BACKGROUND: Acute Disseminated Encephalomyelitis (ADEM) is an acute demyelinating disorder of the central nervous system, characterize by multiple white matter hyperintensities on T2 MRI. Patients usually present with subacute progressive encephalopathy and polyfocal neurological deficits. Possible treatments are corticosteroids, immunoglobulins and plasma exchange. Full clinical recovery is seen in more than half of the cases.
METHODS: We describe a case of a 62-year-old patient presenting with thunderclap headache as the first symptom, two weeks after an upper respiratory tract infection. The clinical course was complicated by progressive coma and intracranial hypertension mandating external ventricular drainage and sedation. Initial treatment with methylprednisolone was unsuccessful but clinical resolution and radiological regression was achieved after plasma exchanges and cyclophosphamide.
CONCLUSIONS: To our knowledge, this is the first reported case of ADEM presenting with thunderclap headache. Intracranial hypertension with the need for invasive neuromonitoring and pressure management is also a very rare complication of ADEM. In this report, we describe the findings of the literature review concerning ADEM, thunderclap headache and intracranial hypertension.

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To report the efficacy of onabotulinumtoxinA (BoNTA) injections in relieving pain in patients with primary trochlear headache (PRTH).
Examination of medical records for patients diagnosed with PRTH according to the International Classification of Headache Disorders, 3rd edition criteria and treated with BoNTA. Data were collected for variables related to pain relief, duration of effectiveness, and adverse effects.
Six patients were included in the study. All had previously undergone standard care interventions, including infiltrations or oral treatments, yet experienced treatment failure or symptom recurrence. All patients received 20 units of BoNTA, administered in the corrugator and procerus muscles. Subsequent to the BoNTA injections, all six patients reported substantial pain relief, with five achieving complete remission of symptoms. The analgesic effect persisted for a duration of 3 months. No adverse events were reported in any of the cases.
Our case series presents the first evidence of the potential of BoNTA as a safe and effective treatment option for PRTH. From a clinical standpoint, having a safer alternative is of paramount significance for patients with limited treatment options, such as those with PRTH. Further research is warranted to validate these findings and explore the long-term efficacy of BoNTA in PRTH management.

Reversible Cerebral Vasoconstriction Syndrome clinically presents as severe headaches with or without neurological deficits accompanied by multilocal caliber variation of the cerebral arteries on imaging studies. Transient Global Amnesia is a benign neurological condition that implies sudden temporary antero- and retrograde amnesia. The exact pathophysiological mechanisms involved in transient global amnesia and reversible cerebral vasoconstriction syndrome remain unclear but suggest similar pathways as both can be triggered by factors that activate the sympathetic nervous system. We herein discuss a potential relationship of the two conditions in a 65-year-old woman that initially presented herself to the emergency department with temporary memory impairment, indicating Transient Global Amnesia. Four days later, the patient revealed a thunderclap headache accompanied by a subarachnoid hemorrhage with transient segmental narrowing of the arteries of the anterior circulation on neuroimaging. In this case report we hypothesize that Reversible Cerebral Vasoconstriction Syndrome might be a potential cause for the clinical symptoms and imaging patterns with Transient Global Amnesia as a possible prodromal stage of Reversible Cerebral Vasoconstriction Syndrome.

BACKGROUND: Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden onset thunderclap headache and multiple segmental reversible cerebral vasoconstrictions that improve within 3 months. The postpartum period is a well-known precipitating factor for the onset of RCVS. Cerebral venous thrombosis (CVT) causes thunderclap headaches in the postpartum period. While headache in CVT is sometimes exacerbated in the supine position, the severity of the headache in RCVS is usually independent of body position. In this study, we report a case of RCVS with thunderclap headache exacerbated in the supine position, and headache attacks that resolved quickly in the standing position during the postpartum period.
METHODS: A 33-year-old woman presented with a sudden increase in blood pressure and thunderclap headache on the fifth postpartum day (day 1: the first sick day). The headache was severe and pulsatile, with onset in the supine position in bed, and peaked at approximately 10 s. It was accompanied by nausea and chills but there were no scintillating scotomas or ophthalmic symptoms. The headache resolved in the standing or sitting position but was exacerbated and became unbearable within a few seconds when the patient was in the supine position. Therefore, she was unable to lie supine at night. Computed tomography angiography (CTA) of the head on day 2 and magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) on day 3 showed no abnormalities. However, considering the possibility of RCVS, verapamil was initiated on day 3. The headache resolved the following day. MRA of the head on day 10 revealed diffuse and segmental stenoses in the bilateral middle and posterior cerebral arteries and basilar artery. Therefore, the patient was diagnosed with RCVS. The headache gradually resolved and disappeared completely on day 42. Cerebral vasoconstriction was also improved on MRA on day 43.
CONCLUSIONS: This postpartum RCVS case was notable for the exacerbation of headaches in the supine position. For the diagnosis of thunderclap headache in the postpartum period, RCVS should be considered in addition to CVT when the patient presents with a headache that is exacerbated in the supine position.

