Concomitant reversible cerebral vasoconstriction syndrome and transient global amnesia

Journal of Neurology - Reversible cerebral vasoconstriction syndrome (RCVS) is a common cause of thunderclap headache (TCH), mainly recurrent, sometimes associated with seizures and/or neurological...     

A pediatric case of reversible segmental cerebral vasoconstriction

BACKGROUND: Reversible segmental cerebral vasoconstriction (RSCV) is a recognizable clinical and radiographic syndrome consisting of thunderclap headache with or without focal neurological symptoms combined with reversible segmental vasoconstriction of proximal cerebral blood vessels. METHODS: We report a case of reversible segmental cerebral vasoconstriction in a child. RESULTS: A healthy 13-year-old boy experienced the sudden onset of a severe, diffuse headache upon surfacing from a deep dive in a swimming pool. Severity was maximal at the onset and improved over several hours. The same headache recurred three times over the next four days and a low baseline headache persisted throughout. Vomiting occurred once and mild photo/osmophobia were reported but throbbing, aura, or autonomic symptoms were absent. Focal neurological signs or symptoms were absent and he denied previous history of headaches, medications, drugs, or trauma. Two normal CT scans were performed within hours of separate headaches. Cerebrospinal fluid study on day 5 was bloody with no xanthochromia. MRI/MRA/MRV of the brain and vasculitic work-up were normal. Cerebral angiography on day 6 demonstrated smooth narrowing of multiple proximal cerebral vessels including supraclinoid internal carotid artery (ICA), M1, and A1 on the right and M1 on the left. By ten days, the patient's headaches had resolved and repeat angiography was normal. CONCLUSION: RSCV should be considered in a child with thunderclap headache.     

Isolated thunderclap headache during sex: Orgasmic headache or reversible cerebral vasoconstriction syndrome?

Orgasmic headache (OH) is an “explosive” headache that occurs at orgasm. Historically, it was considered benign with no treatment needed. Reversible cerebral vasoconstriction syndrome (RCVS) refers to a group of disorders characterized by recurrent thunderclap headache (TCH) and multifocal vasoconstriction. Patients who have RCVS often recover completely, but some may have persistent neurological deficits. We report a 34-year-old woman who presented with isolated and recurrent TCH at orgasm, which fulfilled the diagnosis of OH. However, she was post-partum and had recent exposure to ecstasy, making her symptoms highly suggestive of RCVS. Brain magnetic resonance angiography showed segmental vasoconstriction. We concluded that she could be considered to have either OH or RCVS. This patient suggests the theory that OH could be a presentation of RCVS. Given that RCVS is potentially treatable, early recognition by clinicians is vital in order to prevent devastating complications.     

Hyperperfusion Syndrome after Stent/Coiling of a Ruptured Carotid Bifurcation Aneurysm

The authors report a syndrome of regional, symptomatic cerebral hyperperfusion, and edema mimicking infarction in a 54-year-old woman following coiling of a ruptured right carotid bifurcation aneurysm and stenting of the right middle cerebral artery. The patient presented with a Hunt and Hess grade III subarachnoid hemorrhage 7 days after developing thunderclap headache. She underwent successful coiling under general anesthesia of the 1.6 × 1.5 × 1.6 cm aneurysm, but immediately after the coil was placed occlusion of the proximal M1 segment was developed. This occlusion was stented after ~5-min delay, and flow restored without angiographic evidence of distal emboli. Following the procedure, she was extubated and noted to have left hemiparesis, neglect, and mutism without a CT correlate. Cerebral infarction was suspected, but urgent repeat angiography demonstrated patent cerebral vasculature. On the following day, symptoms persisted, and non-contrast head CT now showed cerebral edema localized to the right middle cerebral artery territory mimicking subacute infarction. CT perfusion imaging and angiography showed a widely patent MCA circulation, and suggested a regional hyperperfusion syndrome. The blood pressure was incrementally lowered, with rapid and sustained neurological improvement. Hyperperfusion events following aneurysm repair and related circumstances are reviewed.     

