Incidental Findings and Normal Anatomical Variants on Brain MRI in Children for Primary Headaches.

When MRI scans of the brain are obtained for evaluation of primary headaches in children, incidental findings and anatomical variants are commonly present. After a review of the prevalence, 11 types are presented.

Case report of hypnic headache: a rare headache disorder with nocturnal symptoms.

BACKGROUND: Headache is one of the commonest complaints reported to physicians worldwide. Yet, arriving at the proper diagnosis can be a challenge in many patients. Although most headaches belong to common categories of migraine and tension-type headache, which are diagnosed and managed relatively easily, several uncommon headache disorders can lead to delays in diagnosis. Certain medications are more efficacious than others in managing these headache disorders, hence establishing the correct diagnosis is of paramount importance. CASE PRESENTATION: An 86-year-old female presented with chronic daily headache of 1 year duration. Her headaches were exclusively nocturnal and woke her up daily around midnight. Clinical examination was unremarkable. All basic investigations were normal. Subsequent gadolinium enhanced Magnetic Resonance Imaging (MRI) brain did not show any significant pathology. There was no satisfactory response to paracetamol, diclofenac sodium, mefenamic acid, tramadol, flunarizine and sodium valproate. Indomethacin was started with the provisional diagnosis of hypnic headache. There was absolute response by day 3 of indomethacin. She remains headache free on low dose indomethacin maintenance at 1 year after the diagnosis. CONCLUSION: Better understanding of uncommon headache syndromes can help in early diagnosis and appropriate treatment. Hypnic headache should be considered in the differential diagnosis of chronic daily headaches, especially when nocturnal and occurs during sleep.

The Headache That Changed History: The Most Famous Thunderclap Headache.

BACKGROUND: Franklin Delano Roosevelt's fatal brain hemorrhage on April 12, 1945 provides an excellent platform for the study of the potential causes of one of the most famous headaches in history. IMPRESSION: The differential diagnosis of thunderclap headache, including hypertension and melanoma, is discussed, as are Roosevelt's health problems in the context of how serious diseases of our leaders can have a tangible effect upon historical interpretation. DISCUSSION: A large body of evidence is presented in support of a probable cover-up of Roosevelt's health problems, perpetrated by his doctors and close confidantes. The necessity of challenging conventional thinking about the health of our leaders and the fundamental importance of understanding their health issues is also emphasized.

Hypnic Headache and Basilar Artery Dolichoectasia.

BACKGROUND: Hypnic headache is regarded by most authors as a primary headache, but in some cases it was considered secondary to hypophysis, brain stem, or posterior fossa lesions. CASE: We report a case of a 69-year-old woman with a history of a headache that fulfils all International Classification of Headache Disorders-II criteria for hypnic headache, with 2 years of evolution, whose complementary investigation with magnetic resonance and magnetic resonance angiography revealed a dolichoectasia of the basilary artery. She was treated with verapamil with excellent response. CONCLUSIONS: To the best of our knowledge, this is the first described case of a patient with hypnic headache and a dolichoectasic basilar artery. The relation between these 2 entities is questionable, but we raise some possible explanations for this relationship.

Biochemistry of primary headaches: role of tyrosine and tryptophan metabolism.

