進行性多灶性白質腦病 (PML) 和急性脫髓鞘性多發性神經病變 (GBS) 都是免疫系統異常導致髓鞘的損傷,也因此干擾神經信號的傳遞,從而導致疾病。
GBS急性脫髓鞘性多發性神經病變
空腸彎曲桿菌(Campylobacter jejuni)所造成的腸炎,最可能引發周邊神經病變(Guillain-Barré症候群,GBS),
主要涉及以下兩個方面:
分子相似性(感染後1-2週): 空腸彎曲桿菌的脂多醣(LPS)與人類周邊神經的髓鞘神經節糖苷(ganglioside)有相似的抗原表位。在感染後抗體誤認下攻擊自身的髓鞘神經節糖苷,導致髓鞘脫髓鞘病變。
細胞介導的免疫反應(感染後2-4週): 空腸彎曲桿菌感染使免疫細胞釋放出促炎細胞因子和免疫活性分子,但因這些物質會損傷周邊神經細胞,導致炎症和神經損傷。
GBS是一種急性脫髓鞘性多發性神經病變,免疫系統攻擊髓鞘自身,進而影響周邊神經,其特徵是肌肉無力、感覺異常和反射消失。治療方法包括血漿置換術和免疫球蛋白靜脈注射,這些療法可以幫助改善患者的症狀和預後。
進行性多灶性白質腦病(progressive multifocal leukoencephalopathy, PML)
進行性多灶性白質腦病(progressive multifocal leukoencephalopathy, PML)是屬於白質腦病的一種,而白質腦病又屬於中樞神經系統脫髓鞘疾病,臨床上常與免疫抑制有關,免疫系統攻擊髓鞘構成的髓鞘少突膠質細胞,主要影響大腦白質。此病主要由JC virus病毒引起,JCV是一種多瘤病毒,在一般人口中很常見,但只有在免疫系統受損的情況下才會導致疾病。
PML的症狀通常是逐漸發生的,可能包括:認知能力下降、視力或聽力問題、虛弱或麻木、行走困難、說話困難、癲癇發作。
The symptoms of PML usually develop gradually and can include:Cognitive decline,Vision or hearing problems,Weakness or numbness,Difficulty walking,Difficulty speaking,Seizures.
大多數患者在診斷後1-2年內死亡。
The prognosis for PML is poor, with most patients dying within 1-2 years of diagnosis.
癲癇/epilepsy
癲癇重積狀態(status epilepticus,SE)
癲癇重積狀態指的是持續性或反覆發作的癲癇,且在發作間期患者並未恢復意識。無論是全身性大發作(tonic-clonic seizure)、局部性發作,或是其他型態的癲癇,只要符合上述條件,都可能發生癲癇重積狀態。
Status epilepticus (SE) is defined as a prolonged or repetitive seizure where the patient does not regain consciousness between seizures. Regardless of whether it's a generalized tonic-clonic seizure, a partial seizure, or another type of seizure, as long as it meets the above criteria, it can be considered status epilepticus.
1.發生時,依醫囑立即予靜脈輸入短效抗癲癇藥,癲癇重積狀態是一種急症,需要立即給予靜脈注射短效型抗癲癇藥物來控制發作。
Immediate intravenous administration of short-acting anti-epileptic drugs: SE is a medical emergency and requires immediate intravenous administration of short-acting anti-epileptic drugs to control the seizure.
2.無論是全身性大發作還是局部性發作(partial seizure),只要符合癲癇重積狀態的定義,都有可能發生,小發作(absence seizure) 雖然通常表現為意識喪失數秒,但若持續時間過長或頻繁發作,也可能發展成癲癇重積狀態。
Both generalized tonic-clonic seizures and partial seizures can evolve into SE: Any type of seizure, including absence seizures (although typically characterized by brief loss of consciousness), can develop into SE if the duration is prolonged or if seizures occur frequently.
3.癲癇重積狀態的定義是持續性或反覆發作的癲癇,且在發作間期患者並未恢復意識。一次發作超過5分鐘只是其中一種情形,並非絕對標準。
Duration is not the sole criterion for SE: While a seizure lasting more than 5 minutes is a common indicator, it's not the only criterion for diagnosing SE. The key is the continuous or repetitive nature of seizures and the lack of recovery of consciousness between them.
