Reversible Cerebral Vasoconstriction Syndrome And Fibromuscular Dysplasia: An Epiphenomenon Or A Causal Relationship?

Fibromuscular dysplasia (FMD) is a rare non-atherosclerotic arterial disease that primarily affects middle-aged Caucasian women. Carotid web (CW) is a variant of FMD characterized by a nonatheromatous, membrane-like tissue protrusion into the carotid bulb. Reversible cerebral vasoconstriction syndrome (RCVS) is defined by severe headaches and reversible narrowing of cerebral arteries, which typically resolves within three months. While most RCVS cases have identifiable triggers, a significant portion occurs without known causes. Recent studies have reported a high prevalence of neurovascular abnormalities in RCVS patients. We present a case of a thirty-year-old woman with a sudden-onset severe headache, diagnosed with RCVS associated with carotid web. The patient had no ischemic involvement and responded well to flunarizine treatment. Follow-up imaging showed no stenosis. This case highlights a potential association between carotid web and RCVS, suggesting that FMD may contribute to vascular hyperreactivity and presents as a risk factor for RCVS. Further investigations are needed to understand the underlying mechanisms connecting these two vascular disorders. Keywords: reversible vasoconstriction syndrome; fibromuscular dysplasia; carotid web; structural abnormalities; vascular hyperreactivity.

Headache characteristics and postoperative course in Chiari I malformation.

BACKGROUND: Chiari I malformation typically presents with cough headache. However, migraine-like or tension-type-like headaches may also occur. There are limited publications on Chiari I malformation-associated headache semiologies and the effect of foramen magnum decompression on different headache types. METHODS: A retrospective analysis complemented by structured phone interviews was performed on 65 patients with Chiari I malformation, treated at our hospital between 2010 and 2021. Headache semiology (according to ICHD-3), frequency, intensity, and radiological characteristics were evaluated pre- and postoperatively. RESULTS: We included 65 patients. 38 patients were female and 27 male. Mean age was 43.9 ± 15.7 years. Headache was predominant in 41 patients (63.0%). Twenty-one patients had cough headache and 20 had atypical headache (12 migrainous, eight tension-type headache-like). Thirty-five patients with headache underwent surgery. Frequency, intensity, and analgesic use was significantly reduced in cough headache (p < 0.001). Atypical headaches improved less (p = 0.004 to 0.176). Exploratory analysis suggested that larger preoperative tonsillar descent correlated with larger postoperative headache intensity relief (p = 0.025). CONCLUSION: Decompression was effective in Chiari I malformation-related cough headache. Atypical headache responded less well, and the causal relation with Chiari I malformation remains uncertain. For atypical headache, decompression should only be considered after failed appropriate preventive therapy and within an interdisciplinary approach involving a neurologist.

Managing Headache Disorders Associated with Tuberous Sclerosis and Neurofibromatosis.

PURPOSE OF REVIEW: Tuberous sclerosis complex (TSC) and neurofibromatosis (NF) are neurocutaneous disorders often encountered by neurologists in clinical practice. This article aims to familiarize adult and pediatric neurologists with common features of these disorders and headache specific evaluation and management. RECENT FINDINGS: Non-malignant intracranial tumors in TSC include cortical tubers (glioneuronal hamartomas), subependymal nodules or subependymal giant-cell astrocytomas (SEGA). Headache disorders in TSC are largely secondary and can cause headaches due to increased intracranial pressure, mass effect, obstructive hydrocephalus, or hemorrhage. Neurosurgical intervention is typically required for management of large SEGAs; however, in patients with increased surgical risk, newer treatment modalities may be offered such as neoadjuvant therapy with an mTOR inhibitor (mTORi). Newer studies indicate headache disorders are more prevalent in neurofibromatosis type 1 (NF1). Primary headache disorders can include migraine and tension-type headache, while secondary headache disorders can be due to associated neoplasms such as optic pathway gliomas or brainstem gliomas, or less commonly vasculopathies such as moyamoya syndrome. Selumetinib is an oral, small molecule mitogen-activated protein kinase (MEK) agent with antineoplastic activity which is in ongoing trials for treatment of NF1-associated pediatric low-grade gliomas. NF1 stands out as having a higher association with primary headache disorders such as migraine. This association may be related to effects of mutation of the neurofibromin gene on pathways involved in pain and migraine genesis, however, warrants future study. Care should be taken when formulating a headache treatment plan to address comorbidities and avoid medications that may be contraindicated.

Neuro-Ophthalmic Symptoms of Primary Headache Disorders: Why the Patient With Headache May Present to Neuro-Ophthalmology.