The pathogenesis of migraine as well as cluster headache (CH) is yet a debated question. In this review, we discuss the possible role of the of tyrosine and tryptophan metabolism in the pathogenesis of these primary headaches. These include the abnormalities in the synthesis of neurotransmitters: high level of DA, low level of NE and very elevated levels of octopamine and synephrine (neuromodulators) in plasma of episodic migraine without aura and CH patients. We hypothesize that the imbalance between the levels of neurotransmitters and elusive amines synthesis is due to a metabolic shift directing tyrosine toward an increased decarboxylase and reduced hydroxylase enzyme activities. The metabolic shift of the tyrosine is favored by a state of neuronal hyperexcitability and a reduced mitochondrial activity present in migraine. In addition we present biochemical studies performed in chronic migraine and chronic tension-type headache patients to verify if the same anomalies of the tyrosine and tryptophan metabolism are present in these primary headaches and, if so, their possible role in the chronicity process of CM and CTTH. The results show that important abnormalities of tyrosine metabolism are present only in CM patients (very high plasma levels of DA, NE and tryptamine). Tryptamine plasma levels were found significantly lower in both CM and CTTH patients. In view of this, we propose that migraine and, possibly, CH attacks derive from neurotransmitter and neuromodulator metabolic abnormalities in a hyperexcitable and hypoenergetic brain that spread from the frontal lobe, downstream, resulting in abnormally activated nuclei of the pain matrix. The low tryptamine plasma levels found in CM and CTTH patients suggest that these two primary chronic headaches are characterized by a common insufficient serotoninergic control of the pain threshold.

Carotidinia, a vueltas con una vieja controversia / Carotidynia, back to an old controversial issue

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Diagnostics to look beyond the normal appearing brain tissue (NABT)? A neuroimaging study of patients with primary headache and NABT using magnetization transfer imaging and diffusion magnetic resonance.

OBJECTIVE: Novel diagnostics can allow us to "look beyond" normal-appearing brain tissue (NABT) to unravel subtle alterations pertinent to the pathophysiology of primary headache, one of the most common complaints of patients who present to their physician across the medical specialties. Using both magnetization transfer imaging (MTI) and diffusion weighted imaging (DWI), we assessed the putative microstructural changes in patients with primary headache who display the NABT on conventional magnetic resonance imaging (conventional MRI). METHODS: Subjects were 53 consecutive patients with primary headache disorders (40 = migraine with aura; 9 = tension headache; 4 = cluster headache) and 20 sex- and age-matched healthy volunteers. All subjects underwent evaluation with MRI, MTI, and DWI in order to measure the magnetization transfer ratio (MTR) and the apparent diffusion coefficient (ADC), respectively, in eight and six different regions of interest (ROIs). RESULTS: Compared to healthy controls, we found a significant 4.3 % increase in the average ADC value of the occipital white matter in the full sample of patients (p = 0.035) and in patients with migraine (p = 0.046). MTR values did not differ significantly in ROIs between patients and healthy controls (p > 0.05). CONCLUSIONS: The present study lends evidence, for the first time to the best of our knowledge, for a statistically significant microstructural change in the occipital lobes, as measured by ADC, in patients with primary headache who exhibit a NABT on MRI. Importantly, future longitudinal mechanistic clinical studies of primary headache (e.g., vis-à-vis neuroimaging biomarkers) would be well served by characterizing, via DWI, occipital white matter microstructural changes to decipher their broader biological significance.

Sinus venous stenosis, intracranial hypertension and progression of primary headaches.

The recently advanced hypothesis that idiopathic intracranial hypertension without papilledema (IIHWOP) is a powerful risk factor for the progression of pain in individuals prone to episodic primary headache implies that IIHWOP is much more prevalent than it is believed to be in the general population and that it can run almost asymptomatic in most of the affected individuals. In this review, we discuss the evidence available supporting that: (a) sinus venous stenosis-associated IIHWOP is much more prevalent than believed in the general population and can run without symptoms or signs of raised intracranial pressure in most of individuals affected, (b) sinus venous stenosis is a very sensitive and specific predictor of intermittent or continuous idiopathic intracranial hypertension with or without papilledema, even in asymptomatic individuals, (c) in primary headache prone individuals, a comorbidity with a hidden stenosis-associated IIHWOP represents a very common, although largely underestimated, modifiable risk factor for the progression and refractoriness of headache.

Surgical treatment of primary headaches.

Neuromodulation for the treatment of drug-refractory cranial neuralgias constitutes an exciting field of research for physicians; in the last decade, several methodologies have been described which could help many patients to exit such desperate conditions; although the exact mechanisms of action of these techniques are still matter of debate, several experimental and neuroradiological modalities can help us to get near the concept of understanding them. In this paper, the authors summarize the most recent surgical procedures used to treat severe and pharmaco-resistant cranial painful conditions, along with brief descriptions of the results obtained in the several published so far.

