Neuromyelitis Optica

Ko te neuromyelitis optica, e kiia ana hoki ko te NMO, he mate o te pūnaha io o waenganui e pā ana ki te mumura i ngā tūāpapa o te kanohi me te iwi o muri.

Ka kiia hoki te NMO ko te mate whānuitanga o te neuromyelitis optica (NMOSD) me te mate a Devic. Ka puta mai i te whakautu a te pūnaha ārai ki ngā pūtau ake o te tinana. Ka puta tōna hua i te nuinga i te iwi o muri, me ngā tūāpapa tirohanga e hono ana i te retina o te kanohi ki te roro. Engari i etahi wā ka puta i te roro.

Ka puta pea te āhuatanga i muri i te mate, ā, ka hono pea ki tētahi atu āhuatanga ārai-whaiaro. Ka hono ngā anitōi i whakarereketia ki ngā pūmua i te pūnaha io o waenganui, ā, ka pā te kino.

Ka tino whakapohehe te neuromyelitis optica hei mate maha o te pūtau (multiple sclerosis), e kiia ana hoki ko te MS, kāore rānei ka kitea he momo MS. Engari he āhuatanga ke te NMO.

Ka taea e te neuromyelitis optica te whakaeke i te matapo, te ngoikore o ngā waewae, o ngā ringa rānei, me ngā wiri mamae. Ka taea hoki e ia te whakaeke i te ngaro o te āhua, te ruaki me ngā hīkīpī, me ngā tohu o te pūkahukahu, o te kopu rānei.

Ka taea e ngā tohu te pai ake, katahi ka kino anō, e kiia ana he whakahokinga. He mea nui te maimoatanga hei aukati i ngā whakahokinga kia āwhina ai i te aukati i te ngoikore. Ka taea e te NMO te whakaeke i te ngaro o te tirohanga pumau me te raruraru o te hikoi.

Ko ngā tohu o te neuromyelitis optica e hono ana ki te mumura e puta ana i ngā tūpuna o te kanohi me te rōpū o te tuaiwi.

Ko ngā huringa tirohanga e puta mai ana i te NMO e kiia ana ko te optic neuritis. Ā, e āhei ana ēnei:

• Tirohanga mōrearea, kāore rānei e kite i tetahi kanohi, i ngā kanohi e rua rānei.
• Kāore e taea te kite i ngā tae.
• Te mamae o te kanohi.

Ko ngā tohu e pā ana ki te rōpū o te tuaiwi e kiia ana ko te transverse myelitis. Ā, e āhei ana ēnei:

• Te pakari, te ngoikore, te kore rānei o te āhua i ngā waewae, ā, i ētahi wā i ngā ringa.
• Te ngaro o te āhua i ngā ringa, i ngā waewae rānei.
• Kāore e taea te whakapakaru i te putea mimi, te raruraru rānei ki te whakahaere i te mahi o te kopū, o te putea mimi rānei.
• Te āhua wiri, te mamae rānei e puta mai ana i te kaki, te tuara, te kopū rānei.

Ko ētahi atu tohu o te NMO e āhei ana:

• Te hikaka.
• Te ruaki, te ruaki.

Ka taea e ngā tamariki te whakapohehe, te mau te pupuhi, te koma rānei. Heoi, ko ēnei tohu i ngā tamariki he mea noa ake i tetahi āhuatanga e hono ana e kiia ana ko te mate huaketo glycoprotein oligodendrocyte myelin (MOGAD).

Ka taea te pai ake o ngā tohu, katahi ka kino ano. Ina kino haere, e kiia ana he whakahokinga. Ka taea te puta mai o ngā whakahokinga i muri i ngā wiki, ngā marama, ngā tau rānei. I te roanga o te wā, ka taea e ngā whakahokinga te arahi ki te matapo katoa, ki te ngaro rānei o te āhua, e kiia ana ko te pararaihi.

Kaore he uauatanga

Karekau ngā tohunga e tino mōhio ki te take o te neuromyelitis optica. I roto i te hunga e pangia ana e te mate, ka whakaeke te pūnaha ārai mate ki ngā kiko hauora i roto i te pūnaha io matua. Kei roto i te pūnaha io matua te tūpuna o te tuaiwi, te roro me ngā uaua tirohanga e hono ana i te whatu o te kanohi ki te roro. Ka puta te whakaeke nā te hono o ngā antibody i whakarereketia ki ngā pūmua i roto i te pūnaha io matua, ā, ka pā te kino.

