title: [Analysis of changes in peripheral and central nervous system in irregularly treated adult patients with primary congenital hypothyroidism].
abstract: Lack of thyroid hormones in the womb and the first years of life causes changes in the nervous system and mental retardation. The aim of the study was to assess changes in peripheral and central nervous system in 29 adult patients with primary congenital hypothyroidism (PCH) depending on the cause of the disease and systematic treatment of L-thyroxine.
  
  
  METHODS
  The analysis was performed in 29 adult patients with PCH (16 women, 13 men) on the basis of the results of neurological examination, EEG, SPECT (Computer tomography single photon emission) of the brain.
  
  
  RESULTS
  Changes in the nervous system were found in 72% of respondents. Patients who had implemented replacement therapy L-thyroxine after completing 12 months of age showed the most neurological disorders. There were variations in the cranial nerves III, IX, IV and VI. In 34% of respondents revealed paraneoplastic cerebellar symptoms, while the pyramid, and extrapyramidal symptoms in 10% and 3% of the people. EEG showed changes in brain bioelectrical activity in the entire study group. In the 83% found a significant asymmetry in regional cerebral blood flow (rCBF). Hypoperfusion outbreak occurred mainly in the stands and leading occipital. The relationship between time of initiation of treatment, and the presence of a systematic change in the nervous system was inversely proportional. In turn, analyzing the causes of most PCH deviations were found in the nervous system in patients with athyreosis. Brain SPECT study in these patients confirmed the organic changes in brain development. CONCLUSIONS. The presence and extent of changes in peripheral and central nervous system depends on the cause PCH, pending the implementation of L-thyroxine treatment and systematic. Studies of brain SPECT and EEG confirmed the existence of developmental changes of the brain in patients with PCH.
authors.affiliation: Uniwersytet Medyczny w Poznaniu, Katedra Endokrynologii, Przemiany Materii i Chorób Wewnetrznych, Poznań. K_Lacka@wp.pl
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Łacka
authors.forename: Jolanta
authors.initials: J
authors.lastname: Florczak
authors.forename: Ilona
authors.initials: I
authors.lastname: Gradecka-Kubik
authors.forename: Justyna
authors.initials: J
authors.lastname: Rajewska
authors.forename: Roman
authors.initials: R
authors.lastname: Junik
mesh_terms: D000293:Adolescent; D000328:Adult; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000209:etiology*; D003409:Congenital Hypothyroidism / Q000150:complications* / Q000188:drug therapy*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis / Q000209:etiology*; D013974:Thyroxine / Q000627:therapeutic use; D015899:Tomography, Emission-Computed, Single-Photon; D055815:Young Adult
chemical_list: D013974:Thyroxine