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Thunderclap headache as a first manifestation of acute disseminated encephalomyelitis: case report and literature review

BMC Neurology volume 24, Article number: 315 (2024) Cite this article

Abstract

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.

Case

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.

Conclusion

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.

Peer Review reports

Background: acute disseminated encephalomyelitis (ADEM)

Acute Disseminated Encephalomyelitis (ADEM) is an acute immune-mediated polyfocal demyelinating disorder of the central nervous system. It may occur at any age but is most common during childhood and occurs in 67% of the cases after an infection or vaccination [1,2,3,4]. Patients usually present with (sub)acute onset of encephalopathy in combination with polyfocal neurologic deficits. Seizures, fever, gradual headache, meningeal signs and vomiting may occur [2,3,4,5]. The clinical course is rapidly progressive with maximization of deficits in a couple of days [1]. Pathophysiology is not fully understood but the proposed mechanism is T- cell-mediated cross-activation and response against myelin proteins through a mechanism of molecular mimicry [2,3,4]. Since there is no distinct biological marker, the diagnosis is based on historical, clinical and radiological features. MRI with T2 and Fluid Attenuated Inversion Recovery (FLAIR) sequences can reveal multiple large and poorly demarcated lesions predominantly in the white matter [2,3,4]. The differential diagnosis includes multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), infectious meningoencephalitis, central nervous system vasculitis or malignancy [4]. Cerebrospinal fluid (CSF) analysis is mainly used for exclusion of other infectious diseases, as the possible findings (i.e. pleocytosis with lymphocyte domination and protein elevation) are nonspecific as well [4]. Pathological hallmarks are congestion and swelling with perivenous sleeves of demyelination at a uniform stage (in contrast to MS) and perivascular inflammation [6]. High-dose intravenous corticosteroids followed by prolonged oral tapering over 4–6 weeks is administered as an acute treatment. Alternative options are intravenous immunoglobulins (IVIG) and plasma exchange [4]. Full recovery is seen in 50–75% of cases [3, 4, 7] but mortality rate reaches 6.1% in adults [1].

Thunderclap headache (TCH) as a first clinical manifestation of ADEM has not been described before. Significant intracranial hypertension in ADEM is rarely reported as well. We present a case which combines both and form a hypothesis regarding the pathophysiology.

Case report

A 62-year-old woman admitted to the emergency department with a holocranial thunderclap headache and a subjective binocular vertical diplopia existing for more than 24 h. There was no meningeal irritation or photophobia. She reported having a persisting bronchitis for 16 days and was on a second course of amoxicillin/clavulanic acid, prescribed by her general practitioner. There was no history of travelling or recent vaccinations and she never had fever. Neurological examination was normal with no ophtalmoparesis. Computed tomography (CT), CT angiography (CTA) of the brain and blood analysis were unremarkable. Lumbar puncture showed an opening pressure of 15mmHg/20cmH2O. Analysis of the cerebrospinal fluid (CSF) revealed increased cellularity with a white blood cell count of 330/ul (with segment 88%, lymphocytes 10% and monocytes 2%), a protein count of 81 mg/dl, glucose level of 62 mg/dl and was negative for intrathecal oligoclonal bands or xanthochromia. Cultures were negative for bacteria and fungi, Polymerase Chain Reaction (PCR) was negative for bacteria (E.Coli, H. Influenzae, L. onocytogenes, N. Meningitidis, S. Agalactiae, S. Pneumoniae) and viruses (Cytomegalovirus, Enterovirus, Herpes simplex virus 1 and 2, Humane herpesvirus 6, Humane parechovirus, Varizella Zoster virus). A masked bacterial meningitis, under a second course of antibiotics, was suspected and the patient was hospitalized with IV ceftriaxone 2 g once a day, Acyclovir 750 mg three times a day and Ampicillin 2 g IV every four hours. The next morning, the patient acutely deteriorated to a subcomatose state with a Glasgow Coma Scale of 11 (eye:3, motor:6, verbal:2) without lateralizing motor or sensory signs. Urgent magnetic resonance imaging (MRI) of the brain showed significant bilateral supra- and infratentorial diffuse white matter hyperintensities on T2 and FLAIR with beginning associated edema (Fig. 1). The periaqueductal grey (PAG) and trigeminal nuclei were affected due to the inflammation. Acute Disseminated Encephalomyelitis (ADEM) was suggested considering the preceding upper respiratory tract infection. Broad infectious serology (including interferon-gamma release assay, human immunodeficiency virus, Borrelia, varicella zoster, Epstein–Barr virus, herpes simplex type 1/2, cytomegalovirus, syphilis, cryptococcus, West Nile and Toxoplasmosis) and neuroinflammatory analysis, including myelin oligodendrocyte glycoprotein (MOG) antibodies and anti-aquaporin 4 (AQP4) antibodies, antibodies for autoimmune encephalitis and paraneoplastic syndromes in serum and CSF were negative. Repeated CSF cytology and flow cytometry could not show malignant cells. Rheumatological and nutritional screening were negative. infections () were negative. A 18-FDG-PET-CT full body did not demonstrate any inflammatory or neoplastic lesions.

