećena je faciopareza po perifernom tipu, što je bilo pripisano cerebralnoj paralizi. Prvi simptomi, problemi s pisanjem zbog grčenja ruke, primijećeni su sa sedam godina. S petnaest godina primijećeni su problemi u govoru i pogoršanje distonije ruke. Distonične kretnje i držanje postupno se pogoršavalo, uzrokujući probleme s hodanjem i narušavajući kvalitetu života pacijenta. Simptomi se nisu popravljali unatoč primjeni farmakoterapije i kada je pacijent imao dvadeset i jednu godinu implantirane su mu elektrode u bazalne ganglije, što je drastično poboljšalo distonične kretnje, držanje i hod. Nakon operacije, unatrag osam godina, pacijent je redovito praćen, elektrode su reprogramirane uz kontinuiranu fizikalnu i terapiju govora. U dva navrata nastupio je povratak kliničke slike radi ispražnjene baterije neurostimulatora koja je prvi put zamijenjena nepunjivom, a drugi put punjivom baterijom, što je dovelo do potpunog oporavka. Zaključak: Idiopatska distonija često je neprepoznata. Iako je DBS sigurna i učinkovita metoda, nije dovoljno korištena za liječenje distonije otporne na lijekove. Ovaj slučaj pokazao je perzistentan i izvrstan učinak DBS-a na generaliziranu distoniju i pacijentovu kvalitetu života kroz duži vremenski period.Aim: Generalized dystonia is characterized by involuntary muscle contractions leading to abnormal postures and repetitive movements. Deep brain stimulation (DBS) is effective in medication-refractory dystonia, but there is a lack of studies about long-term effect. The goal of this case report is to show long-term effect in a patient with generalized dystonia treated with DBS. Case report: We present a case of a 29-years-old patient with isolated generalized dystonia. At birth, peripheral facial palsy was noticed which was attributed to cerebral paralysis. First symptoms noticed at 7-years-old were problems with handwriting due to hand spasms. At 15, speech problems started and dystonia of his hand worsened. Then, dystonic movements and postures gradually expanded causing walking problems and poor quality of life. Symptoms did not improve with available medications and at the age of 21 electrodes were implanted into basal ganglia which extremely improved his dystonic movements, posture and gait. After the surgery, during these eight years, the patient was regularly controlled, electrodes were reprogramed with continuous physical and speech therapy. Twice he presented with returned complete clinical presentation due to the empty battery of the neurostimulator that was replaced first with a non-rechargeable and then with a rechargeable battery leading to complete improvement. Conclusion: Idiopathic dystonia is often unrecognized. Although, DBS is a safe and effective method, it is not used enough for treating medication-refractory dystonia. Our case has shown persistent and excellent effect of DBS on generalized dystonia and patients’ quality of life over a long period of time

SMJERNICE ZA OBRADU TRAUMATSKIH OZLJEDA GLAVE U ODRASLOJ POPULACIJI U HITNOJ SLUŽBI U KLINIČKOJ BOLNICI DUBRAVA

Head trauma is a common presentation in the Emergency Department (ED), ranging from skull fractures, minor traumatic brain injuries (TBIs) to severe TBIs in polytraumas. In moderate traumatic brain injuries, patient assessment and diagnostic work-ups can be ameliorated with the application of Clinical Decision Rules (CDRs) such as the Canadian CT Head Rule (CCHR) and the National Institute for Care and Excellence (NICE) guidelines. Optimal adherence to these CDRs greatly benefi ts patients, reduces waiting times, ED overcrowding, mortality and ED clinician pitfalls. The aim of this report is to provide the reader with a brief review of the CCHR and NICE guidelines, which are implemented in Dubrava University Hospital, with an overview as to how our ED collaborates with its neurosurgical team and other surgical specialists in situations of polytrauma and TBI patients, mainly focusing on TBI. In addition, we will introduce the Dubrava Model, one of the neurotrauma models implemented in fast treatment of TBIs in rural hospitals devoid of resident neurosurgeon.Trauma glave, sežući od prijeloma lubanje i manje traumatske ozljede mozga do teške traumatske ozljede mozga u politraumama, česta je prezentacija u hitnoj službi. Kod umjerenih/srednje teških traumatskih ozljeda mozga pristup bolesniku i dijagnostička obrada mogu se poboljšati primjenom kliničkih smjernica kao što su Canadian CT Head Rule (CCHR) i smjernice National Institute for Care and Excellence (NICE). Optimalno pridržavanje navedenih smjernica uvelike koristi bolesnicima, smanjuje vrijeme čekanja, prenapučenost hitne službe, smrtnost i pogreške liječnika u hitnoj službi. Cilj ovoga rada je pružiti čitatelju kratak pregled smjernica CCHR i NICE koje se primjenjuju u Kliničkoj bolnici Dubrava, s osvrtom na suradnju naše hitne službe i neurokirurškog tima te liječnika drugih kirurških grana u obradi politraumatiziranih bolesnika i bolesnika s traumatskim ozljedama mozga. Uz to, prikazujemo Model “Dubrava”, jedinstveni model pristupu neurotraumi koji se primjenjuje u brzom liječenju traumatskih ozljeda mozga u ruralnim bolnicama lišenim službujućeg neurokirurga

Intracranial mature teratoma in an adult patient: a case report

Introduction: Primary intracranial teratoma is a subtype of germ cell tumors, classified into three subtypes. They occur very rarely, with only several reported individual cases in adults. ----- Case Description: We present a patient with an intermittent headache in the right frontal region. Magnetic resonance imaging (MRI) revealed a right sided high frontal parasagittal mass that compressed the falx, the right lateral ventricle, as well as the brain parenchyma. Patient underwent surgical treatment. Histopathological analysis described mature teratoma. Four months after the surgical treatment there were no signs of residual intracranial mass or relapse. ----- Discussion: Primary intracranial teratoma in adults has a nonspecific clinical presentation. MRI reveals a solitary irregular mass with multilocularity and mixed signals derived from different tissues. The patients age, biochemical markers, and patohistological analysis are necessary to confirm the diagnosis. ----- Conclusion: Teratoma treatment strategy still remains controversial. It includes radical resection whenever possible. Since the residual portion of mature teratoma may contain part of immature or malignant tissue, tumor recurrence after surgical removal is possible. Also, new tumor mass could occur at other sites intracranial after the initial one was removed. Thus, although patients usually recover, they should be followed-up for a long period of time