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Benigni paroksizmalni tranzitorni tortikolis dojenčeta – prikaz 10 naših bolesnika

UVOD: Benigni paroksizmalni tortikolis (BPT) je poremećaj koji se manifestira ponavljajućim epizodama abnormalne rotacije i naginjanja glave na jednu stranu (tortikolis), nerijetko praćenu asimetričnim držanjem – savijanjem trupa prema istoj strani (tortipelvis), kontrakcijom muskulature stražnje strane vrata (retrocolis) ili generaliziranom hipotonijom. Atake uvijek počinju u dojenačkom periodu, traju nekoliko sati do nekoliko dana (i do dva tjedna). Ponavljaju se u tjednim ili mjesečnim razmacima. Prognoza BPT je dobra, napadaji spontano nestaju do školske dobi. ISPITANICI I METODE: U petnaestogodišnjem periodu (1993 – 2008.g.) u našim ustanovama dijagnosticirali smo i klinički pratili 10 djece s BPT (4 dječaka, 6 djevojčica). Većina djece bila je upućena na inicijalnu neuropedijatrijsku procjenu pod sumnjom na konvulzivne / epilepsijske napadaje: REZULTATI: Kliničko ispoljavanje BPT u naših bolesnika bilo je tipično, bez gubitka svijesti. U prvih bolesnika zbog heteroanamnestičkih podataka o atakama koje su za roditelje bile dramatične razmatrali smo i mogućnost epilepsijskih napadaja, a koje smo isključili u tijeku kliničkog praćenja. EEG nalazi u budnosti i spavanju u svih ispitanika bili su uredni ili pokazivali nespecifične promjene. Sva djeca imala su uredan psihomotorni razvoj. Srednja dob početka prvih ataka BPT bila je 5 mjeseci (raspon od 2 mjeseca do 14 mjeseci). Učestalost ataka kretala se od 1 atake do 41 , u različitim intervalima, a trajanje ataka od 1-2 minute do 7 dana. U jednog dječaka kojeg pratimo 5 godina i 7 mjeseci, atake BPT u drugoj ustanovi dijagnosticirane su kao epilepsijske – parcijalne kompleksne krize i liječen je Phemitonom. Dječak je sada u dobi 6 godina i 8 mjeseci, još uvijek ima rijetke atake tortikolisa u trajanju više sati koje su praćene intenzivnim glavoboljama uz povraćanje i opću prostriranost što upućuje na povezanost BPT i migrene. Isti bolesnik također potvrđuje pojavu migrene u više članova obitelji (majka dječaka od djetinjstva pati od učestalih migrena). ZAKLJUČAK: Dijagnosticiranje BPT kao paroksizmalnog neepilepsijskog (nekonvulzivnog) poremećaja u dojenčadi i male djece temelji se na kliničkoj slici napadaja (očuvana svijest, odsustvo toničkih i/ili toničko kloničkih grčeva ), duljini trajanja i do nekoliko dana (tjedana) te normalnog nalaza iktalnog i interiktalnog EEG-a. INTRODUCTION: Benign paroxysmal transitory torticolis (BPT) is a disorder which manifests with repetitive episodes of abnormal rotations and head leaning on one side (torticolis) often associated with asymetric posturing – body bending towards the same side (tortipelvis), contraction of muscles on the back side of neck (retrocolis) or generalized hypotonia. Attacks always begin in infant period and last for several hours or days (and up to two weeks). They are repeated themselves in weekly or monthly intervals. Prognosis of BPT is favourable and attacks spontaneously disappear till the school age. METHODS: During the period of fifteen years (1993 – 2008.g.) in our clinics we diagnosed and surveyed clinically 10 children with BPT ( 4 boys, 6 girls). Most children were referred to the initial neuropediatric evaluation under the suspicion of convulsive/epileptic seizures. RESULTS: Clinical expression of BPT in our patients was typical, without loss of consciousness. In the first patients we have considered the possibility of epileptic seizures because of heteroanamnestic data from the parents about the dramatic attacks, and we excluded it during the clinical follow-up. EEG findings during wake and sleep were normal in all children showing only some non-specific changes. All children had normal psychomotor development. Average age at the time of the beginning of first BPT attacks was 5 months (range from 2 month to 14 month ). Frequency of attacks was from 1 to 41 in different intervals and duration of attacks from 1-2 minutes up to 7 day. In one boy which we are following 5 years and 7 months, BPT attacks were diagnosed in another hospital as epileptic – partial complex seizures and he was treated with phenobarbital (Phemiton). The boy is now at the age of 6 years and 8 months , he still suffers from rare attacks of torticolis in duration of couple hours which are accompanied by intensive headaches with vomiting and general prostration that points to the relationship between BPT and migrene. The same patient also confirms the existence of migrene in several relatives (boy's mother suffers from the childhood from frequent migrene). CONCLUSION: Diagnosing of BPT as the paroxysmal non-epileptic (nonconvulsive) disorder in infants and children is based on clinical features of attack (preserved consciousness, lack of tonic or tonic/clonic seizures), duration of attacks up to several days (weeks) and normal finding of ictal and interictal EEG.

benigni paroksizmalni tranzitorni tortikolis ; dojenče

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