36 research outputs found
Cerebellar cognitive affective syndrome CCAS : a case report
The aim of the study was to describe a case of the patient with cerebellar cognitive affective syndrome
CCAS, characterize the role of cerebellum in the regulation of cognitive functions and present the
procedure of neuropsychological diagnosis useful in indicating the specific cognitive and emotional problems
in patients with cerebellar damage.
Case report. A 41- year old man with an ischemic cerebellar stroke of its right hemisphere manifested
the neuropsychological symptoms typical for the frontal damage: euphoric mood, disorganized behavior,
lack of criticism and mental plasticity, tendency to shorten the personal distance, problems with mistake
correction. In neuropsychological diagnosis we used following methods: Raven Progressive Matrices
Test, Mini Mental Stage Examination (MMSE), Trail Making Test, Wisconsin Card Sorting Test, Stroop
Interference Test, Word Fluency Test, Auditory Verbal Learning Test by 艁uria, Benton Visual Retention
Test, Digit Span.
Results. Analyzing the obtained results we observed the significant decrease of all executive functions:
planning, abstract thinking, cognitive flexibility, adaptation to new situations as well as memory impairments
and changes in emotional and behavioral state similar to frontal syndrome. The whole of impairments
including the typical cerebellar symptoms (ataxia, dysarthria, dysmetria,hypotonia) create the cerebellar
cognitive affective syndrome CCAS with leading role of dysexecutive syndrome.
Conclusions. The cerebellum takes part in the regulation of cognitive functions. The cerebellar damages
can imitate the emotional- cognitive problems of patients after frontal damages what additionally stress
the functional link between these two brain structures. Patient鈥檚 with cerebellar damages should have neuropsychological
and neuropsychiatric diagnosis and care
Schizencephaly鈥攄iagnostics and clinical dilemmas
Background Schizencephaly is an uncommon congenital disorder of cerebral cortical development. The defect is characterized by the presence of a cleft in the brain extending from the surface of the pia mater to the cerebral ventricles. The margins of the cleft are lined with heterotropic, dysplastic gray matter. The causes of schizencephaly are heterogeneous and can include teratogens, prenatal infection, maternal trauma, or EMX2 mutations. Method In the present paper, the authors described difficulties in employing diagnostic imaging in differentiating between type II (open-lip) schizencephaly and much more common intracranial fluid spaces of a different origin (arachnoid cysts and hydrocephalus). Result In all the three cases, the treatment consisted in implantation of a shunt system; nevertheless, it should be emphasized that a surgical intervention in the third presented case (type II schizencephaly) aimed at relieving the symptoms of intracranial hypertension鈥攁 directly life-threatening condition鈥攕ince shunting is not a method of treating schizencephaly itself. Conclusions Although proper interpretation of the character of intracranial fluid spaces is of significance for further therapeutic management, yet, the key decision as to the surgical intervention is made based on clinical presentation, predominantly on symptoms of intracranial hypertension
Multiple Autologous Bone Marrow-Derived CD271+ Mesenchymal Stem Cell Transplantation Overcomes Drug-Resistant Epilepsy in Children
There is a need among patients suffering from drug鈥恟esistant epilepsy (DRE) for more efficient and less toxic treatments. The objective of the present study was to assess the safety, feasibility, and potential efficacy of autologous bone marrow cell transplantation in pediatric patients with DRE. Two females and two males (11 months to 6 years) were enrolled and underwent a combined therapy consisting of autologous bone marrow nucleated cells (BMNCs) transplantation (intrathecal: 0.5 脳 109; intravenous: 0.38 脳 109-1.72 脳 109) followed by four rounds of intrathecal bone marrow mesenchymal stem cells (BMMSCs) transplantation (18.5 脳 106-40 脳 106) every 3 months. The BMMSCs used were a unique population derived from CD271鈥恜ositive cells. The neurological evaluation included magnetic resonance imaging, electroencephalography (EEG), and cognitive development assessment. The characteristics of BMMSCs were evaluated. Four intravenous and 20 intrathecal transplantations into the cerebrospinal fluid were performed. There were no adverse events, and