A Retrospective, Cross-Sectional Analysis of Motor Development, Cognition, and Mood in 87 Patients With Childhood-Onset Hereditary Spastic Paraplegias.
Pediatr Neurol
; 152: 93-97, 2024 Mar.
Article
en En
| MEDLINE
| ID: mdl-38242023
ABSTRACT
BACKGROUND:
HSP is a heterogeneous group of rare genetic diseases. In childhood, little is known of the development and psychological manifestations.METHODS:
Retrospective analysis of 87 patients with childhood-onset HSP. Patient consent was obtained and data regarding gross motor, fine motor, and language development; equipment usage; surgical procedures; cognition; and mood were collected at each clinic visit and by phone call and analyzed using mean, median, range, and interquartile ranges (IQRs).RESULTS:
The cohort contained 18 genetic types of HSP. Participant data ranged from birth to 36 years. Follow-up was variable spanning from a single clinic visit to 24 years of longitudinal visits. The mean age in months of sitting = 7.37, median = 6, range = 5 to 48, IQR = 0; crawling mean = 9.6, median = 9, range 7 to 23, IQR = 0; pulling to stand mean = 10.7, median 9, range 9 to 36, IQR = 0; and the age for walking was mean = 16.25, median = 15, range = 11 to 63 IQR = 6. Eighteen patients did not achieve independent ambulation. Twenty-five were noted to have initial gait abnormalities. Median age for first word spoken was 12 months. Fifty-five of 87 participants were enrolled in mainstream or honors classes. Twenty-two of 87 had attention deficit disorder. Patients reported experiencing sadness around their diagnoses, and 26 of 87 reported being diagnosed with anxiety or depression.CONCLUSIONS:
In childhood-onset HSP, motor disorder is the predominant feature; however, screening for attention deficit, anxiety, and depression is indicated.Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Paraplejía Espástica Hereditaria
/
Trastornos del Conocimiento
Tipo de estudio:
Prevalence_studies
Límite:
Humans
/
Infant
Idioma:
En
Revista:
Pediatr Neurol
Asunto de la revista:
NEUROLOGIA
/
PEDIATRIA
Año:
2024
Tipo del documento:
Article