Stimulation of Functional Vision in Children with Perinatal Brain Damage

Cerebral visual impairment (CVI) is one of the most common causes of bilateral visual loss, which frequently occurs due to perinatal brain injury. Vision in early life has great impact on acquisition of basic comprehensions which are fundamental for further development. Therefore, early detection of visual problems and early intervention is necessary. The aim of the present study is to determine specific visual functioning of children with perinatal brain damage and the influence of visual stimulation on development of functional vision at early age of life. We initially assessed 30 children with perinatal brain damage up to 3 years of age, who were reffered to our pediatric low vision cabinet in »Little house« from child neurologists, ophthalmologists Type and degree of visual impairment was determined according to functional vision assessment of each child. On the bases of those assessments different kind of visual stimulations were carried out with children who have been identified to have a certain visual impairment. Through visual stimulation program some of the children were stimulated with light stimulus, some with different materials under the ultraviolet (UV) light, and some with bright color and high contrast materials. Children were also involved in program of early stimulation of overall sensory motor development. Goals and methods of therapy were determined individually, based on observation of child’s possibilities and need. After one year of program, reassessment was done. Results for visual functions and functional vision were compared to evaluate the improvement of the vision development. These results have shown that there was significant improvement in functional vision, especially in visual attention and visual communication

Effects of Vibrotactile Stimulation on the Control of Muscle Tone and Movement Facilitation in Children with Cerebral Injury

Afferent signals from the muscle’s proprioceptors play important role in the control of muscle tone and in the facilitation of movements. Peripheral afferent pathway enables the restoration of connections with supraspinal structures and so includes mechanism of synaptic inhibition in the performance of normal movement. Different sensory stimuli, as vibrotactile stimulation, excite muscle’s proprioceptors which then send sensorimotor information via spinal cord. In this way afferent signals promote cortical control and modulation of movements. The goal of this study is to evaluate the effects of vibrotactile stimulation on the spasticity and motor performance in children with cerebral injury. Subjects included in this study were 13 children who were developing the classification of spastic cerebral palsy. For all children perinatal brain damage was documented by medical reports and neonatal brain ultrasound scan. At the mean age of 3 years and 6 months subject underwent the assessment of motor development by Gross Motor Function Measurement (GMFM-88). Gross Motor Classification System (GMFCS) has been used to classify functions of lower extremities. Therapeutic intervention was conducted once a week during 3 months. All subjects were stimulated with vibrotactile stimuli of 40Hz in duration of 20 minutes in order to reduce spasticity. After the ending of the treatment subjects underwent second assessment of motor performance and the classification of lower extremities functions. The results have shown that there was a significant improvement in motor performance, what has been seen in the facilitation of rotations, better postural trunk stability and head control and in greater selectivity of movements. Further randomized, control trial investigations with bigger sample and included spasm scale are needed to gain better insight in the role of vibrotactile stimulation in the facilitation of normal movements

Prognostička vrijednost intrakranijskog ultrazvuka u usporedbi s magnetskom rezonancijom mozga u djece s cerebralnom paralizom: populacijska studija