the therapy was safe and feasible over 2 years of follow鈥恥p. The therapy resulted in neurological and cognitive improvement in all patients, including a reduction in the number of epileptic seizures (from 10 per day to 1 per week) and an absence of status epilepticus episodes (from 4 per week to 0 per week). The number of discharges on the EEG evaluation was decreased, and cognitive improvement was noted with respect to reactions to light and sound, emotions, and motor function. An analysis of the BMMSCs' characteristics revealed the expression of neurotrophic, proangiogenic, and tissue remodeling factors, and the immunomodulatory potential. Our results demonstrate the safety and feasibility of BMNCs and BMMSCs transplantations and the considerable neurological and cognitive improvement in children with DRE. Stem Cells Translational Medicine 2018;7:20-3
Spinal meningiomas in pediatric patients - A case series and literature review
BACKGROUND: Meningiomas are the most frequent intracranial tumors in the adult population; however, they are rare in pediatric patients. In children, meningiomas often require further diagnosis of genetic comorbidities. As many as, 50% of young patients with meningiomas suffer from neurofibromatosis type 2 (NF2). Spinal meningiomas include only 10% of pediatric meningiomas. CASE DESCRIPTION: Between 2000 and 2017, three children were hospitalized in the Neurosurgery Department. The patients reported prolonged periods of increasing neurological symptoms. In each case, a total gross tumor resection was performed. Histopathology result in each patient was meningioma psammomatosum. Only one girl required adjuvant radiotherapy (RTH) due to recurrent tumors. Magnetic resonance imaging (MRI) showed spinal nerves schwannomas and bilateral vestibular schwannomas in two patients with NF2. CONCLUSION: A slow tumor growth is characteristic of spinal meningiomas. Back pain is a frequent initial symptom of a slowly growing tumor mass. Subsequently, neurological deficits gradually increase. Patients require a long follow-up period and control MRI-scan. Children with diagnosed spinal meningioma should be strictly controlled because of the high risk of their developing other tumors associated with NF2. Surgical resection is the primary treatment modality of meningiomas. Adjuvant RTH should be recommended only for selected patients
Neuropsychological and psychological interventions in patients with cerebellar mutism syndrome
Zesp贸艂 tylnojamowy (Posterior Fossa Syndrome, PFS) dotyka pacjent贸w po zabiegach neurochirurgicznych w obr臋bie tylnego do艂u czaszki. Osiowymi objawami PFS s膮 mutyzm m贸偶d偶kowy (Cerebellar Mutism Syndrome), zaburzenia motoryczne i poznawczo-afektywne. Pacjenci dotkni臋ci PSF cz臋sto trac膮 mo偶liwo艣膰 komunikacji, tak偶e niewerbalnej, przy wzgl臋dnie zachowanej 艣wiadomo艣ci. Uwa偶a si臋, 偶e poprawa nast臋puje spontanicznie w okresie do 6 miesi臋cy. Cz臋艣ciowe deficyty w zakresie funkcji j臋zykowych, ruchowych i poznawczo-afektywnych utrzymuj膮 si臋 cz臋sto wiele lat. Prawid艂owe post臋powanie mo偶e skr贸ci膰 czas ostrej fazy objaw贸w i poprawi膰 dalsze funkcjonowanie pacjenta. Nie ma udowodnionej skuteczno艣ci leczenia farmakologicznego w przypadku CMS, a kluczowe znaczenie ma terapia neuropsychologiczna i psychologiczna. Podejmowane wobec pacjenta dzia艂ania powinny mie膰 na celu w pierwszej kolejno艣ci odbudow臋 komunikacji i uwzgl臋dnia膰 wiek dziecka i jego ewentualne deficyty neurologiczne.Posterior fossa syndrome (PFS) is a collection of neurological symptoms that occurs following a surgical resection of a posterior fossa tumour. It is characterised by either a reduction or an absence of speech (Cerebellar Mutism Syndrome), motor deficits as well as cognitive and affective symptoms. Patients affected by PSF often lose the ability to communicate, including non-verbal communication, staying at least partially aware. It is believed that the improvement occurs spontaneously over the period of up to 6 months. However, partial deficits in language, motor control and cognitive-affective functions may be chronic. A proper treatment scheme may shorten the time of the acute phase of symptoms and improve the patient鈥檚 further functioning. The effectiveness of pharmacological treatment for CMS is limited. The neuropsychological and psychological therapy can contribute to the recovery of these patients. The main aim of the therapy should always be to rebuild the communication skills, taking into consideration the child鈥檚 age and his/her possible neurological deficits