Ka puta tēnei āhuatanga o te pūnaha ārai mate i te pupuhi, e mohiotia ana ko te mumura, ā, ka arahi ki te pakaru o ngā pūtau io.

Ko te neuromyelitis optica he mate onge. Ko etahi take ka piki pea te tūponotanga o te whiwhi NMO ko ēnei:

• Te ira tangata i whānau ai. Ka tino pā te NMO ki ngā wāhine atu i ngā tāne.
• Te tau. Ko te nuinga, ka pā te NMO ki ngā tāngata pakeke. Ko te tau toharite o te whakatau i te mate ko te 40. Heoi anō, ka taea hoki e ngā tamariki me ngā kaumātua te whiwhi neuromyelitis optica.
• Te iwi, te momo, te āhua rānei. Ka nui ake te pā o te NMO ki ngā tāngata o te iwi Hispanic, Ahia, Awhurika, Awhurika-Karipiana rānei atu i ngā tāngata Pākehā.

E whakaatu ana etahi rangahau ka piki pea te tūponotanga o te neuromyelitis optica ki te kore e ranea te hua o te wītamini D i roto i te tinana, te kai paipa, me te iti o ngā mate i te timatanga o te ora.

Kaore he uauatanga

Kaore he uauatanga

Ko te whakatau i te neuromyelitis optica e whakauru ana i te whakamātautau tinana me ētahi atu whakamātautau. Ko tetahi wāhanga o te tukanga whakatau ko te whakakore i ētahi atu āhuatanga o te pūnaha io e rite ana ō rātou tohu. Ka titiro hoki ngā tohunga hauora ki ngā tohu me ngā hua whakamātautau e hono ana ki te NMO. I whakaturia ngā tikanga hei whakatau i te mate neuromyelitis optica spectrum disorder (NMOSD) i te tau 2015 e te International Panel for NMO Diagnosis.

Ka arotakehia e tetahi tohunga hauora tō hītori hauora me ō tohu, ā, ka mahi i tetahi whakamātautau tinana. Ko ētahi atu whakamātautau e whakauru ana:

• Whakamātautau Neurological. Ka tirotirohia e te neurologist te nekehanga, te kaha o ngā uaua, te hononga, te āhua, te mahara, te whakaaro, te tirohanga me te kōrero. He taote kanohi pea ka uru atu ki te whakamātautau.
• MRI. Ko tēnei whakamātautau whakaahua e whakamahi ana i te mara hiko me ngā ngaru reo hei hanga i te tirohanga taipitopito o te roro, ngā uaua tirohanga me te tūpuna o te tuaiwi. Ka whakaatu pea ngā hua i ngā lesions, i ngā wāhi pakaru rānei i te roro, ngā uaua tirohanga, i te tūpuna o te tuaiwi rānei.
• Whakamātautau toto. He tohunga hauora pea ka whakamātautau i te toto mō te autoantibody e hono ana ki ngā pūmua, ā, e pā ana ki te NMO. Ko te autoantibody ko te aquaporin-4-immunoglobulin G, e mohiotia ana hoki ko te AQP4-IgG. Ka taea e te whakamātautau mō tēnei autoantibody te āwhina i ngā tohunga hauora ki te wehewehe i waenga i te NMO me te MS, ā, ki te mahi i tetahi whakatau wawe o te NMO.

Ka āwhina ētahi atu biomarkers pēnei i te serum glial fibrillary acidic protein, e kiia ana hoki ko te GFAP, me te serum neurofilament light chain ki te kite i ngā whakahokinga. He whakamātautau antibody myelin oligodendrocyte glycoprotein immunoglobulin G, e kiia ana hoki ko te whakamātautau antibody MOG-IgG, pea ka whakamahia hoki hei rapu i tētahi atu mate mumura e rite ana ki te NMO.

• Lumbar puncture, e mohiotia ana hoki ko te spinal tap. I te wā o tēnei whakamātautau, ka whakauruhia e tetahi tohunga hauora he ngira ki te tua o raro hei tango i te iti o te wai o te tuaiwi. Ka whakatauhia e tēnei whakamātautau ngā taumata o ngā pūtau mate, ngā pūmua me ngā antibodies i roto i te wai. Ka taea pea e tēnei whakamātautau te wehewehe i te NMO i te MS.

Ka whakaatu pea te wai o te tuaiwi i te taumata teitei rawa o ngā pūtau toto ma i ngā wāhanga NMO. He nui ake tēnei i te taumata e kitea ana i te MS, ahakoa kaore tēnei tohu e puta tonu.