BACKGROUND: Primary headache disorders can cause many ophthalmic symptoms that lead many patients to present for neuro-ophthalmic evaluation. Neuro-ophthalmologists frequently encounter these patients in clinical practice. EVIDENCE ACQUISITION: A literature review was completed in PubMed using the following terms paired with "migraine" and "headache:" dry eye, eye pain, monocular diplopia, binocular diplopia, photophobia, visual field defect, tunnel vision, floaters, amaurosis fugax, transient visual obscuration, autonomic symptoms, anisocoria, visual snow, Alice in Wonderland syndrome, and palinopsia. RESULTS: Patients with migraine experience a wide range of visual disturbances including aura and more complex perceptual abnormalities such as Alice in Wonderland syndrome and visual snow. Visual disturbances may consist of positive and/or negative phenomena and may be binocular or monocular. Migraine and other primary headache disorders can be associated with photophobia, eye pain, dry eye, autonomic features, and anisocoria. CONCLUSIONS: Patients with primary headache disorders may experience a wide range of visual and ophthalmic symptoms. An understanding of the typical features of these disorders allows providers to help patients find appropriate treatment without unnecessary testing and to recognize when atypical presentations require additional evaluation.

Primary angiitis of the CNS and reversible cerebral vasoconstriction syndrome: A comparative study.

OBJECTIVES: To further improve the distinction between primary angiitis of the CNS (PACNS) and reversible cerebral vasoconstriction syndrome (RCVS). METHODS: We compared 2 large French cohorts of patients with PACNS (n = 110, retrospectively and prospectively enrolled) and RCVS (n = 173, prospectively enrolled). RESULTS: Patients with RCVS were predominantly female (p < 0.0001), with migraines (p < 0.0001), and were more often exposed to vasoactive substances (p < 0.0001) or postpartum (p = 0.002) than patients with PACNS. Headache, especially thunderclap headache, was more frequent in RCVS (both p < 0.0001). Thunderclap headache was absent in only 6% of patients with RCVS and was mainly recurrent (87%) and provoked (77%) mostly by sexual intercourse, exertion, or emotion. All other neurologic symptoms (motor deficit, seizure, cognitive disorder, or vigilance impairment, all p < 0.0001) were more frequent in PACNS. At admission, brain CT or MRI was abnormal in all patients with PACNS and in 31% of patients with RCVS (p < 0.0001). Acute ischemic stroke was more frequent in PACNS than in RCVS (p < 0.0001). Although intracerebral hemorrhage was more frequent in PACNS (p = 0.006), subarachnoid hemorrhage and vasogenic edema predominated in RCVS (p = 0.04 and p = 0.01, respectively). Multiple small deep infarcts, extensive deep white matter lesions, tumor-like lesions, or multiple gadolinium-enhanced lesions were observed only in PACNS, whereas cervical artery dissection was found only in RCVS. CONCLUSIONS: Our study confirms that careful analysis of clinical context, headache features, and patterns of brain lesions can distinguish PACNS and RCVS within the first few days of admission in most cases. However, diagnosis remains challenging in a few cases.

Cerebral MRI and EEG studies in the initial management of pediatric headaches.

BACKGROUND AND STUDY PURPOSE: High resolution imaging modalities and electroencephalographic studies (EEG) are used in the assessment of children with headaches. We evaluated the role of cerebral MRI (cMRI) and EEG in the initial assessment of children with headache as the chief complaint of initial presentation. METHODS: A retrospective chart analysis was performed at a tertiary University Hospital. RESULTS: 209 patients were included in this study [mean age 11.3 years; male 91 (43.5%); female 118 (56.5%)]. The following types of headaches were seen: Unclassified headache: 23.4%; probable migraine 17.2%, migraine without aura 13.4%, complicated migraine 12.4%, migraine with aura 1.0%; tension-type 15.3%, and cluster headaches 0.5%, and secondary headaches 16.7%. In 93 children (44.5%) abnormal physical/neurological findings were noted (multiple entries possible). On cMRI studies the following findings were seen: Infection of sinuses (7.2%), pineal cysts (2.4%), arachnoidial cyst and Chiari malformation (1.9%), unspecified signal enhancement (1.0%), and pituitary enlargement, inflammatory lesion, angioma, cerebral ischaemia, and intra-cerebral cyst (each 0.5%). Electroencephalographic findings included both focal and generalised abnormal slowing (5.3%) and Spike-wave complexes (3.3%). CONCLUSIONS: Despite abnormal findings on neurological/physical examination in a substantial number of children with headaches, the yield of pathological cMRIs was low. The use of EEG recordings was not contributory to the diagnostic and therapeutic approach. More research is needed to better define those patients who are likely to have an intracranial pathology.

Painful temporomandibular disorders and headaches in 359 dental and medical students.

