Dravet ciwo. Mai tsananin myoclonic epilepsy na jariri

Daga cikin daban-daban manifestations na yarantaka epilepsy Dravet ciwo ne na musamman wuri, kuma shi ne mai yiwuwa ya fi tsanani, kuma rai-barazanar cuta na yaro. Wannan ciwo bayyana kanta a cikin shekarar farko ta rayuwa da jariri da kuma sau da yawa take kaiwa zuwa tsanani rushewa da ya psychomotor ci gaba, da kuma a wasu lokuta - da kuma mutuwa. Wannan shi ne quite a rare cutar da manyan cututtuka da jiyya, da kuma za mu tattauna a wannan talifin.

A wasu lokuta, nuna gaban Dravet ciwo?

Dravet ciwo ne ba na kowa - lura da cewa wannan cuta shi ne batun 1 mutumin da dubu 40 jarirai (da boys sa up game da 66% daga cikin lokuta) .. Amma wannan, ta hanyar, kaiwa zuwa da cewa daidai ganewar asali, likitoci wani lokacin wuya shigar da, ta haka ne, muhimmanci da lokaci da aka rasa. Kuma a lokacin da ya kira ciwo ba tare da tabbatarwa far da yaro ta yanayin yawanci worsens da shekaru.

Idan akwai wani jariri wanda ya ba kai shekara, m bouts da serial ko matsayi a cikin (watau. E., su bi da juna, da kuma haƙuri a cikin gibba ba ta zo hankali) da kuma resistant zuwa mataki na anticonvulsant kwayoyi, za a iya zargin ciwo Drava.

Yara da wannan sau da yawa da mahara kai hare-hare a lokacin da rana, da kuma wannan yanayin yana for game da mako guda. Sa'an nan a lull for mako biyu, da kuma duk abin da aka maimaita sake.

Status epilepticus da Dravet ciwo - fairly kowa sabon abu. Ya iya a tare da convulsions ko zama ba convulsive, wani daban-daban tsanani hargitsi na sani da segmental myoclonia (azumi tsoka twitching).

Dravet ciwo: haddasawa

Babban dalilin da ciwo aka bayyana ta da masu bincike kira a kayyade predisposition, wato - gaban a cikin haƙuri na sodium tashar gene maye gurbi.

M dalilai na farko daga cikin yanayin da aka bayyana a cikin jarirai zazzabi sau da yawa ya yi aikin a lokacin rashin lafiyarsa, shan mai zafi wanka, zafi fiye da kima. Har ila yau, zai iya zama mai tsanani ko kuma gajiya ruri haske (walƙiya fitilu, da miƙa mulki daga duhu dakin a cikin haske, da sauransu. N.). Ya kamata a lura cewa duk da wannan kuma, a cikin wadannan shekaru haƙuri da rayuwa zai zama haɗari, haddasa hare-haren a kan ikon da shi.

Dravet ciwo: Alamun

Babban manifestations Dravet ciwo za a iya dangana, da kuma mai da hankali da kuma jimlace seizures. Mai da hankali seizures ne halin a cewa zumudi yankin, har suka sa su aukuwa, shi ne kawai wani sashi na kwakwalwa. A cikin hali na pathological aiki na neurons a duka hemispheres shi ne na ra'ayin kowa, damƙa.

Convulsions a Dravet ciwo sau da yawa ne polymorphic a yanayi. Yaro a shekara iya fuskantar clonic (tare da canji a cikin tsoka sautin), tonic (wanda suke fairly dogon tsoka spasm) da kuma kowa yana da myoclonic seizures.

Sau da yawa akwai kuma kai hare-hare a cikin nau'i na atypical absences - yanayi a cikin abin da yaro ya sani ba partially ko gaba daya da amsa zuwa ga yanayi. The yaro zai iya kangararre a lokacin, neman a daya batu, lankwasa da baya, kwatsam digo ko kawai sauke cewa a hannunsa.