BACKGROUND: Bath-related headache (BRH) is a rare primary headache disorder with only about 50 cases reported from 2000 to 2017 and none since. It is an abrupt onset excruciating headache occurring predominantly in middle-aged Asian women, most commonly following exposure to hot water. This is the first report in a Sri Lankan woman.
METHODS: A 60-year-old Sri Lankan woman presented with an abrupt onset, severe throbbing holocephalic headache immediately following a hot-water shower. The headache was not associated with photo- or phonophobia, nausea, or vomiting, and she did not report a past history of migraine. However, she had experienced a similar headache 2 years previously precipitated by a hot-water shower. Her neurological examination, blood investigations, and magnetic resonance imaging of brain and intracranial vessels were normal. She was treated with opioid and nonsteroidal antiinflammatory drug analgesics, but the headache resolved only after treatment with nimodipine. The headache did not recur during a follow-up of 2 years since she avoided hot-water showers.
CONCLUSIONS: Bath-related headache is a thunderclap primary headache disorder with a benign prognosis, but its recognition requires awareness to differentiate it from subarachnoid hemorrhage. It warrants inclusion in the International Classification of Headache Disorders.

Reversible cerebral vasoconstriction syndrome (RCVS) is a disease characterized by reversible multifocal narrowing of the cerebral arteries with clinical manifestations that typically include thunderclap headache and occasionally brain edema, stroke, or seizure. The exact pathophysiology of RCVS is not well known.
A 46-year-old female with history of episodic migraine presented with 1-month duration of worsening headaches that had become more severe over the past 2 weeks. The headaches were episodic and thunderclap in onset and aggravated by physical exertion or emotional situations. A neurological examination was unremarkable including initial head computed tomography (CT). A CT angiogram of the head showed multifocal stenosis in the right anterior cerebral artery, bilateral middle cerebral arteries, and right posterior cerebral artery. Cerebral angiogram confirmed the CT angiogram findings. A repeated CT angiogram a few days later showed improvement in the multifocal cerebral arterial stenosis. Lumbar puncture and autoimmune workup were not suggestive of neuroinflammatory etiology. She had one generalized tonic-clonic seizure during her second day of hospitalization. The patient\'s thunderclap onset headaches resolved in 1 week after she was managed with blood pressure control and pain medication. She denied any illicit drug use or any new medications other than the placement of a levonorgestrel-releasing intrauterine device (IUD) about 6 weeks prior to her presentation.
Our case suggests a possible link between RCVS and levonorgestrel-releasing IUDs.

A 71-year-old man had persistent cervical pain secondary to thunderclap headache during sleep. MRI conducted the next morning revealed subdural hematoma and convexity subdural hemorrhage on the right occipital region, and the patient was hospitalized. MRA showed vascular narrowing in the bilateral PCA. Follow-up MRA on day 8 of admission showed aggravated vascular narrowing of PCA, indicative of reversible cerebral vasoconstriction syndrome (RCVS). The patient was treated with a calcium-channel antagonist. Post-discharge MRA showed improvement of PCA narrowing, and the diagnosis of RCVS was confirmed.

OBJECTIVE: This retrospective case series study aimed to investigate the demographic and clinical patterns of primary stabbing headache (PSH). In addition, we tried to identify subgroups of treatment responses in a neurology outpatient consultation at a Portuguese tertiary hospital.
METHODS: Clinical records were retrospectively reviewed and patients meeting the International Classification of Headache Disorders, 3rd edition, criteria for PSH were identified from January 2014 to December 2020. We collected data regarding demographic characteristics, clinical features of the headache, primary headache comorbidities, and information about treatment-related do PSH.
RESULTS: Of 1857 patients, 32 (1.7%; mean [SD] age of onset 56 [3.5] years) had the final diagnosis of PSH. Regarding headache characteristics, 20 patients (62.5%) reported episodes of stabbing in fixed locations and 12 (37.5%) in multiple areas; the duration of each attack was between ≤5 s (seven [21.9%]), 5-60 s (20 [62.5%]), and ≥60 s (five [15.6%]). In all, 18 patients (56.3%) had an episodic course (vs. six of 32 [18.8%] an acute course and eight of 32 [25%] a chronic course). In all, 17 patients started medical treatment (53.1%), with total or partial improvement in 10 (58.8%) of them. It was found that patients with pain in fixed locations had a better response to treatment when compared to patients with multiple locations, in a statistically significant way (eight of 11 vs. two of six, p = 0.023).
CONCLUSIONS: In our sample, the mean age of onset of PSH was >50 years and there was a wide range of PSH duration. The duration of each attack (>5 s), the pain in fixed locations, non-daily episodes of the pain in each attack, and the intermittent course of headache were the most prevalent clinical features. Finally, patients with stabbing in localized areas had a better response to treatment.

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