• Whakamātautau whakautu stimuli. Hei ako i te pai o te whakautu a te roro ki ngā stimuli pēnei i ngā oro, ngā tirohanga, i te pa rānei, pea ka whai koe i tetahi whakamātautau e kiia nei he whakamātautau evoked potentials, he whakamātautau evoked response rānei.

Ka honoa ngā waea e kiia nei ko ngā electrodes ki te pūtake o te matenga, ā, i ētahi wā, ngā ihu, te kaki, ngā ringa, ngā waewae me te tua. Ka tuhia e ngā taputapu e hono ana ki ngā electrodes ngā whakautu a te roro ki ngā stimuli. Ka āwhina ēnei whakamātautau ki te kimi i ngā lesions, i ngā wāhi pakaru rānei i ngā uaua, te tūpuna o te tuaiwi, te uaua tirohanga, te roro, i te brainstem rānei.

• Optical coherence tomography. Ka titiro tēnei whakamātautau ki te paparanga uaua retinal me tōna māngai. Ko ngā turoro he uaua tirohanga mumura mai i te NMO he nui ake te ngaro o te tirohanga me te whakaheke o te uaua retinal i te hunga he MS.

Kaore e taea te whakaora i te neuromyelitis optica. Engari ka taea e te maimoatanga te arahi i te wa roa kaore he tohu, e kiia ana ko te remission. Ko te maimoatanga NMO kei roto nga rongoa hei whakahoki i nga tohu hou me te aukati i nga whakaeke o muri mai.

• Te whakahoki i nga tohu hou. I te tīmatanga o te whakaeke NMO, ka hoatu pea e te tohunga hauora te rongoa corticosteroid penei i te methylprednisolone (Solu-Medrol). Ka hoatuhia ma te uaua i te ringa. Ka tangohia te rongoa mo te rima ra pea, katahi ka whakaitihia noa iho i roto i nga ra maha.

  He maha nga wa ka taunakihia te whakawhiti plasma hei maimoatanga tuatahi, tuarua ranei, i te nuinga o te wa hei taapiri ki te rongoa steroid. I roto i tenei tukanga, ka tangohia etahi toto mai i te tinana, ka wehea nga pūtau toto mai i te wai e kiia nei ko te plasma. Ka whakaranu nga pūtau toto ki te otinga whakakapi ka whakahokia te toto ki te tinana. Ka taea e tenei tukanga te tango i nga mea kino me te horoi i te toto.

  Ka taea hoki e nga tohunga hauora te awhina i te whakahaere i etahi atu tohu pea, penei i te mamae, te raruraru o te uaua ranei.

• Te aukati i nga whakaeke o muri mai. Ka taunaki pea to tohunga hauora kia tango koe i te wa iti o te corticosteroids i te wa roa hei aukati i nga whakaeke NMO me nga whakahokinga mai o muri mai.

Te whakahoki i nga tohu hou. I te tīmatanga o te whakaeke NMO, ka hoatu pea e te tohunga hauora te rongoa corticosteroid penei i te methylprednisolone (Solu-Medrol). Ka hoatuhia ma te uaua i te ringa. Ka tangohia te rongoa mo te rima ra pea, katahi ka whakaitihia noa iho i roto i nga ra maha.

Plasma exchange is often recommended as the first or second treatment, usually in addition to steroid therapy. In this procedure, some blood is removed from the body, and blood cells are mechanically separated from fluid called plasma. The blood cells are mixed with a replacement solution and the blood is returned to the body. This process can remove harmful substances and cleanse the blood.

Healthcare professionals also can help manage other possible symptoms, such as pain or muscle problems.

Te whakaiti i nga whakahokinga. Kua whakaatuhia i roto i nga whakamatautau hauora ko nga antibodies monoclonal he pai ki te whakaiti i te morearea o nga whakahokinga NMO. Kei roto i enei rongoa te eculizumab (Soliris), satralizumab (Enspryng), inebilizumab (Uplizna), ravulizumab (Ultomiris) me te rituximab (Rituxan). He maha o enei kua whakaaetia e te U.S. Food and Drug Administration (FDA) mo te aukati i nga whakahokinga i roto i nga pakeke.

Ka taea e nga immunoglobulins intravenous, e mohiotia ana ko nga antibodies, te whakaiti i te tere o te whakahokinga o te NMO.

Kaore he uauatanga