Mafi sau da yawa da aka ambata seizures faruwa a lokacin falke, kazalika a falke (a cikin mafarki, suka rubuce kawai 3% da marasa lafiya tare da wannan ganewar asali).

Yadda za a ci gaba na asibiti manifestations Dravet ciwo?

Kamar yadda mai mulkin, Dravet ciwo ne halin da cewa da aka ambata a sama bayyanar cututtuka faruwa a wasu jerin. Doctors rarrabe tsakanin uku da manyan lokaci na rashin lafiya.

1. A gwada da m lokaci, tare da bayyanar clonic seizures (azumi fizge, daya bayan daya, bayan wani gajeren lokaci). A matsayin sa halin da ake ciki, a matsayin mai mulkin, yana tsaye da zazzabi Yunƙurin a cikin yaro, amma a nan gaba za su iya bayyana da kansa daga gare shi.
2. Tashin m - tare da zuwan yawa myoclonic seizures. Su ne sau da yawa febrile (t. E. danganta da yawan zafin jiki karuwa) da kuma amfani da akwati da extremities. Domin myoclonic seizures shiga atypical rashi seizures da hadaddun mai da hankali seizures.
3. A tsaye lokaci a cikin abin da weaker harin, tun lokacin da yaron ya zauna a tsanani neurological da tabin hankali cuta.

Babban ãyõyin tsanani myoclonic epilepsy na yarantaka

Kamar yadda muka ce, a cikin ra'ayi na gaskiya cewa Dravet ciwo - a rare cuta, da masana sau da yawa da wahala a diagnosing shi. Saboda haka, iyaye yana da muhimmanci ga samar da cikakken bayanai game da ci gaban da pathological yanayin yaro. Mai suna ciwo iya zargin idan akwai samuwa da wadannan siffofin:

• cutar ya cigaban daya shekara da shekaru.
• polymorphous seizures (.. watau su bambancin bayyananen).
• hari da aka ba tsaya liyafar hankula anticonvulsants.
• da ya faru na seizures daura da karuwa a yaro ta yanayin jiki.
• jaririn m raya ci gaban jinkiri (wannan alama za a iya bayyana, a sãɓãwar launukansa digiri).
• furta ataxia cututtuka (raskoordinirovannosti ƙungiyoyi).
• MRI shaida ba tabbatar da kasancewar cutar (musamman a farkon cutar).
• EEG - slowing na baya kari kuma multifocal keta ƙaddamar da zangarniya da kuma jinkirin tãguwar ruwa.

Bugu da kari a wadannan cututtuka ga yara da Dravet ciwo yawanci halin gaban Hyperactivity da hankali gaira cuta.

Forecast na ci gaba Dravet ciwo

Hasashen tsanani myoclonic epilepsy ne yawanci ya fi karfinsu. Dukan marasa lafiya bincikar lafiya tare da Dravet ciwo lura shafi tunanin mutum retardation, da kuma rabin shi ne nauyi. Bayan shekaru hudu da marasa lafiya akwai wani m deterioration, tare da ci gaba da halayya munanan, ciki har da psychosis.

Abin baƙin ciki, na mutuwa a bayyana pathologies ne ma sosai high - har zuwa 18%, kuma shi sa mafi m hatsarori a lokacin seizures ko matsayi epilepticus.

Domin rage hadarin tsanani sakamakon a lokacin da satar wa yaro, iyaye bukatar mai kyau fahimtar yadda za a samar da taimakon farko ga seizures.

Yadda za a samar da taimakon farko ga seizures sa ta high yanayin zafi?

Idan yaro yana da seizures a mayar da martani ga wani Yunƙurin a zazzabi (wanda, kamar yadda ka iya tuna, wannan cuta ne daya daga cikin manyan siffofin), da tsayar da wadannan sharudda:

• Sa da jariri a kan wani lebur surface.
• samar da sabo iska.
• tsaftace jariri ta bakin gamsai.
• kunna yaro ta kai daya gefen.
• Ku ciyar antipyretic ayyukan.

