Stanisław Kwiatkowski

Continuous Improvement after Multiple Mesenchymal Stem Cell Transplantations in a Patient with Complete Spinal Cord Injury

Interruption of spinal cord (SC) continuity leads to functional loss below the lesion level. The purpose of this study was to evaluate the safety and efficacy of bone marrow nucleated cell (BMNC) and multiple mesenchymal stem cell (MSC) transplantations in spinal cord injury (SCI). A patient with total SC interruption at the Th2-3 level underwent experimental therapy with BMNC and MSC transplantations followed with intensive neurorehabilitation treatment. At admission, 6 h after SCI, the patient was scored ASIA A, had a Th1 sensation level, paraplegia with sphincter palsy, and was without the ability to control trunk movement. Neurophysiology examination showed bilateral axonal damage to the motor and sensory neural fibers with no motor unit potentials or peripheral motor nerve conduction in the lower extremities. The standard therapy had been applied and had not produced any positive results. The patient was treated with autologous BMNCs injected intravenously (3.2 × 109) and intrathecally (0.5 × 109) 10 weeks after the SCI and with five rounds of MSCs every 3-4 months (1.3-3.65 × 107) administered via lumbar puncture. Total number of transplanted MSC cells during the course of treatment was 1.54 × 108. There were no complications related to transplantations and no side effects related to the therapy during 2 years of treatment. The ASIA score improved from A to C/D (from 112 to 231 points). The sensation level expanded from Th1 to L3-4, and the patients ability to control the body trunk was fully restored. Bladder filling sensation, bladder control, and anal sensation were also restored. Muscle strength in the left lower extremities improved from plegia to deep paresis (1 on the Lovett scale). The patients ability to move lower extremities against gravity supported by the movements in quadriceps was restored. The patient gained the ability to stand in a standing frame and was able to walk with the support of hip and knee ortheses. Magnetic resonance imaging (MRI) revealed that at the Th2/Th3 level, where the hemorrhagic necrosis was initially observed, small tissue structures appeared. Our results suggest that repeated intrathecal infusions of MSCs might have the potential to produce clinically meaningful improvements for SCI patients.

Preliminary Study of Autologous Bone Marrow Nucleated Cells Transplantation in Children With Spinal Cord Injury

The objective of this study was to assess the safety and efficacy of transplanting bone marrow nucleated cells (BMNCs) to treat children with complete interruption of spinal cord (SC) continuity. The present study was conducted from 2005 to 2011. The inclusion criteria were a magnetic resonance imaging‐confirmed complete interruption of SC continuity and no improvement in neurological status within 6 months after standard therapy. Bone marrow was isolated from the iliac ala and submitted to BMNC isolation. Subsequently, the cell suspension was administered into the SC cavity and intravenously. In total, 18 of 19 intraspinal and intravenous BMNC transplantation procedures performed caused no adverse events. One case was connected with transient bradycardia. The experimental therapy showed no late complications in the 1‐ to 6‐year follow‐up evaluation period. Neurological improvement was observed in two patients who received multiple implantations. One patient demonstrated improved superficial sensation from Th3 to Th12/L1 and a restored bladder‐filling sensation. In the other case, superficial sensation was improved from C2 to C5, and the respiratory drive, the swallowing reflex, and tongue movements were restored. Spasticity and quality of life were improved in three of five patients. In addition, skin pressure ulcers healed and did not recur. Our preliminary results demonstrate the safety and feasibility of BMNC transplantation in children with complete SC injury. The results indicate that a certain degree of neurological and quality‐of‐life improvement can be attained by children with chronic complete SC injury who receive multiple BMNC implantations.

