Werdnig-Hoffmann: chimiro uye chikuru zviratidzo

Werdnig-Hoffmann - chaizvo zvokurwara chakakomba icho achiperekedzwa amyotrophy, kuparadzwa zvishoma nezvishoma huru etsinga fibers pamusoro musana, uye, naizvozvo, tsandanyama atrophy. Pakarepo zvinokosha kuziva kuti ichi ndicho chinhu nhaka kuvabereki chirwere, icho chinopfuudzwa ari autosomal recessive nenzira.

Chii Werdnig-Hoffmann?

Zviri chetsinga chirwere pamwe nokuparadzwa zvishoma nezvishoma pamusoro dzinokosha Zvivako tsinga innervating. Somuenzaniso, kazhinji pane demyelination pamusoro Anterior midzi musana. Mukuwedzera, chirwere chinobata zvimwe cranial tsinga.

Chokwadi, kukuvara etsinga fibers kunokanganisa mamiriro tsandanyama. Zvisinei, nokuti chirwere ichi kunoratidzwa vanonzi danda tsandanyama kurasikirwa, umo chikamu tsandanyama anoramba anokwanisa kubata, ukuwo vakaparadzana "mwanda" atrophy.

Spinal atrophy Verdniga-Hoffmann richiratidzwa pauduku. Nhasi rakakamurwa huru nhatu siyana chirwere.

Vachiberekwa chirwere Verdniga-Hoffmann yaro zviratidzo

Kazhinji, zviratidzo chimiro ichi chirwere rokutanga vatova kuoneka mumazuva mashoma okutanga pashure pokuberekwa. Mwana kupa usimbe paresis wamativi. Kuchema vana vanorwara vasina simba uye haawanzotauri anzwike, mukuwedzera, vane simba kwacho raputsika.

Mwana wako paanokura, unogona kuona lag mu kwomuviri. Sick vacheche regai kuchengeta musoro, havagoni kugara kana kumira. Chete zvisingawanzoitiki zviitiko, mwana anokwanisa kubata muviri wakarurama nzvimbo, asi mano ichi haarambi nokururamisira nokukurumidza sezvo kuparadzwa etsinga fibers.

Uyezve, pane dzimwe abnormalities mu skeletal kukura, kunyanya hudyu dysplasia, scoliosis, paumbwe fanera muchipfuva, clubfoot, hydrocephalus.

Munguva akaipa chirwere, uye degeneration tsinga harinzwi ari kufambira mberi nokukurumidza. Atrophy inogona kurondedzerwa kwete chete skeletal tsandanyama, asiwo fibers ari zvomukati. Kazhinji zvakakanganisa diaphragm, zvinoita kuti kufema kukundikana. Zvinosuruvarisa vana vane chirwere chakafanana kurarama (avhareji) kusvika makore mapfumbamwe.

Early Verdniga-Hoffmann chirwere

The chikuru Zviratidzo zviri chirwere anozviratidza yechipiri hafu youpenyu. Yokutanga mishomanana kukura mwana chaiko uchipfuura zvakajairika - mbudzana anodzidza kubata musoro uye vagare, dzimwe nguva kunyange anogona kugara tisingabatsirwi. Asi unyanzvi zvose izvi vakarasika mushure activation chirwere. Zviya, kazhinji chinokurwadzai chirwere syndrome.

Tremor minwe uye mudzira contracture - zviratidzo kuparadzwa tsinga fibers okutanga. Gare gare anovandudza atrophy uye kuoma tsandanyama. Avhareji nguva upenyu wevarwere riri pakati 14 kusvika 16 makore.

Late chirwere Verdniga-Hoffmann

chirwere kwakadaro munyoro. Kazhinji, mwana hwapedyo zvikuru anowanzoita kusvikira makore maviri. The mbudzana anodzidza kugara, mira uye ufambe. Chete chete nguva vabereki vanotanga kuona zvimwe kutsaudzira.

Chokutanga, kushandura gait mwana anorwara - anoenda, zvikuru vakafugama mabvi, kazhinji unowira, usarega pachiyero. Sezvo zvokurwara ungacherechedza zvimwe zvachinja marangwanda, akadai deformation pechipfuva. Verdniga-Hoffman chirwere kunoratidzwa rakasimba tremor, ungaderedza tsandanyama inzwi, kunyangarika huru unconditional hunenge.

Kazhinji makore 10-12 ane mwana zvachose anorasikirwa mano kuzvimirira kufamba. Zvisinei, muchiitiko ichi varwere vararame kusvika 20, uye dzimwe nguva kusvika makore 30.