OBJECTIVE: to assess the prevalence of headaches and painful tempormandibular disorders (TMDs) and examine these via demographic and specific lifestyle parameters, as well as examine the relationship among TMDs, headaches, and depression rates. METHOD AND MATERIALS: a group of 359 medical and dental students completed a detailed questionnaire regarding demographic features and the presence of headaches and facial pain. The survey included a section on lifestyle (nutrition, alcohol and tobacco consumption, physical activity) and a Zung depression assessment. RESULTS: about eleven percent of the subjects reported pain: 8.6% from the jaw joint, 1.7% from the muscles of mastication, and 0.8% had both (ie, painful TMD). Eighty-three percent reported a lifetime prevalence of any headache, most of which were episodic tension-type headaches (56.9%) or migraines (19.2%). There was no significant correlation between headache diagnosis and the presence of painful TMD. Patients with painful TMD had higher depression scores than patients without and smoked more tobacco. This was not observed in headache patients. Patients with headache complained of significantly more dizziness and fatigue, particularly in the migraine and frequent episodic tension-type headache groups. CONCLUSION: TMD patients should be carefully assessed for the presence of emotional problems and referred to a suitable care provider.

Disorders presenting with headache as the sole symptom.

Headache is one of the commonest medical complaints, and ranks high among the reasons why people consult neurologists and general practitioners. Most headache patients are, however, managed suboptimally; and indeed, many neurologists find outpatient headache management one of the least engaging parts of their job. Headache may present as part of a symptom complex or it may present alone. When it is part of a complex, the total presentation of the patient serves as pointer to any underlying disease. When it is the sole symptom however, identifying the specific cause can be more difficult. Even though the diagnosis and management of most cases of headache probably do not require sophisticated neurological skills or investigations, failure to recognise an underlying disorder or an attitude of total neglect can be fatal. In this paper, we briefly review some of the disorders reported to have presented with headache as the sole symptom with the aim of drawing attention to the need for proper attitude to every headache complaint even when it initially appears to be trivial. Two groups of headache are recognised - primary and secondary. More than 90% of headaches seen in practice are of the primary type, which includes migraine, tension and cluster headaches. Secondary headache results from a wide range of disorders which may be intracranial, extracranial or systemic. Intracranial causes of headache include tumours, haematomas, infections, idiopathic intracranial hypertension and vascular disorders. Some of the more common extracranial and systemic causes are shown in Tables 1 and 2 respectively. Recognition of these conditions requires a standardised diagnostic approach to history and examination, wherein the patient's history alerts the physician while the physical examination provides support for the diagnosis.

Is vasospasm requisite for posterior leukoencephalopathy in patients with primary thunderclap headaches?

Primary thunderclap headache (TCH) is sometimes associated with cerebral vasospasm. However, the role of vasospasm in relation to the development of reversible or irreversible posterior leukoencephalopathy among patients with primary TCH has never been fully addressed. This paper includes a report on a 51-year-old woman with primary TCH complicated with posterior leukoencephalopathy and a literature review of 16 further patients with the same illness. Their magnetic resonance or conventional angiographic findings were clearly described. Our review found that all these 17 patients showed evidence of cerebral vasospasm. Eleven (65%) of them developed permanent ischaemic infarctions, almost exclusively located at the watershed zones. We suggest that the presence of vasospasm might be requisite for posterior leukoencephalopathy as well as for permanent infarctions in these patients. Therefore, searching for any clue of vasospasm is mandatory in treatment of patients with primary TCH. Absence of an accompanying vasospasm might predict a good outcome.

Primary headaches in obese patients

INTRODUÇÃO: Obesidade é um problema de saúde publica relevante em todo o mundo. Pouco se sabe sobre a prevalencia e impacto das cefaléias em pacientes obesos.OBJETIVO: Avaliar a prevalência de cefaléias primárias em pacientes obesos. MÉTODO: Setenta e quatro pacientes consecutivos originários de um centro de tratamento de obesidade foram estudados e comparados com controles com índices de massa corporal menores que 25. Resultados: Cinquenta e cinco pacientes (75%) apresentaram algum diagnóstico de cefaléia primária, 49 enxaqueca (66%), 7 cefaléia do tipo tensional (9%), 36 (48%) tiveram cefaléias incapacitantes. CONCLUSÃO: Cefaléias primárias são mais comuns e incapacitantes em pacientes obesos que controles, a enxaqueca é o diagnóstico mais freqüente nesta população. Cefaléias devem ser adequadamente diagnosticadas e tratadas em pacientes obesos.

Primary headaches in obese patients.

BACKGROUND AND OBJECTIVE: Obesity is a major public health problem worldwide. Little is known about the prevalence and impact of headache disorders in obese patients. The objective of this study was to assess the prevalence of primary headaches in obese patients and controls. METHOD: Seventy-four consecutive obese patients from the obesity surgery service were studied, and compared to controls with body mass index less than 25. RESULTS: Fifty-six patients (75%) had a headache diagnosis, 49 migraine (66%), 7 tension-type headache (9%), 36 (48%) had incapacitating headaches. CONCLUSION: Primary headaches are more common and incapacitating in obese patients than controls, migraine is the most important diagnosis in this population. Headaches should be properly diagnosed and treated in obese patients.