Fig. 1
figure 1

MRI (T2 FLAIR) showing diffuse white matter hyperintensities with diffuse edema

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High dose IV methylprednisolone (1000 mg/d) was initiated. Regardless, on day 2 of the treatment, the patient had further deteriorated to a comatose state (EMV 2-4-2) with equal and reactive pupils. Obstructive breathing with respiratory failure was evident, with need for intubation and mechanical ventilation. An urgent control CT scan showed progressive white matter abnormalities and edema compressing the basal cisterns and fourth ventricle. Intracranial hypertension was suspected and an external ventricular drain (EVD) was placed for invasive neuromonitoring and CSF drainage. Despite continuous sedation and classical measures such as 30 degrees elevation of the head and hypertonic saline, the ICP was above 20 mmHg for significant periods (Fig. 2). Additional curarisation and sedation was necessary for the following first weeks due to ICP spikes above 30 mmHg.

Fig. 2
figure 2

Graphical overview of ICP spikes on the ICU

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After 7 days of high dose corticosteroids (five days methylprednisolone 1000 mg/d followed by 125 mg/d) there was a lack of clinical and radiological response and persisting elevated ICP. Therefore, second line treatment with cyclophosphamide and alternate day plasma exchange (PLEX) were simultaneously initiated. After six sessions of PLEX, ICP stabilization with reduced need for sedation and a slow neurological recuperation was achieved. Follow-up imaging showed progressive decrease in T2/FLAIR white matter hyperintensities supra- and infratentorial. Four and a half months after presentation, the patient could leave the hospital with complete neurological recuperation. There was no relapse after six months of clinical and radiological follow-up (Fig. 3). Repeated analysis of anti-MOG and-AQP4 antibodies in serum remained negative.

Fig. 3
figure 3

MRI (T2/FLAIR) after 3.5 months showing progressive resolution

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Literature search

The literature search was carried out on PubMed (Table 1) and restricted to studies published in English. There was no limit on publication date for referencing articles about association between ADEM, intracranial hypertension or thunderclap headache, or headache in other demyelinating diseases/multiple sclerosis (MS).

Table 1 Search overview
Full size table

All the available ADEM articles were screened for items related to increased intracranial pressure, opening pressure or neurosurgery in the title (search 1). The same was applied for more specific searches concerning demyelinating diseases (searches 2 and 3). No articles focusing on TCH and ADEM (search 4) were found. A further search was performed (search 4) focusing on articles about TCH and screening for anything related to ADEM or demyelination in the text. A total of 122 articles concerning TCH were found, from which 85 articles were selected after screening the abstract. An analysis of the selected literature, including > 30 reviews, was performed to search for causes of TCH, with focus on ADEM or demyelinating disorders. The same was applied for demyelinating diseases and headache (searches 5–8).