The aim of this population-based study was to evaluate the characteristics of cerebral palsy (CP) in relation to the predominant pattern of the Magnetic Resonance Imaging Classification System (MRI CS) that was analogously applied to the neonatal/early infant cranial ultrasound (CUS). The study included children born during the 2004-2007 period from the Croatian part (C28 RCP-HR ) of the Surveillance of Cerebral Palsy in Europe (SCPE) CP register. Motor functions, accompanying impairments and brain MRI were evaluated in 227 children, 185 of which also had CUS. Concerning CP types, 56% of children had bilateral spastic, 34% unilateral spastic, 9% dyskinetic and 1% ataxic CP type. Gross Motor Function Classification System (GMFCS) revealed that 62.05% had mild (GMFCS I-III ) and 37.85% had severe motor impairment (GMFCS IV-V). CUS showed white matter injury in 60%, gray matter injury in 12%, maldevelopments in 8%, miscellaneous changes in 14%, while 6% were normal; MRI showed significant agreement (κ=0.675, p<0.001). Neuroimaging findings of maldevelopments and predominant gray matter injury were associated with more severe CP, but 7% of children with CP had normal MRI . As we found very good agreement between CUS and MRI findings, CUS is recommended in children at an increased risk of CP if MRI is not available.Cilj ove populacijske studije bio je procijeniti karakteristike cerebralne paralize (CP) u odnosu na predominantni uzorak na magnetskoj rezonanciji mozga prema klasifikacijskom sustavu Magnetic Resonance Imaging Classification System (MRI CS) koji je analogno primijenjen i na novorođenački/rani dojenački intrakranijski ultrazvuk (UZV). Istraživanje je uključivalo djecu rođenu od 2004. do 2007. godine iz hrvatskog dijela (C28 RCP-HR ) Registra Europskog projekta nadzora cerebralne paralize (Surveillance of Cerebral Palsy in Europe, SCPE). Ispitivane su grube i fine motoričke funkcije, pridružena odstupanja i slikovni prikazi mozga u 227 djece s MRI mozga od kojih je 185 imalo i neonatalni/rani dojenački UZV. U odnosu na tip CP, 56% djece imalo je bilateralno spastičnu, 34% jednostrano spastičnu, 9% diskinetsku i 1% ataktičku CP. Prema funkcionalnoj klasifikaciji grubih motoričkih funkcija Gross Motor Function Classification System (GMFCS), 62,05% djece imalo je blaže motoričko oštećenje (GMFCS I-III ), a 37,85% teško motoričko oštećenje (GMFCS IV-V). Intrakranijski UZV pokazao je oštećenje bijele tvari mozga u 60%, ozljedu sive tvari u 12%, poremećaj razvoja mozga u 8%, razne promjene u 14% ispitanika, dok ih je 6% imalo normalan nalaz; MRI je pokazala značajnu podudarnost (κ=0,675, p<0,001). Poremećaji razvoja mozga i predominantne ozljede sive tvari mozga bile su povezane s težim stupnjem CP, no 7% djece s CP imalo je normalnu MRI mozga. Zbog vrlo dobre podudarnosti UZV i MRI nalaza u našem istraživanju UZV se preporučuje kod djece s povećanim rizikom od CP ako MRI nije dostupna

Funkcionalna klasifikacija djece s cerebralnom paralizom u Krapinsko-zagorskoj županiji

The aim was to study functional abilities and to create functional classification of children with cerebral palsy (CP) in Krapina-Zagorje County, based on the classification of gross and fine motor skills and associated impairments. Classification was performed according to the SCPE (Surveillance of Cerebral Palsy in Europe) criteria. We used standardized and complementary functional classification systems for cerebral palsy to create a functional profile. Research included 44 children with CP in the age range of 4 to 18 years. The results showed that the majority of children had bilateral spastic CP (63.6%), followed by unilateral spastic (22.7%) while the representation of dyskinetic CP was 9.09% and ataxic CP 4.55%. Based on the classification of gross and fine motor skills, 43.2% of children had the ability to walk, 11% of children could walk with assistive mobility devices, while 45.4% of children had a low functional level. The study also analyzed the associated impairments where higher classification score of motor impairment correlated with the severity of impairment. The results showed that children with dyskinetic CP and severe motor impairment could have mild cognitive impairment. We systematically present the neuropsychological and functional profile according to the CP type.Cilj je bio istražiti funkcionalnu klasifikaciju djece s cerebralnom paralizom (CP) u Krapinsko-zagorskoj županiji na osnovi klasifikacije grubih i finih motoričkih funkcija te pridruženih odstupanja. Klasifikacija je provedena sukladno preporukama europskog projekta pod naslovom Surveillance of Cerebral Palsy in Europe. Primijenjeni su standardizirani i međusobno kompatibilni klasifikacijski sustavi s ciljem formiranja funkcionalnog profila. U istraživanju je sudjelovalo 44 djece s CP u dobi od 4-18 godina. Rezultati su pokazali da je najučestaliji tip CP obostrani spastični (63,6%), a potom jednostrani spastični tip (22,7%). Zastupljenost diskinetske CP u našem istraživanju bila je 9,09%, a ataktične CP 4,55%. Sukladno klasifikaciji grubih i finih motoričkih funkcija 43,2% djece je bilo pokretno, uz pomoć pomagala se moglo kretati 11% djece, dok je nisku funkcionalnu razinu imalo 45,4% djece. U studiji su također analizirana i pridružena odstupanja, s tim da su viši stupnjevi klasifikacije motoričkih funkcija korelirali s težinom oštećenja. Rezultati su ukazali na to da djeca s diskinetskim tipom CP i teškim motoričkim oštećenjem mogu imati blaže kognitivno oštećenje. Istraživanjem je sustavno prikazan neuropsihološki profil i karakteristike pridruženih odstupanja u odnosu na tip CP te je formiran funkcionalni profil za svakog bolesnika

