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可逆性腦血管收縮症候群
Reversible cerebral vasoconstriction syndrome, RCVS
Reversible cerebral vasoconstriction syndrome, RCVS
Recurrent thunderclap headache (發作的一分鐘內即達到最嚴重程度)
血管攝影可見到可逆性的節段性血管收縮
reversible multifocal narrowing of the cerebral arteries, String and beads
less commonly focal neurologic deficits related to brain edema, stroke, or seizure.
The character of these headaches is usually different from the patient's prior migraine headaches,
血管攝影可見到可逆性的節段性血管收縮
reversible multifocal narrowing of the cerebral arteries, String and beads
less commonly focal neurologic deficits related to brain edema, stroke, or seizure.
The character of these headaches is usually different from the patient's prior migraine headaches,
女性居多, 好發的年紀女性約40-50歲, 男性病患30-40歲
有很明顯的頭痛誘發因子
有時也會合併有一些局部神經學症狀, 但往往都是因為已經產生了腦中風、水腫或出血等併發症
原發性 39-94%
次發性 6-61%
(1) 要有明顯劇烈的頭痛(雷擊頭痛)
(2) 血管攝影可以看到節段性血管收縮, 且在3個月內緩解
(3) 排除其他原因, 特別是 SAH
穿顱彩色標定都卜勒超音波是非侵入性且適合反覆追蹤血管收縮的工具
鈣離子阻斷劑特別是 nimodipine 目前被認為治療此病之首選藥物
穿顱彩色標定都卜勒超音波是非侵入性且適合反覆追蹤血管收縮的工具
鈣離子阻斷劑特別是 nimodipine 目前被認為治療此病之首選藥物
Usual stroke preventive medications, such as antiplatelets, anticoagulants, and cholesterol-lowering agents,
are probably not indicated.
10 %的病人在追蹤過程中產生新的類似雷擊頭痛的急性嚴重頭痛，而其中的一半最後確診為RCVS復發
PACNS usually have an insidious progressive clinical course with chronic headaches,
and rarely have thunderclap headache that is typical of RCVS.
and rarely have thunderclap headache that is typical of RCVS.
Other possible differential for thunderclap headache :
(少數可能有 thunderclap headache 之表現)
- Meningitis
- Carotid or vertebral artery dissection
- Cerebral venous sinus thrombosis
- Spontaneous intracranial hypotension
(少數可能有 thunderclap headache 之表現)
- Meningitis
- Carotid or vertebral artery dissection
- Cerebral venous sinus thrombosis
- Spontaneous intracranial hypotension
姿勢性的頭痛為主要表現, 可能合併噁心嘔吐, 暈眩, 聽覺改變, 複視等神經學表現, 少部分合併雷擊性頭痛
此疾病因脊髓腦膜的缺陷, 導致 CSF leakage 而腦壓下降
重要特徵：姿勢性頭痛, 低CSF壓力, 腦部MRI上出現廣泛的腦膜顯影
- Posterior reversible encephalopathy syndrome (PRES)
- Ischemic stroke
- Primary thunderclap headache 原發性雷擊性頭痛 : diagnosis of exclusion
此疾病因脊髓腦膜的缺陷, 導致 CSF leakage 而腦壓下降
重要特徵：姿勢性頭痛, 低CSF壓力, 腦部MRI上出現廣泛的腦膜顯影
- Posterior reversible encephalopathy syndrome (PRES)
- Ischemic stroke
- Primary thunderclap headache 原發性雷擊性頭痛 : diagnosis of exclusion
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