Lefu la Lambert-Eaton

Lambert-Eaton syndrome (LES) ke lefu le sa tloaelehang leo ho lona puisano e fosahetseng lipakeng tsa methapo le mesifa e lebisang bofokoling ba mesifa.

LES ke boloetse bo ikemetseng. Sena se bolela hore sesole sa hau sa 'mele se fosahetse ho hlasela lisele le lisele tse phetseng hantle' meleng. Ka LES, lithibela-mafu tse hlahisoang ke sesole sa 'mele li hlasela lisele tsa methapo. Sena se etsa hore lisele tsa methapo li sitoe ho ntša khemikhale e lekaneng e bitsoang acetylcholine. K'hemik'hale ena e fetisa maikutlo lipakeng tsa methapo le mesifa. Phello ke bofokoli ba mesifa.

LES e ka hlaha ka mofets'e joalo ka mofets'e o monyane oa lisele tsa matšoafo kapa mathata a ho itšireletsa mafung joalo ka vitiligo, e lebisang tahlehelong ea letlalo la letlalo.

LES e ama banna khafetsa ho feta basali. Lilemo tse tloaelehileng tsa ketsahalo li ka ba lilemo tse 60. LES ha e fumanehe hangata ho bana.

Bofokoli kapa tahlehelo ea motsamao e ka bang boima kapa ho feta, ho kenyelletsa:

• Ho thata ho hloa litepisi, ho tsamaea kapa ho phahamisa lintho
• Bohloko ba mesifa
• Ho koba hlooho
• Tlhoko ea ho sebelisa matsoho ho tsoha setulong kapa sebakeng sa leshano
• Mathata ho bua
• Mathata a ho hlafuna kapa ho koenya, ho ka kenyelletsang ho koenya kapa ho bipetsa
• Pono ea liphetoho, joalo ka pono e lerootho, pono e habeli, le bothata ba ho shebisisa

Bofokoli ka kakaretso bo bonolo ho LES. Mesifa ea maoto e ameha haholo. Bofokoli bo ka ntlafala kamora ho ikoetlisa, empa boiteko bo tsoelang pele bo baka mokhathala maemong a mang.

Matšoao a amanang le likarolo tse ling tsa sistimi ea methapo a hlaha khafetsa, 'me a kenyelletsa:

• Khatello ea mali ea fetoha
• Botsoa ha u eme
• Molomo o omileng
• Ho se sebetse ha Erectile
• Mahlo a omileng
• Ho sokela
• Ho fufuleloa ho fokotsehileng

Mofani oa tlhokomelo ea bophelo o tla etsa tlhahlobo ea 'mele mme a botse ka matšoao. Tlhahlobo e ka bonts'a:

• Ho fokotseha ha maikutlo
• Ho ka etsahala ho lahleheloa ke lisele tsa mesifa
• Bofokoli kapa ho holofala ho ntlafalang hanyane ka ts'ebetso

Liteko tsa ho thusa ho fumana le ho netefatsa LES li ka kenyelletsa:

• Diteko tsa madi ho sheba masole a mmele a hlaselang methapo
• Electromyography (EMG) ho leka bophelo bo botle ba likhoele tsa mesifa
• Leqhubu la methapo ea methapo (NCV) ho leka lebelo la ts'ebetso ea motlakase ka methapo

CT scan le MRI ea sefuba le mpa, e lateloe ke bronchoscopy bakeng sa batho ba tsubang e kanna ea etsoa ho khetholla mofets'e. PET scan e ka boela ea etsoa haeba ho belaelloa hlahala ea matšoafo.

Morero oa mantlha oa kalafo ke ho:

• Khetholla le ho phekola mathata afe kapa afe a teng, joalo ka mofetše oa matšoafo
• Fana ka kalafo ho thusa ka bofokoli

Phapanyetsano ea lero la mali, kapa plasmapheresis, ke kalafo e thusang ho tlosa 'mele liprotheine life kapa life tse kotsi tse sitisang tšebetso ea methapo. Sena se kenyelletsa ho tlosa lero la mali le nang le lithibela-mafu. Liprotheine tse ling (tse kang albumin) kapa plasma e fanoeng li kenella 'meleng.

Mokhoa o mong o kenyelletsa ho sebelisa immunoglobulin (IVIg) e kenang methapong ho kenya lisireletsi tse ngata tse thusang ka kotloloho maling.

Meriana eo le eona e ka lekang e kenyelletsa:

• Lithethefatsi tse hatellang karabelo ea sesole sa 'mele
• Lithethefatsi tsa li-anticholinesterase ho ntlafatsa molumo oa mesifa (leha tsena li sa sebetse haholo ha li fanoa li le mong)
• Lithethefatsi tse eketsang tokollo ea acetylcholine liseleng tsa methapo

Matšoao a LES a ka ntlafala ka ho phekola lefu la mantlha, ho hatella sesole sa 'mele kapa ho tlosa li-antibodies. Leha ho le joalo, paraneoplastic LES e kanna ea se arabele kalafo. (Matšoao a Paraneoplastic LES a bakoa ke phetoho ea boits'ireletso ba mmele ho hlahala). Lefu le bakoa ke bokhopo bo ka sehloohong.

Mathata a LES a ka kenyelletsa:

• Ho phefumoloha ho thata, ho kenyelletsa ho hloleha ho hema (ho sa tloaelehang)
• Ho thata ho koenya
• Ts'oaetso, joalo ka pneumonia
• Likotsi tsa ho oa le mathata a ho hokahana

Bitsa mofani oa hau haeba matšoao a LES a hlaha.

Lefu la Myasthenic; Lefu la Eaton-Lambert; Lefu la Lambert-Eaton myasthenic; LEMS; LES

• Mesifa ea ka ntle e kantle

Evoli A, Vincent A. Mathata a phetisetso ea neuromuscular. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 394.

Mohlomong HE. Matšoao a methapo ea mahlo le sefahleho. Ka: Liu GT, Volpe NJ, Galetta SL, li-eds. Liu, Volpe, le Neuro-Ophthalmology ea Galetta. 3rd ed. Philadelphia, PA: Elsevier; 2019: khaolo ea 14.

Sanders DB, Guptill JT. Mathata a phetisetso ea neuromuscular. Ka: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, bahlophisi. Neurology ea Bradley ho Tloaelo ea Bongaka. La 7th. Philadelphia, PA: Elsevier; 2016: khaolo ea 109.