Infantile Spasms in Children with Down Syndrome

Down syndrome (DS) is the most common genetic cause of mental retardation. It is estimated that 5–13% of persons affected by DS have seizures. Infantile spasms are the most common type of seizures and usually are well controlled with steroids and antiepileptic drugs.We present 11 children at the age of 3 years and 4 months to 10 years and 7 months with DS and infantile spasms, treated at Children’s Hospital Zagreb from January 2000 until July 2009. Infantile spasms began at the age of 5 to 10.5 months in 10 children, in one child at the age of 16 months. Only one child had perinatal risk factors for the development of IS. Changes in EEG correlated to hypsarrhythmia. Infantile spasms were treated initially with antiepileptic drugs, most often with valproic acid. Treatment was inefficient in 10/11 patients. After application of ACTH, infantile spasms stopped between 7 and 15 days in 6 patients, until 28th day in 4 patients. Hypsarrhythmia vanished in all children. During follow-up period (2 years and 7 months to 9 years and 5 months) none of the children developed another type of seizures. No major epileptogenic changes were registered in EEG. Antiepileptic therapy was discontinued in 4 children (aged 4 years and 2 months to 5 years). In this group is the boy who died of heart failure. Infantile spasms associated with DS are categorized into symptomatic group. The existence of cerebral pathology and delayed psycho-motor development precedes occurrence of seizures. It is possible to achieve good control of seizures and disappearance of hypsarrhythmia with application of ACTH and antiepileptic drugs

Lenticulostriatal Vasculopathy – a Marker for Congenital Cytomegalovirus Infection?

Lenticulostriate vasculopathy (LSV) is an ultrasound (US) visible lesion of the brain, which appears as echogenic streaks or spots in the arteries of thalamus and basal ganglia. LSV has varied etiology. Transfontanelar Color Doppler (TFCD) can easily display lenticulostriatal blood flow and assess: stage I LSV with present flow within echogenic changes and stage II LSV in which the flow disappears, despite a presence of streaks and spots, which at this stage most probably correspond to calcification. The objectives of this study are to determine: (1) Whether there are differences in distribution (unilateral or bilateral) and presence (during first year of age) of TFCD flow between congenital CMV infection positive and negative group of children with LSV; (2) Could US and TFCD findings of LSV be an indication for further investigation of possible congenital CMV infection, because of their variable and often adverse neurodevelopmental outcome? We examined and followed-up 98 infants with LSV. One group (37/98) with congenital CMV infection and second (61/98) negative. All infants had clinical signs of neuromotor delay and ultrasound and TFCD markers of LSV. Our study shows that most of the patients from both groups had TFCD visible flow at the age of 0–4 months. In majority of them in both groups, at the age of 5–8 months, there was no more visible flow. TFCD showed no statistically significant difference among congenital CMV infection positive group and negative group, nor in youngest age period (0–4 months), nor in later course of flow in LSV, unilaterally or bilaterally. Although the LSV presents nonspecific marker for intracranial infection (ICI), all infants presenting with LSV should be evaluated for possible ICI. Thus, the Doppler findings of LSV in infants require a detailed examination, monitoring and follow-up of neuromotor outcome

LENTICULOSTRIATAL VASCULOPATHY (LSV) – A MARKER FOR CONGENITAL CMV INFECTION?