Magnitude, impact, and stability of primary headache subtypes: 30 year prospective Swiss cohort study.

OBJECTIVE: To determine the prevalence, impact, and stability of different subtypes of headache in a 30 year prospective follow-up study of a general population sample. DESIGN: Prospective cohort study. SETTING: Canton of Zurich, Switzerland. PARTICIPANTS: 591 people aged 19-20 from a cohort of 4547 residents of Zurich, Switzerland, interviewed seven times across 30 years of follow-up. MAIN OUTCOME MEASURES: Prevalence of headache; stability of the predominant subtype of headache over time; and age of onset, severity, impact, family history, use of healthcare services, and drugs for headache subtypes. RESULTS: The average one year prevalences of subtypes of headache were 0.9% (female:male ratio of 2.8) for migraine with aura, 10.9% (female:male ratio of 2.2) for migraine without aura, and 11.5% (female:male ratio of 1.2) for tension-type headache. Cumulative 30 year prevalences of headache subtypes were 3.0% for migraine with aura, 36.0% for migraine without aura, and 29.3% for tension-type headache. Despite the high prevalence of migraine without aura, most cases were transient and only about 20% continued to have migraine for more than half of the follow-up period. 69% of participants with migraine and 58% of those with tension-type headache manifested the same predominant subtype over time. However, the prospective stability of the predominant headache subtypes was quite low, with substantial crossover among the subtypes and no specific ordinal pattern of progression. A gradient of severity of clinical correlates and service use was present across headache subtypes; the greatest effect was for migraine with aura followed by migraine without aura, and then tension-type headache and unclassified headaches. CONCLUSIONS: These findings highlight the importance of prospective follow-up of people with headache. The substantial longitudinal overlap among subtypes of headache shows the developmental heterogeneity of headache syndromes. Studies of the causes of headache that apply diagnostic nomenclature based on distinctions between discrete headache subtypes may not capture the true nature of headache in the general population.

Reversible cerebral vasoconstriction syndrome: a rare entity in children presenting with thunderclap headache.

Reversible cerebral vasoconstriction syndrome is characterized by a reversible segmental and multifocal vasoconstriction of cerebral arteries, and severe headaches with or without focal neurologic deficits or seizures. A 15-year-old boy presented with thunderclap headache. He had severe hypertension, although his neurologic examination was normal. Initial workup for thunderclap headache to exclude subarachnoid or intracranial hemorrhage, meningitis, pituitary apoplexy, or venous sinus thrombosis was negative. Brain magnetic resonance angiography and cerebral angiography demonstrated bilateral anterior and posterior circulation diffuse, multifocal, vascular irregularities (beading and stenosis) suggestive of underlying vasculopathy or vasculitis. He was started on verapamil. There was complete reversal of the vascular abnormalities in 6 weeks evident by magnetic resonance angiography, with resolution of headache and normalization of blood pressure. Reversible cerebral vasoconstriction syndrome has been rarely reported in children. This case report highlights the diagnostic dilemma and management of the rare childhood presentation of this condition.

Hypothalamic gray matter volume loss in hypnic headache.

OBJECTIVE: Hypnic headache (HH) is a rare primary headache disorder characterized by strictly nocturnal headache attacks that mostly occur at the same time at night. The pathophysiology of this disease is poorly understood, but hypothalamic involvement was suspected as the hypothalamus represents the cerebral management center of sleep regulation and pain control. METHODS: Fourteen patients with HH and 14 age-matched and gender-matched healthy controls were investigated using magnetic resonance imaging-based voxel-based morphometry. RESULTS: We detected gray matter volume decrease in the posterior hypothalamus of HH patients. Additional gray matter decrease was observed in brain areas known to be associated with cerebral pain processing, including the cingulate cortex, operculum, and frontal lobe, as well as in the temporal lobe. INTERPRETATION: Our data confirm the hypothesized involvement of the posterior hypothalamus in the pathophysiology of HH and emphasize the importance of this structure for sleep regulation and pain control.