Thunderclap headache

Thunderclap headache (TCH) is head pain that begins suddenly and is severe at onset. TCH might be the first sign of subarachnoid haemorrhage, unruptured intracranial aneurysm, cerebral venous sinus thrombosis, cervical artery dissection, acute hypertensive crisis, spontaneous intracranial hypotension, ischaemic stroke, retroclival haematoma, pituitary apoplexy, third ventricle colloid cyst, and intracranial infection. Primary thunderclap headache is diagnosed when no underlying cause is discovered. Patients with TCH who have evidence of reversible, segmental, cerebral vasoconstriction of circle of Willis arteries and normal or near-normal results on cerebrospinal fluid assessment are thought to have reversible cerebral vasoconstriction syndrome. Herein, we discuss the differential diagnosis of TCH, diagnostic criteria for the primary disorder, and proper assessment of patients. We also offer pathophysiological considerations for primary TCH.     

Nimodipine for treatment of primary thunderclap headache

Eleven patients with primary thunderclap headache (TCH) were treated with oral nimodipine 30 to 60 mg every 4 hours or IV nimodipine 0.5 to 2 mg/h if the oral regimen failed or images showed cerebral vasospasm. With oral nimodipine, headache did not recur in the nine patients without vasospasm. IV nimodipine was given in two patients with vasospasm, including one who developed ischemic stroke. Nimodipine may be effective for TCH. Vasospasm may warrant IV nimodipine.     

Case of posterior reversible encephalopathy syndrome with cerebral vasoconstriction]

A 56-year-old woman attended our hospital because of acute severe (thunderclap) headache. Neurological examination was normal and no abnormality was found on head CT or by cerebrospinal fluid examination. A few days later, she experienced a recurrence and suffered a seizure in her left upper and lower extremities. On neurological examination, she had conjugate deviation of the eyes toward the right side and left lower limb paralysis with Chaddock sign. MRI showed multiple hyperintense lesions in the bilateral occipital and parietal lobes, predominantly in the subcortical white matter at the right side on T2-weighted and FLAIR images. We diagnosed posterior reversible encephalopathy syndrome (PRES) because the ADC map showed a vasogenic edema pattern (increased ADC values in the hypodense lesions on diffusion-weighted image). Her blood pressure was normal and there were no underling diseases. As MRA showed vasoconstriction especially in bilateral posterior cerebral arteries, we initiated a therapy with a Ca-channel blocker. On follow-up MRI, the hyperintense lesions on T2-weighted and FLAIR images had almost disappeared, and vasoconstriction was also improved on MRA. This case suggested that cerebral vasoconstriction could underlie both thunderclap headache and PRES.     

Ischemic stroke revealed by a thunderclap headache: contribution of diffusion-weighted mri sequences

INTRODUCTION: Sub-arachnoid hemorrhage, cerebral venous thrombosis and cerebral reversible angiopathies are the main causes of secondary thunderclap headache. Ischemic stroke is rarely revealed by thunderclap headache. CASE REPORT: A 80-year-old woman developed for the first time a thunderclap headache associated with transient distal motor deficit of the right hand. Diffusion-weighted sequences revealed a recent left infarct in a vascular border zone. CONCLUSION: Ischemic stroke is a rare cause of thunderclap headache but practitioners should bear in mind this etiology when the diagnostic work-up is negative. Diffusion-weighted sequences of the brain MRI can provide the diagnosis.     

A case of reversible cerebral vasoconstriction syndrome (RCVS) triggered by a Chinese herbal medicine]

A 51-year-old woman started taking Chinese medicine containing ephedara herba as a nasal decongestant. One week later, she had three episodes of thunderclap headache, one during defecation and the others while taking a bath. She then had a convulsive seizure upon resolution of the second headache. A cranial CT did not show subarachnoid hemorrhage. Repeated CSF examinations showed neither xanthochromia nor inflammation. Brain diffusion-weighted and FLAIR MR images revealed high intensity lesions in bilateral hemispheres. A cerebral angiography showed multifocal segmental stenosis of bilateral cerebral arteries. Four months later, follow-up angiography showed normalized flow in all cerebral arteries and we gave a diagnosis of reversible vasoconstriction syndrome (RCVS). She has had no symptoms and signs since the third attack of headache. RCVS is an important disease in the differential diagnosis of thunderclap headache without neurological deficit. This is the first report of RCVS triggered by Chinese herbal medicine.     