Discussion

Increased intracranial pressure (ICP) or intracranial hypertension in ADEM

Increased ICP is a complication of a variety of central nervous system pathologies, most commonly space-occupying lesions and inflammation. It is defined as a cerebrospinal fluid (CSF) pressure higher than 20 cm H2O or 15mmHg (8). In ADEM, intracranial hypertension has been sporadically described both in adults and children, with only a minimal amount of documented articles [9,10,11,12,13,14,15,16,17,18,19,20]. Since the presenting signs and symptoms of ADEM such as headache, vomiting and encephalopathy are also the main manifestations of intracranial hypertension, these are no useful clinical tools for distinguishing between ADEM with or without increased ICP [10]. ADEM with intracranial hypertension is significantly associated with increased morbidity, prolonged hospitalization, ICU admission, edema and/or herniation requiring neurosurgical management [9]. A retrospective cohort study showed increased cerebrospinal fluid opening pressure in 42% of pediatric patients with ADEM [10]. While none of these patients needed additional measures or pressure management, it suggests that intracranial hypertension is more common in ADEM than documented. Limited data is available about intracranial hypertension in other demyelinating diseases [12, 21]. Comparable ICP’s between variable demyelinating diseases and idiopathic intracranial hypertension in children has been described [12]. A few reports associate intracranial hypertension with multiple sclerosis (MS), while others claim the opposite [22,23,24,25,26]. There are some theories for the raised ICP in ADEM or demyelinating diseases. The extensive encephalomyelitis or demyelinating lesions can cause diffuse vasogenic edema [24]. The changes in the CSF composition (increased cytokines, immunoglobulins and leukocytes) can alter the CSF flow dynamics and increase the oncotic pressure. Combined with the edema, this might prevent adequate CSF reabsorption at arachnoid villi and cause hydrocephalus [12, 21, 24].

In very rare cases, ADEM can lead to significant ICP rises requiring decompressive neurosurgery with favorable outcomes on mortality [14,15,16, 18,19,20]. Functional outcome has yet to be shown in prospective studies.

In our case, the intracranial hypertension was probably due to diffuse inflammation and edema, peaking 7–15 days after onset. There was no hydrocephalus. Imaging correlated accurately to the clinical state of the patient, further supporting this theory (Fig. 4).

Fig. 4
figure 4

Consequent MRI scans 3, 10 and 17 days after onset

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Thunderclap headache (TCH) and ADEM

TCH is defined by the International Classification of Headache Disorders (ICHD-3) as a sudden severe headache reaching maximum intensity within one minute and lasting for more than five minutes [27]. Next to primary thunderclap headache, it is associated with a nonlimited series of intracranial disorders: subarachnoid hemorrhage, reversible cerebral vasoconstrictive syndrome, cervical artery dissection, venous thrombosis, unruptured aneurysm, posterior reversible encephalopathy syndrome, hypertensive encephalopathy, spontaneous intracranial hypotension, ischemic and hemorrhagic stroke, intracranial mass lesions, meningoencephalitis, vasculitis (giant cell arteritis), pituitary apoplexy, acute glaucoma, carbon monoxide poisoning, colloid cyst, acute sinusitis and even myocardial infarction [28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,102,103,104,105,106,107,108,109,110,111,112]. Neither ADEM nor demyelination has been reported as a differential diagnosis.