Fetus in fetu: a medical curiosity—considerations based upon an intracranially located case

Introduction“Fetus in fetu” (FIF) is defined as the abnormal monozygotic twin inside the body of its “host twin.” Intracranial FIFs are extremely rare.Case presentationA male premature newborn was admitted to the hospital due to a large intracranial tumor diagnosed in the 31st week of gestation. The child died before surgical treatment because of failure of the respiratory system due to fetal respiratory distress syndrome. During general autopsy, a large intracranial tumor with four relatively well-developed limbs was found. Microscopically, apart from relatively well-formed musculoskeletal structures of limbs that were covered with skin, there were haphazardly distributed different tissues or fragments of organs. However, various neuroectodermal derivatives were dominant.ConclusionWe believe that intracranial FIFs, theoretically with poor prognosis, can be successfully curable in cases revealed prenatally, provided that optimal treatment is introduced and the achievement of proper pulmonary maturity of the host is accomplished prior to the operation of the tumor.

Schizencephaly—diagnostics and clinical dilemmas

BackgroundSchizencephaly 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.MethodIn 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).ResultIn 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.ConclusionsAlthough 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.

“Cerebellar cognitive affective syndrome CCAS – a case report”

Anna Starowicz-Filip1, Olga Milczarek2, Stanislaw Kwiatkowski2 , Barbara Betkowska-Korpala1, Katarzyna Prochwicz3: 1Medical Psychology Department, Psychiatry, Jagiellonian University Medical College; 2Neurosurgery Ward, Children’s University Hospital in Cracow; 3Institute of Psychology, Jagiellonian University. Correspondence address: starow@poczta.onet.pl Cerebellar cognitive affective syndrome CCAS – a case report

The visuospatial functions in children after cerebellar low‐grade astrocytoma surgery: A contribution to the pediatric neuropsychology of the cerebellum

The aim of this study was to specify whether cerebellar lesions cause visuospatial impairments in children. The study sample consisted of 40 children with low-grade cerebellar astrocytoma, who underwent surgical treatment and 40 healthy controls matched with regard to age and sex. Visuospatial abilities were tested using the spatial WISC-R subtests (Block Design and Object Assembly), Rey-Osterrieth Complex Figure, Benton Judgment of Line Orientation Test, PEBL Mental Rotation Task, and Benton Visual Retention Test. To exclude general diffuse intellectual dysfunction, the WISC-R Verbal Intelligence IQ, Performance IQ, and Full-Scale IQ scores were analysed. Post-surgical medical consequences were measured with the International Cooperative Ataxia Rating Scale. Compared to controls, the cerebellar group manifested problems with mental rotation of objects, visuospatial organization, planning, and spatial construction processes which could not be explained by medical complications or general intellectual retardation. The intensity of visuospatial syndrome highly depends on cerebellar lesion side. Patients with left-sided cerebellar lesions display more severe spatial problems than those with right-sided cerebellar lesions. In conclusion, focal cerebellar lesions in children affect their visuospatial ability. The impairments profile is characterized by deficits in complex spatial processes such as visuospatial organization and mental rotation, requiring reconstruction of visual stimuli using the imagination, while elementary sensory analysis and perception as well as spatial processes requiring direct manipulation of objects are relatively better preserved. This pattern is analogous to the one previously observed in adult population and appears to be typical for cerebellar pathology in general, regardless of age.

EPA-1136 – Cerebellar cognitive – affective syndrome ccas in children after posterior fossa tumor surgery

Introduction Cerebellum was typically associated with human motor functions. However, the past two decades of research have brought an increased number of reports indicating a markedly more extensive function of the cerebellum that also includes cognitive processes. In 1997 Schmahmann and Sherman described the complex neuropsychological problems manifested by the patients with cerebellar damages, which they termed as cerebellar cognitive-affective syndrome’ (CCAS). It consists of: executive dysfunctions, visual-spatial problems, mood disturbances and language impairments. This syndrome was observed mainly in adult patients. Aim The aim of the study was to describe the specificity of cerebellar cognitive affective syndrome in children with cerebellar damage. Material and method We examined 25 children after posterior fossa tumor surgery, mainly astrocytomas, treated at the Division of Pediatric Neurosurgery University Hospital in Cracow, Poland. The children were assessed by neuropsychological age-appropriate tests: Wisconsin Card Sorting Test, Word fluency, Rey-Osterrieth Complex Figure Test, Rey Auditory Verbal Learning Test, Digit Span WISC-R, Ravens Progressive Matrices Test, Benton Visual Retention Test. Results The results of our research reveal the cognitive and emotional problems of children with cerebellar damages. We observed the executive dysfunctions, language and memory impairments, visual-spatial problems and emotional difficulties. The type of the cognitive impairments was significantly connected with the location of the cerebellar lesion. Conclusions Cerebellum plays an important role in the cognitive and emotional development of the children.