4.可能發生意識在兩次發作間沒有完全恢復
Impaired consciousness between seizures: One hallmark of SE is that the patient's consciousness does not fully recover between seizures.
任何型態的癲癇都有可能發展成癲癇重積狀態。及早診斷和治療是改善預後的關鍵,而癲癇重積狀態是一種神經急症,若未及時處理,可能導致腦損傷、甚至死亡。因此,一旦懷疑患者發生癲癇重積狀態,應立即尋求醫療協助。
Any type of seizure has the potential to progress into status epilepticus. Early diagnosis and treatment are crucial for a favorable outcome. SE is a neurological emergency that, if left untreated, can lead to brain damage or even death. Therefore, if you suspect someone is experiencing status epilepticus, seek medical attention immediately.
Dilantin
治療癲癇的時候,有一種Dilantin(癲能停)抗癲癇藥物
Dilantin (phenytoin) is an anticonvulsant medication used to treat epilepsy.
1.Dilantin通常靜脈輸注時使用生理鹽水稀釋,而不用5%葡萄糖稀釋。
Dilantin is typically diluted with saline solution for intravenous infusion, rather than 5% dextrose.
2.Dilantin的血清治療濃度範圍為10~20 mcg/dL,而不是80~100 mcg/dL。
The therapeutic serum concentration range for Dilantin is 10-20 mcg/dL, not 80-100 mcg/dL.
Dilantin的常見副作用包括以下幾種:
Common Side Effects:
1.會刺激牙齦生長,導致牙齦增生。牙齦增生是一種可逆的副作用,通常可以在停止服用Dilantin後消退。
Dilantin can stimulate gum growth, leading to gingival hyperplasia. Gingival hyperplasia is a reversible side effect that usually subsides after discontinuing Dilantin.
2.導致口腔黏膜發炎,導致口腔潰瘍、疼痛等症狀。
Dilantin can cause inflammation of the oral mucosa, leading to mouth ulcers, pain, and other symptoms.
3.導致牙齒脫落,但這種副作用較為罕見。
Dilantin can cause tooth loss, but this side effect is relatively rare.
4.血液惡病質和心律不整是較為罕見的副作用。
Blood dyscrasias and heart rhythm disturbances are rare side effects.
所以需要維持良好的口腔衛生、定期進行口腔檢查、按摩牙齦及避免使用刺激性物質
Maintain good oral hygiene,Have regular dental checkups,Massage the gums and Avoid using irritating substances
癲癇-生酮飲食療法
Ketogenic Diet for Epilepsy
生酮飲食主要用於藥物難治型癲癇的兒童,當傳統的抗癲癇藥物無法有效控制病情時,生酮飲食才被視為一種替代或輔助治療方式。
The ketogenic diet is primarily used as a treatment for children with intractable epilepsy, meaning when traditional anti-epileptic drugs are ineffective in controlling seizures.
高脂肪、低碳水化合物、適度蛋白質的飲食
生酮飲食的核心是極低碳水化合物,迫使身體將脂肪轉化為酮體作為能量來源。高脂肪提供身體所需的能量,而過多的蛋白質會產生葡萄糖,影響生酮效果。
High-fat, low-carbohydrate, and moderate-protein diet
The core of the ketogenic diet is extremely low carbohydrate intake, forcing the body to convert fat into ketones for energy. High fat provides the body with the energy it needs, while excessive protein can produce glucose, which can interfere with the ketogenic effect.
酮(ketones)具有鎮定效果,可增加穩定性神經傳導物質GABA的產生,有助於抑制過度興奮的神經元,且酮體具有抗氧化作用,可以保護神經細胞免受損傷,達到抗癲癇的效果
Ketones have a sedative effect, increasing the production of the stabilizing neurotransmitter GABA. This helps to inhibit overexcited neurons. Additionally, ketones have antioxidant properties that can protect nerve cells from damage, achieving an anti-epileptic effect.
需要增加液體攝取,為了預防酮症酸中毒時可能發生的脫水現象。
Increased fluid intake is necessary to prevent dehydration, which can occur during ketoacidosis.
留言