Abstract
The current study was designed to investigate co-occurrence of absolute/relative microcephaly, absolute/relative macrocephaly and congenital nervous system disorders or neurological syndromes with symptoms visible since infancy, based on fundamental data acquired during the admission procedure at a neurological rehabilitation ward for children and adolescents. The study applied a retrospective analysis of data collected during the hospitalization of 327 children and adolescents, aged 4–18 years, affected since infancy by congenital disorders of the nervous system and/or neurological syndromes associated with a minimum of one neurodysfunction. To identify subjects with absolute/relative microcephaly, absolute/relative macrocephaly in the group of children and adolescents, the adopted criteria took into account z-score values for head circumference (z-score hc) and head circumference index (z-score HCI). Dysmorphological (x+/−3s) and traditional (x+/−2s) criteria were adopted to diagnose developmental disorders of head size. Regardless of the adopted criteria, absolute macrocephaly often coexists with state after surgery of lumbar myelomeningocele and hydrocephalus, isolated hydrocephalus, hereditary motor and sensory polyneuropathy, and Becker’s muscular dystrophy (p < 0.001, p = 0.002). Absolute macrocephaly is often associated with neural tube defects and neuromuscular disorders (p = 0.001, p = 0.001). Relative microcephaly often occurs with non-progressive encephalopathy (p = 0.017, p = 0.029). Absolute microcephaly, diagnosed on the basis of traditional criteria, is often associated with epilepsy (p = 0.043). In children and adolescents with congenital nervous system disorders or neurological syndromes with one or more neurodysfunction visible since infancy, there is variation in abnormal head size (statistically significant relationships and clinical implications were established). The definitions used allowed for the differentiation of abnormal head size.
Highlights
And macrocephaly are developmental disorders of the head (DDH) and are treated as clinical symptoms
The aim of the study is seeking the relationship between relative and absolute microcephaly, relative and absolute macrocephaly and units or disease syndromes with neurodysfunction in a group of children, based on data collected during the admission procedure to the Department of Children and Youth Neurological Rehabilitation and establishing clinical implications to facilitate the diagnostic-differential process
The abnormal head size in this group of children is most often represented by relative microcephaly
Summary
And macrocephaly are developmental disorders of the head (DDH) and are treated as clinical symptoms. The head circumference index (HCI) and Michalski’s classification can be used to assess the size of the head. Children and adolescents from the city of Rzeszow (Poland) have differentiated head sizes in different age groups. The sizes of heads in boys and girls of different age groups were classified with consideration of parameters such as hc and body height (h), the correlation between them, and Michalski’s classification. In boys aged 4–11, head size was assessed as very large, in boys aged 12–13—large, in boys aged 14—medium, and in boys aged 15–18—small. From a clinical point of view, this type of head size classification reflects the process of differentiating body proportions in children and adolescents, but still remains unsatisfactory. It is important that hc and h are measurements determined by the size of the skeleton, while body weight does reflect the size of the skeleton, and is determined to a lesser extent genetically than h [5]
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