MR CSF Flow Examination as a Non-Invasive Method of Determining Shunt-Independent Hydrocephalus in Children with Implanted Ventriculoperitoneal Shunt. Preliminary Results.

The study discusses the value of MR examination of CSF flow through the cranial part of the ventriculoperitoneal shunt in the evaluation of shunt-dependent hydrocephalus. Sixteen patients aged eight to 18 years were examined in the course of many years of treatment using the ventriculoperitoneal shunt. No clinical manifestations of shunt obstruction had been observed in any of the patients prior to MR examination. A Signa Horizon 1.5T (GEMS) unit was used. Axial and sagittal T1- and T2-weighted images were performed to find the ventriculoperitoneal shunt canal. A 2D CINE/GR/PC sequence was performed in the plane perpendicular to the shunt canal. CSF-flow was calculated by the Flow Analysis program. Patients were divided into two groups: 1) no CSF-flow was noted in five children indicating shunt-independent hydrocephalus; 2) CSF-flow was present (average flow volume from 0.1 to 1.9 ml/min) in 11 children, distinguishing the group with shunt-dependent hydrocephalus. Conclusions: 1) MR CSF flow examination will establish the absence or presence of CSF flow and determine its direction and velocity. 2) With no clinical manifestations of shunt impatency the absence of flow in the shunt canal found in MR CSF flow examination may indicate shunt-independent hydrocephalus. 3) When the CSF flow in the shunt is found in MR shunt-dependent hydrocephalus is diagnosed.

The Role of the Cerebellum in the Regulation of Language Functions

The present paper is a review of studies on the role of the cerebellum in the regulation of language functions. This brain structure until recently associated chiefly with motor skills, visual-motor coordination and balance, proves to be significant also for cognitive functioning. With regard to language functions, studies show that the cerebellum determines verbal fluency (both semantic and formal) expressive and receptive grammar processing, the ability to identify and correct language mistakes, and writing skills. Cerebellar damage is a possible cause of aphasia or the cerebellar mutism syndrome (CMS). Decreased cerebellocortical connectivity as well as anomalies in the structure of the cerebellum are emphasized in numerous developmental dyslexia theories. The cerebellum is characterized by linguistic lateralization. From the neuroanatomical perspective, its right hemisphere and dentate nucleus, having multiple cerebellocortical connections with the cerebral cortical language areas, are particularly important for language functions. Usually, language deficits developed as a result of a cerebellar damage have subclinical intensity and require applying sensitive neuropsychological diagnostic tools designed to assess higher verbal functions.

Cognitive disorders in a patient with an arachnoid cyst of the sylvian fissure and improvement after surgical treatment: Case description

ABSTRACT Arachnoid cysts cause a wide spectrum of clinical symptoms. Often it is difficult to clearly establish the relation between the presence of an arachnoid cyst and problems reported by the patient, which may, although not necessarily, be due to chronically elevated intracranial pressure. In recent years, increasing attention is paid to cognitive disorders accompanying arachnoid cysts, such as memory disorders, attention disorders, or language function disorders. This article presents a case of an 11-year-old patient, treated at the University Children’s Hospital in Cracow for an arachnoid cyst, located in the left middle cranial fossa. This analysis is focused on the description of cognitive and intellectual deficits of the child, as well as the discussion on their connection with the arachnoid cyst. Prior to the neurosurgical procedure, the boy presented significant deficits in cognitive functions, especially mnestic and attention functions and, also, a quite low level of intellectual functioning. Neuropsychological examination performed after the procedure indicated a significant improvement of cognitive functioning for all the functions, as well as a higher level of intellectual functioning. This analysis attempts to answer whether and to what extent the child still suffers from cognitive deficits from the organic lesions.