Pooled analysis of patients with thunderclap headache evaluated by CT and LP: Is angiography necessary in patients with negative evaluations?

BackgroundSevere, abrupt onset headache raises concern for aneurysmal subarachnoid hemorrhage (SAH). The current standard work-up is brain CT scan followed by LP if the CT is non-diagnostic in patients with a normal neurological exam. Some have suggested that angiography is also indicated in this common clinical situation. Is evaluation with brain CT and LP for thunderclap headache to rule out SAH sufficient and is angiography needed?MethodsWe systematically searched for studies that followed neurologically-intact patients with thunderclap headache and normal CT and LP for at least 1 year. The primary outcome was SAH. We estimated the proportion of patients who developed SAH and the one-sided upper 95% confidence bound.ResultsSeven studies including 813 patients were identified. None of the patients developed SAH during follow-up (pooled proportion = 0, upper 95% confidence bound = 0.004).ConclusionAlthough our methods have important limitations, we believe that this analysis will give clinicians better tools to decide whether or not to pursue further work-up with angiography in patients with thunderclap headache and normal neurological exam, CT, and LP.     

Postpartum RCVS and PRES with normal initial imaging findings

Postpartum angiopathy as clinical presentation of reversible cerebral vasoconstriction syndrome remains an intriguing phenomenon. The diagnosis needs to be considered in women presenting with thunderclap headache with or without associated neurological deficits. Here, we present a patient with thunderclap headache with initial normal laboratory and cerebral imaging findings, including intracranial angiography. Her condition worsened over several days and magnetic resonance imaging revealed changes initially compatible with posterior reversible encephalopathy syndrome, sequentially ischemic stroke and narrowing of the intracranial arteries. Although the patient was in coma for several days, she completely recovered and the focal vasoconstriction fully resolved. This case underscores the complex and variable presentation of postpartum angiopathy and illustrates the diagnosis to be (re)considered even if cerebral vasoconstriction is not documented at the onset of symptoms.     

Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical,imaging, and angiographic comparison

Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. However, prompt and accurate diagnosis remains challenging. Here we compared the features of 159 RCVS to 47 PACNS patients and developed criteria for prompt bedside diagnosis. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS‐spectrum disorders. In patients without TCH and positive angiography, neuroimaging can discriminate RCVS (no lesion) from PACNS (deep/brainstem infarcts). Ann Neurol 2016;79:882–894     

X线摄片在脑部疾病诊断中的应用

目的:探讨X线摄片技术在脑部疾病诊断和鉴别诊断中的应用价值。方法:报道1年内我科住院患者中见到的4例较典型病例,依赖CT和MRI误诊神经系统疾病。结果:4例病人分别诊断为面肌痉挛脑梗塞的paget′s病例,初诊意识障碍原因待诊-代谢性脑病?脑干梗死?的多发性骨髓瘤病例;诊断帕金森病的股骨颈骨折病例;诊断脑梗死的肺癌病例。结论:CT和MRI决不能替代以症状和体征为基础的临床诊断学,X线摄片技术在脑部疾病诊断和鉴别诊断仍具有重要价值。     