In an effort to explain the TCH as the presenting manifestation of ADEM, a search for headache in demyelinating diseases was conducted. A significantly increased prevalence of trigeminal neuralgia in MS patients is reported with lifetime prevalence ranging from 4.0 to 9.5%, in comparison to 0.16–0.3% in the general population. Demyelinating lesions of the trigeminal nucleus or tracti in the brainstem are considered as causative [113, 114]. Cluster headache and glossopharyngeal neuralgia have been sporadically reported as well, again with brainstem lesions as presumed mechanisms [113, 115,116,117,118]. The prevalence of other headache syndromes in MS has been reviewed multiple times, showing highly variable numbers between 4.0 and 78%, with migraine and tension-type headache (TTH) being the most common [113, 114, 119,120,121,122,123,124,125,126,127,128,129,130,131,132,133]. Migraine was found to be more frequent in relapsing-remitting MS (RR-MS) and TTH more prevalent in progressive MS [134]. Further elaboration has been described in RR-MS, with more stabbing headache during a relapse and more a migraine type headache or TTH during remission [119, 135, 136]. Although without a clear causative correlation, headache as the first clinical manifestation of cerebral MS has been reported as well [126, 137, 138]. It is important to mention that there are case-control studies that could not show a difference in 1 year prevalence of migraine and TTH between MS and normal population [139, 140].

One theory of headache in demyelinating disorders suggests that the inflammation is not limited to the white matter, but also extends to the gyri and meninges [113, 126, 128, 135]. Demyelination in the pain-producing centers in the midbrain is another explanation where a plaque in the PAG has shown to be associated with a higher risk of developing migraine [113, 129, 134, 141,142,143]. Another significant part of headache in MS is iatrogenic due to side effects of medication such as interferons, natalizumab and fingolimod. Medication overuse headache is also reported [113, 119, 120, 127, 131, 132, 144]. In our case, we have no radiological evidence for hydrocephalus or mass effect at the initial presentation, excluding these as explanation for TCH as the initial presentation. Hereby, brain edema only developed three days after the TCH, making meningeal reaction or pressure on vasculature with stimulation of nociceptors also less probable. We assume excessive demyelinating inflammation in the brainstem anatomically both impacting the PAG and the trigeminal nuclei as a plausible explanation.

Conclusion

TCH as a first clinical manifestation of ADEM has not been described before. Significant intracranial hypertension in ADEM is rarely reported as well. We present a case which combines both, which seem to be caused by excessive demyelinating inflammation. The delayed intracranial hypertension was probably due to diffuse inflammation leading to edema. We assume the presentation with TCH was due to acute inflammation of the periaqueductal grey and trigeminal nuclei. The patient made a significant recovery after corticosteroids, plasma exchange, cyclophosphamide and cerebrospinal fluid drainage. In this report, we review the correlation of TCH and intracranial hypertension in ADEM.

Key findings

  • ADEM should be in differential diagnosis of patients presenting with thunderclap headache.

  • Acute inflammation in the brainstem anatomically both impacting the PAG and the trigeminal nuclei as a plausible explanation for thunderclap headache in a demyelinating disorder.

Data availability

The authors have no unpublished data to report.

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  1. A. Ciçek and L. De Temmerman contributed equally to this work.

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  1. Department of Neurology, Delta General Hospital, Roeselare, Belgium

    Abdulhamid Ciçek, Louise De Temmerman, Mieke De Weweire, Hilde De Backer, Maarten Buyle & Frederik Clement

  2. Department of Neurosurgery, Delta General Hospital, Roeselare, Belgium

    Abdulhamid Ciçek

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Abdulhamid Ciçek conceived the presented manuscript. Abdulhamid Ciçek and Louise De Temmerman did the literature review and wrote the manuscript. The total contribution of Abdulhamid Ciçek and Louise De Temmerman was equal. Frederik Clement and Maarten Buyle reviewed the article with comments. Mieke De Weweire and Hilde De Backer reviewed the article and had no additional comments.

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Ciçek, A., De Temmerman, L., De Weweire, M. et al. Thunderclap headache as a first manifestation of acute disseminated encephalomyelitis: case report and literature review. BMC Neurol 24, 315 (2024). https://doi.org/10.1186/s12883-024-03803-z

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