Idan yaro ya bayyana a zazzabi, cewa shi ne, goshinsa zafi, sai fuskarsa ta fatattaki, sa'an nan taimakon farko ga seizures ya kamata a da nufin rage yawan zafin jiki (sanyi rigar damfara a goshin, sanyi da armpits kuma a cikin makwancin gwaiwa, jiki rubdown da ruwa da kuma vinegar a cikin wani rabo na 1 1, antipyretics).

Idan yaro a kan wani bango na high zazzabi fata ne kodadde, bluish lebe da kuma yatsun, jin sanyi, sanyi da dugaduganmu, kuma dabino, da rubdown da sanyi compresses bai kamata a yi. Dumi Toddler kamata ba antipyretics, da Allunan "A'a-dima jiki" ko "Papaverine" da kudi na 1 MG da 1 kg jiki nauyi ga vasodilatation.

Taimaka a lokacin da tura farfadiya seizures

A taron na tura farfadiya, damƙa da kowa yana da clonic da tonic convulsions na yaro dole ne:

• aza a kan wani lebur surface.
• a karkashin shugaban to ƙulla wani abu da taushi, jariri bai yi yãƙi da shi.
• samar da sabo iska ci.
• tsabtace cikin bakin da makogwaro na gamsai.
• ku jũyar da kai ga daya gefen.
• haɗa wani yanki na zane a cikin wani kulli da kuma saka shi tsakanin hakora don hana cizon da harshe, da lebe, a matsayin yaro mai shekara iya zama abu mai ƙwari (a cokali, a sanda) ya karya masa hakora.
• shafa ya bayyana bakin kumfa tawul.
• don tabbatar da cewa a lokacin wannan harin da jariri ba buga a kan wani abu.

Idan convulsions dauki cikin irin matsayi, tabbata a kira "motar asibiti."

Ka'idodi na lura da yara da Dravet ta ciwo

rashin lafiya yaro lura da cutar da aka bayyana deceleration an rage su hana ci gaban seizures da kuma matsayin su form.

Lokacin da ganewar asali na lura "Dravet ciwo" zame amfani da yadu da aka sani antiepileptic kwayoyi: "Carbamazepine", "Finlepsin", "Phenytoin" da "lamotrigine" tun da suka kawai worsen haƙuri da yanayin, tabi cikin data kasance siffofin seizures.

Bugu da kari ga m miyagun ƙwayoyi jiyya, yana da muhimmanci a tuna da rigakafin zazzabin, tun da shi ne a jihar musamman m ga mãsu haƙuri. Don ware tsokane hare-hare na haske ruri da aka miƙa don sa tabarau tare da blue ruwan tabarau ko daya taped gilashi.

Dravet ciwo Drug Mafia

Idan ganewar asali aka tabbatar, magani fara da farko na da miyagun ƙwayoyi "Topiramate". Kuma an wajabta, kashi na 12.5 MG / rana., A hankali kara zuwa 3-10 MG / kg / rana. (Medicine dauka sau biyu a rana). Wannan kayan aiki ne musamman da amfani a lokuta inda yara da aka bayyana epilepsy bayyana jimlace seizures da kuma paroxysms na convulsions tare da sauya sheka daga daya da rabi daga cikin jiki zuwa wani (gemikonvulsiyami).

Wadannan formulations domin monotherapy - wani wanda aka samu daga valproic acid (syrup "Konvuleks", "Konvulsofin", da dai sauransu ...) - shi ne musamman tasiri a atypical absences da myoclonus, kuma barbituric acid ( "Phenobarbital") a yi amfani da ra'ayin kowa convulsions da propensity zuwa matsayi na yanzu. Ba zato ba tsammani, a cikin wannan harka, da kuma lura high dace na bromides.

Idan dole, yi amfani da haɗin kwayoyi. A mafi tasiri daga cikinsu shi ne mai hade da valproate da nufin "topiramate".