Wallenberg's syndrome in a case of Vogt-Koyanagi-Harada disease

A 45-year-old Japanese man, who had had bilateral visual disturbance due to Vogt-Koyanagi-Harada (VKH) disease 17 years before entry, was admitted to this hospital because of headache, vertigo and vomiting. On examination at entry, no abnormalities except for poliosis, patches of vitiligo on his left shin, sunset glow fundus, and positional nystagmus with Frenzel glasses were found. Laboratory data other than leukocytosis and elevated level of gamma-GTP were normal and the results of brain CT scan were within normal limits. On the following day, diplopia was developed and the neurological symptoms including loss of bilateral visual acuity, Horner's syndrome on the right side, right facial palsy, bilateral sensorineural hearing disturbance, palsy of the soft palate on the right side with swallowing difficulty, and dissociated sensory disturbance on the right face and the upper and lower extremities on the left side appeared with a few days. He couldn't get up. The cerebrospinal fluid (CSF) was clear and had pleocytosis with normal sugar content. The protein, immunoglobulin G and myelin basic protein (MBP) were elevated but the tests for oligoclonal band and antiviral antibodies were negative. Brain CT scan showed low density areas in right cerebellar hemisphere and in left putamen without abnormality with contrast material and evoked potentials were normal. Prednisolone was prescribed and his symptoms were subsided but his gait remained ataxic. Magnetic resonance imaging (MRI) 4 months later showed an atrophy of the lower half of bilateral cerebellar hemisphere supplied by posterior inferior cerebellar artery, suggesting cerebellar infarction, and high intensity areas on T2 image in bilateral cerebral white matters, basal ganglia, and left cerebral peduncle.(ABSTRACT TRUNCATED AT 250 WORDS)     

The CT pattern of a ruptured P3 posterior cerebral artery aneurysm

A 48-year-old male smoker with a history of significant alcohol use developed thunderclap headache during sexual intercourse. On neurological exam, he was confused and agitated with a Glasgow Coma Score of 11 (E3, M6, V2). Computed tomography (CT) of the brain revealed diffuse subarachnoid hemorrhage (SAH) with blood notably present in the quadrigeminal and supracerebellar cisterns (Figure 1). Cerebral angiogram demonstrated a distal P3 posterior cerebral artery (PCA) aneurysm (Figure 2). The PCA aneurysm was successfully treated with endovascular coil occlusion.     

Reversible cerebral vasoconstriction syndrome or primary angiitis of the central nervous system?

BACKGROUND: Reversible Cerebral Vasoconstriction Syndrome (RCVS) may present as thunderclap headache (TCH), accompanied by reversible cerebral vasospasm and focal neurological deficits, often without a clear precipitant. RCVS may be mistaken for Primary Angiitis of the Central Nervous System (PACNS) due to the presence of similar angiographic features of segmental narrowing of cerebral arteries. We discuss the clinical features of a young female migraine patient who developed TCH and was found to have RCVS following initial treatment with corticosteroids for PACNS, in the context of a systematic review of the available medical literature. METHODS: A Medline search was performed to identify all case reports since 1966 describing RCVS and PACNS that provide sufficient clinical detail to permit diagnostic classification according to published criteria. RCVS included case studies in which there was angiographic or transcranial Doppler ultrasound evidence of near-to-complete resolution of cerebral vasoconstriction in the absence of a well-recognized secondary cause. PACNS included reports of histologically confirmed PACNS either through biopsy or necropsy. RESULTS: Reversible Cerebral Vasoconstriction Syndrome occurs primarily in females and is characterized by sudden, severe headache at onset, normal CSF analysis, vasoconstriction involving the Circle of Willis and its immediate branches, and angiographic or TCD ultrasound evidence of near-to-complete vasospastic resolution within 1-4 weeks. It occurs typically in the context of vasoconstrictive drug use, the peripartum period, bathing, and physical exertion. CONCLUSION: Initial and follow-up (within 4 weeks) non-invasive angiographic studies are indicated in patients who present with TCH or who have clinical presentations that could be consistent with RCVS or PACNS in the absence of a well-recognized secondary cause, such as subarachnoid haemorrhage. Early reversibility of cerebral vasospasm is the key neuroradiological feature that supports the clinical diagnosis of RCVS.     

