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 Łuria, 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’s with cerebellar damages should have neuropsychological and neuropsychiatric diagnosis and care

Schizencephaly—diagnostics 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—a directly life-threatening condition—since 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‐resistant 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 × 109; intravenous: 0.38 × 109-1.72 × 109) followed by four rounds of intrathecal bone marrow mesenchymal stem cells (BMMSCs) transplantation (18.5 × 106-40 × 106) every 3 months. The BMMSCs used were a unique population derived from CD271‐positive 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‐up. 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

Zespół tylnojamowy (Posterior Fossa Syndrome, PFS) dotyka pacjentów po zabiegach neurochirurgicznych w obrębie tylnego dołu czaszki. Osiowymi objawami PFS są mutyzm móżdżkowy (Cerebellar Mutism Syndrome), zaburzenia motoryczne i poznawczo-afektywne. Pacjenci dotknięci PSF często tracą możliwość komunikacji, także niewerbalnej, przy względnie zachowanej świadomości. Uważa się, że poprawa następuje spontanicznie w okresie do 6 miesięcy. Częściowe deficyty w zakresie funkcji językowych, ruchowych i poznawczo-afektywnych utrzymują się często wiele lat. Prawidłowe postępowanie może skrócić czas ostrej fazy objawów i poprawić dalsze funkcjonowanie pacjenta. Nie ma udowodnionej skuteczności leczenia farmakologicznego w przypadku CMS, a kluczowe znaczenie ma terapia neuropsychologiczna i psychologiczna. Podejmowane wobec pacjenta działania powinny mieć na celu w pierwszej kolejności odbudowę komunikacji i uwzględniać 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’s 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’s age and his/her possible neurological deficits