Tracing neural connections to pain pathways with relevance to primary headaches.

BACKGROUND: Symptoms associated with primary headaches are linked to cranial vascular activity and to the central nervous system (CNS). REVIEW: The central projections of sensory nerves from three cranial vessels are described in order to further understand pain mechanisms involved in primary headaches. Tracers that label small and large calibre primary afferent fibres revealed similar distributions for the central terminations of sensory nerves in the superficial temporal artery, superior sagittal sinus and middle meningeal artery. The sensory nerve fibres from the vessels pass through both the trigeminal and rostral cervical spinal nerves and terminate in the ventrolateral part of the C1-C3 dorsal horns and the caudal and interpolar divisions of the spinal trigeminal nucleus. The C-fibre terminations were located mainly in the superficial layers (Rexed laminae I and II), and the Aδ-fibres terminated in the deep layers (laminae III and IV). The rostral projections from the ventrolateral C1-C2 dorsal horn revealed terminations in the medial and lateral parabrachial nuclei, the cuneiform nucleus, the periaqueductal gray, the deep mesencephalic nucleus, the thalamic posterior nuclear group and its triangular part, and the thalamic ventral posteromedial nucleus. The terminations in the pons and midbrain were predominately bilateral, whereas those in the thalamus were confined to the contralateral side. CONCLUSIONS: The observations, done in rats with the understanding that similar trigeminovascular organization exists in man, reveal vascular projections into the brainstem and some aspects of the central regions putatively involved in the central processing of noxious craniovascular signals.

Cough-associated headache in patients with Chiari I malformation: CSF Flow analysis by means of cine phase-contrast MR imaging.

The purpose of this study was to analyze the CSF flow in patients with Chiari I to determine differences between patients with and without CAH. Thirty patients with Chiari I malformation underwent cine-PC CSF flow imaging in the sagittal plane. CSF flow pulsations were analyzed by placing regions of interest in the anterior cervical subarachnoid space. Maximum CSF systolic (craniocaudal) and diastolic (caudocranial) velocities as well as the durations of CSF systole and diastole (measured in fractions of the cardiac cycle) were determined. In the region of interest just below the foramen magnum, patients with CAH had a significantly shorter CSF systole and longer diastole (P=.02). A CSF diastolic length of ≥0.75 of the cardiac cycle was 67% sensitive and 86% specific for CAH. Our results indicate that Cine-PC imaging can show differences in CSF flow patterns in patients with Chiari I with and without CAH.

Reversible cerebral vasoconstriction syndrome associated with hormone therapy for intrauterine insemination.

INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) comprises a heterogeneous group of acute neurological diseases which are characterized by thunderclap headache and evidence of reversible multifocal constriction of cerebral arteries. A number of precipitating factors have been described in the literature, including recent childbirth and use of vasoactive substances. CASE DESCRIPTION: Here we present the case of a female patient with RCVS which occurred in the setting of hormonal ovarian stimulation for intrauterine insemination. DISCUSSION: This case possibly contributes to the understanding of the pathophysiological mechanisms underlying reversible cerebral vasoconstriction.

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.

Pearls: headache.

Distinguishing primary headache from secondary headache is the first objective of every new clinical encounter with a patient complaining of headache. The history is king in headache medicine-90% of patients presenting with headache have a primary headache disorder and the examination is normal. The history must be elicited because patients will not always volunteer seminal information. A standard series of questions must be asked of each patient to guide an appropriate diagnostic evaluation and ensure that secondary causes are not overlooked. The second objective, of course, is making the correct diagnosis of the primary headache disorder. Although at first glance this appears obvious and almost patronizing, making the correct diagnosis is often not a priority, nor is it a process that is emphasized in undergraduate and postgraduate training programs. Knowing some simple rules and standard questions will make the process almost fail proof.