Lentikulostrijatusna vaskulopatija (LSV) je ultrazvukom (UZV) vidljiva lezija mozga, koja se manifestira kao linearna ili točkasta hiperehogenost u području bazalnih ganglija i talamusa. Registrirana je kod 0,27-2,5% živorođene novorođenčadi, 5,1-32% nedonoščadi i 1,9-5,8% bolesne novorođenčadi, najčešće s konatalnom citomegalovirusnom (CMV) infekcijom. CMV infekcija najčešća je virusna bolest koja se prenosi “in utero” i napada središnji živčani sustav. CMV uništava stanice i oštećuje endotel korionskih resica i fetalnih krvnih žila, najčešće lentikulostrijatusnih arterija, uzrokujući lentikulostrijatusnu vaskulopatiju, koja se dobro registrira ultrazvukom i transkranijskim obojenim doplerom. Doplerom se potvrđuje vaskularna priroda tih lezija, tj. pulzirajući arterijski protok unutar njih. Neuropatološko objašnjenje za LSV još je nejasno. Može odgovarati mineralizaciji - depozitu kalcija i željeza, hipercelularnosti zidova arterija, što je prisutno i u drugim infekcijama: sifilis, toksoplazmoza ili HIV. Slične promjene nađene su i kod djece s kromosomskim aberacijama (trisomijom 13, mozaicizmom 46XX/47XXX, nebalansiranom translokacijom kromosoma 11), fetalnog alkoholnog sindroma, oštećenja lijekovima, kod sialidoze, kongenitalnih srčanih grješaka, neonatalne asfiksije i drugih neklasificiranih dizmorfičnih sindroma. LSV može biti jednostran ili obostran, izoliran ili pridružen. Pridruženi LSV manifestira se u tri oblika: blagi, umjereni i teški. U blagom obliku LSV-a, uz hiperehogenosti u BG-u prisutne su i subependimalne ciste, intraventrikulsko krvarenje II. stupnja i periventrikulska leukomalacija I. stupnja.. Kod umjerenog oblika LSV-a u mozgu je prisutna IVH gr II./III. i PVL II., dok u teškom obliku LSV-a postoji difuzna atrofija mozga i/ili cistični PVL. U ranom stadiju, tijekom prvih 6 mjeseci života, TCCD registrira protok u hiperehogenostima, dok se nakon tog vremena, u II. stadiju, protok više ne registrira. Ovim radom prikazali smo UZV i doplerske nalaze LSV-a kod neurorizične dojenčadi pregledane u Klinici za dječje bolesti Zagreb, radi utvrđivanja etiologije LSV-a, stupnjevanja i podjele lezije, te neurorazvojnog ishoda te djece, s posebnim osvrtom na udjel djece s konatalnom CMV infekcijom.Lenticulostriatal vasculopathy (LSV) is an ultrasonographic (US) brain lesion which appears as branched candlestick - shaped echogenicities or hyperechogenic punctuations located within the basal ganglia (BG) and the thalamus. It has been reported in 0.27-2.5% live neonates, 5.1-32% preterm infants and 1.9-5.,8% ill neonates, the most often in those with congenital cytomegalovirus (CMV) infection. Congenital CMV infection is an important cause of morbidity and mortality in neonates and infants. Brain ultrasonography (US) showed: abnormal echogenicity of the parenchyma, echogenic sulci and gyri, ventricular dilatation, complications and sequellae of the infection (ventriculitis, abscesses, hydrocephalus and calcifications), disturbances of neuronal migration, cerebellar hypoplasia, delays in myelination, SE cysts and LSV. LSV may be caused by a variety of conditions including infection, haemorrhage, hypoxic-ischemic insult, hypoglycaemia, infarction, calcifications, vascular lesions, metabolic disorders or chromosomal abnormalities. Transfontanellar Color Duplex Doppler (TFCD) imaging confirms the vascular origin of these lesions with pulsatile flows as an arterial spectrum. It revealed in the first months of life, but after that time TFCD showed no more flow within the hyperechogenicities, suggesting true calcifications. LSV can be graduated as unilateral or bilateral, isolated or with coexistent abnormalities, grading to minor, moderate and major LSV. The aim of this work is to present TFCD findings in neonates and infants with risk for development of neuromotor disabilities. All infants have US findings of LSV in BG and thalamus. TFCD showed a flow inside during the first few months of life, but with no flow within the echogenicities at the end of the first year. We would like to perform an etiology and graduation of LSV with implications for neurodevelopmental outcome, especially in infants with congenital CMV infection