Severe Reversible Cerebral Vasoconstriction Syndrome with Large Posterior Cerebral Infarction

Reversible cerebral vasoconstriction syndrome is characterized by thunderclap headache and multifocal cerebral vasoconstriction. Cerebral vasoconstriction is reversible, and most cases have good prognosis. However, clinical outcome is possibly severe when it is complicated by stroke, yet detailed reports on such a case are few. We experienced a case of severe reversible cerebral vasoconstriction syndrome in a 32-year-old woman with medical history of preeclampsia 3years prior. She presented with sudden sharp headache followed by altered mental status and vasoconstriction of the bilateral posterior cerebral arteries. She was treated with intravenous and oral calcium channel blockers, edaravone, and glycerol. However, the cerebral infarction in the posterior circulation subsequently remained, and her impaired consciousness did not recover. Furthermore, although imaging findings of vasoconstriction showed improvement a day after the occurrence of symptom, the same vessels showed poor visualization 7 weeks later, which indicated the recurrence of vasoconstriction, without additional symptom due to the fixed infarction. Although most cases of reversible cerebral vasoconstriction syndrome show good prognosis, neurologists must monitor the possibility of worse clinical course and permanent neurological deficit when associated with stroke, such as cerebral infarction. Strict management and treatment are needed in these cases.     

An autopsy case of Neuro-Beh?et's disease with the right middle cerebral artery occlusion on cerebral angiogram

A case of Neuro-Beh?et's disease with the right cerebral artery occlusion on cerebral angiogram was reported. A 63 years old man complained of headache and slight fever without exacerbations of ocular and mucocutaneous lesions, 16 years after he had suffered from recurrent oral aphthous ulcers, genital ulcers, uveitis and erythema nodosum. Laboratory examination demonstrated pleocytosis in the cerebrospinal fluid, a low density area with contrast enhancement in right temporal and parietal lobes in brain CT, a high signal intensity in the same area in T2-weighted image in brain MRI and the occlusion of the right middle cerebral artery on cerebral angiogram. After admission, left homonymous hemianopsia and hemiparesis appeared. With steroid therapy, these symptoms diminished and abnormal findings in brain CT and MRI disappeared, but psychiatric symptoms were exacerbated gradually. Finally he died of agranulocytosis and pneumonia. Neuropathologic findings showed panarteritis of branches of the right middle cerebral artery and infarction of its territories in addition to perivascular infiltrations and foci of demyelination which were prominent in the cerebral basal regions.     

A case of bilateral cerebellar infarction in the distribution of the bilateral superior cerebellar artery]

We reported a case of bilateral cerebellar hemorrhagic infarction in the distribution of the bilateral superior cerebellar artery. A 58-year-old man suddenly developed dizziness and transient loss of consciousness. The neurological examination revealed left hearing disturbance, left sensory disturbance involving face, dysarthria and bilateral ataxia. This patient was considered to be classic clinical syndrome of right superior cerebellar artery. CT and MRI revealed hemorrhagic infarction corresponding to the full territory of the bilateral superior cerebellar artery. The right posterior cerebral artery was filling through the right posterior communicating artery on the right carotid angiography taken 2 hours after the onset. Bilateral vertebral angiography on the 18th day demonstrated no occlusions in the basilar artery and the bilateral superior cerebellar artery. Hemorrhagic infarction corresponding to the full territory of the bilateral superior cerebellar artery, sparing other territories as the present case, is extremely rare. In this case, cerebral embolism (top of the basilar syndrome) was suggested because of existence of atrial fibrillation and sudden onset.     

Delayed onset massive oedema and deterioration in traumatic brain injury.

A 52-year-old man fell from standing and a computed tomography (CT) scan revealed traumatic intracerebral haematoma and subarachnoid haemorrhage in the temporal cortex. He was treated without surgery and discharged. On day 30 after the accident, he had no neurological deficit. On day 37 he complained of headache and urinary incontinence, and on day 39 he was hospitalized due to progressive neurological deterioration (reduced conciousness, dilated pupils, and left hemiplegia). A CT scan revealed a diffuse low-density in the right cerebral hemisphere with marked midline shift. Emergency decompressive craniectomy and right temporal lobectomy were performed. Angiography after surgery revealed moderate vasospasm in the right middle and anterior cerebral arteries. The patient remained severely disabled. Delayed onset neurological deterioration can be caused by brain oedema and vasospasm after traumatic brain injury, despite an intervening period of improvement.