[Neurological picture and 1H MRS in 4 children with hypoparathyroidism].
Hypoparathyroidism can result from lack or decreased secretion of parathormon (PTH) or from defective responsiveness of effector organs to PTH. Neurological signs of hypoparathyroidism include symptomatic or latent tetany, seizures, in the case of basal ganglia and cerebellar calcifications also choreoatetosis, parkinsonism and cerebellar syndrome. Seizures may occur at any age and are usually generalized tonic-clonic ones. They may also precede other signs of impaired function of extrapyramidal system. The aim of the study was the assessment of neurological signs in children with hypoparathyroidism and also the evaluation of different neuroimaging procedures especially magnetic resonance spectroscopy. In the recent two years 4 children were diagnosed as suffering from hypoparathyroidism. The cause for admitting children were paroxysmal states. Because of drug-resistant epilepsy antiepileptic drugs have been administered in one child for 8 years. In one case during the neurological examination signs of cerebellar syndrome were observed. EEG showed paroxysmal changes in three cases. Performed computed tomography (CT) of the head revealed basal ganglia calcifications. In the child with ataxia magnetic resonance imaging (MRI) demonstrated small atrophic areas in the cerebellum. The neuroimaging diagnostics was supplemented by the 1H magnetic resonance spectroscopy (MRS). The spectra were recorded from the areas localized within subcortical nuclei as well as from frontal (white matter) and temporal lobes. In case of all the 1H MRS spectra obtained from the subcortical nuclei regions significant decrease in the NAA/tCr ratio was observed which could reflect the reduced concentration of N-acetyloaspartate, known as a neurons marker. All the metabolic profiles obtained from the spectra point to severe metabolic disturbances. The applied therapy (calcium, magnesium, Vit D3 preparations) allowed for seizures disapearance.
Paprocka
Justyna
J
Klinika Pediatrii i Neurologii, Wieku Rozwojowego SLAM, Katowice. neurdziec@slam.katowice.pl
Jamroz
Ewa
E
Wackermann-Ramos
Anna
A
Sokół
Maria
M
Marszał
Elzbieta
E
D000293:Adolescent; D001479:Basal Ganglia / Q000378:metabolism / Q000473:pathology*; D001480:Basal Ganglia Diseases / Q000175:diagnosis* / Q000209:etiology; D002114:Calcinosis / Q000175:diagnosis* / Q000209:etiology; D002648:Child; D005260:Female; D006801:Humans; D007011:Hypoparathyroidism / Q000150:complications* / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed