Cluster headache-like headache, Hageman trait deficiency, retrobulbar neuritis, and giant aneurysm

A 56-year-old, previously reported woman with cluster headache-like headache with bouts of unilateral (the side of predominance changing through the years) severe headache had a familial history (three generations) of partial Hageman factor deficiency and bleeding episodes. A giant aneurysm was found to be lodged in the anterior communicating artery on the left side. Clinically, the features were atypical for cluster headache: onset at a young age (14 years), episodes of retrobulbar neuritis appearing at the side of pain, etc. Studies of forehead sweating indicated that the right side was the pathologic one, from an autonomic point of view, as did pupillometric studies. However, during attacks, which were left-sided at the time, forehead sweating was marked laterally on the left side and on the upper eyelid, but not on the right. The "signal" usually reaching the autonomically stigmatized side during attacks of cluster headache, therefore, did not seem to reach the sweat glands on that (the right) side during the attack in the present case. This headache may, therefore, be distinct from cluster headache, both from a clinical and from an autonomic function point of view.     

Cluster headache: Further observations on the dissociation of pain and autonomic findings

A 51-year-old male cluster headache patient had during five bouts in the course of 11 years always had the headache attacks on the left side. Autonomic abnormalities were, however, present on the right side. Pupillometrically, there was thus a Horner-like syndrome on the right (non-symptomatic) side, with miosis and a relatively more marked dilatation of that eye subsequent to topical application of a directly working sympathomimetic agent (phenylephrine) than after an indirectly working one (hydroxyamphetamine), whereas this was not the case on the symptomatic side. The findings on evaporimetry were not as clear-cut as the pupillometric findings; however, even facial sweating was consistent with a pathologic condition on the right (non-symptomatic) side. A primary dichotomy of pain and autonomic signs (that is, not due to change of side of pain localization) thus seems to be present in this case.     

The sweating anomaly in cluster headache

We describe a patient with a typical history of cluster headache for more than 18 years. During the first approximately 10 years of his disease, the pain was right-sided, and pupillometric and evaporimetric measurements indicated a sympathetic deficiency on this same side. However, for the next >6 years, his pain was consistently left-sided, although the signs of sympathetic dysfunction still were more marked on the right side. This was also true for the findings obtained during the interictal period and for the heating test performed within an attack. The implications of this interesting case are discussed. The view that two separate lines of symptom production lead to the pain and the autonomic phenomena seems to be supported by this case history. The cluster headache syndrome may also be a bilateral disorder, with only the weight of balance pointing one way or the other. Finally, the autonomic test results of this patient could reflect an autonomic "scar" in the previous headache side.     

Headaches add to the burden of epilepsy

The objective was to investigate and classify headaches in 109 consecutive adult patients with epilepsy. A semi-structured interview was performed in those who confirmed such symptoms (65%). Interictal headaches were present in 52%; 20% had interictal migraine. Postictal headache was reported in 44%. Migraine characteristics were present in 42% of these, and most of them (74%) also suffered from interictal migraine. Six percent had preictal headache. In partial epilepsy, there was an association between headache lateralisation and interictal EEG abnormalities (p=0.02). We conclude that headache, including migraine, is very common in patients with epilepsy. Unilateral headache may represent a lateralising sign in focal epilepsy. Seizures often trigger postictal headaches with migraine features, which often are associated with interictal migraine. Migrainous headaches sometimes proceed into epileptic seizures. The comorbidity of migraine and epilepsy should receive ample clinical attention, as it may influence antiepileptic drug choice, and the headache may need specific treatment.     

Cerebral venous thrombosis initially considered as a complication of spinal-epidural anaesthesia

Cerebral venous thrombosis is an unusual condition characterized by headache, nausea, vomiting, focal deficits and epileptic seizures. In this case report we describe a patient who presented with headache and focal motor deficits after an uneventful Caesarean section, performed using combined spinal-epidural anaesthesia. Magnetic resonance imaging was performed because of the pronounced neurological symptoms, and a diagnosis of cerebral venous thrombosis was made. The patient was treated with anticoagulant agents and made a complete recovery. This case emphasizes the importance of considering cerebral venous thrombosis in the differential diagnosis of headache in the post-partum period prior to instituting conventional therapy.     

Relationship between migraine and epilepsy in pediatric age

OBJECTIVE: Many studies have supported the hypothesis of alteration of cortical hyperexcitability as a possible pathological mechanism underlying the onset of migraine and epileptic attacks. Different biochemical pathways involving cellular structures may increase or decrease the excitability of neuronal membranes. The aim of this study was to identify a possible link between migraine and epilepsy from a clinical and neurophysiologic point of view. METHODS: One-hundred thirty-seven children and adolescents consecutively diagnosed for tension-type headache and idiopathic migraine with and without aura were studied. Anamnestic, clinical, and instrumental data were collected by a neurological examination, a specific questionnaire, and awake electroencephalogram (EEG) registrations. EEG features of nonheadache and nonseizures control group were compared. RESULTS: Fourteen cases (10.2%) had a positive history for seizures with fever, isolated seizures, or epilepsy. Distribution differed according to headache diagnosis; migraine with aura (MWA) was largely prevalent. Specific electroencephalographic abnormalities were present in 11.7% of the sample, with a significant different distribution across the groups of children with headache and the control group: specific interictal abnormalities were found in 10 of 23 (43.5%) children with MWA. Two factors, seizures and specific interictal electroencephalographic abnormalities, showed a different distribution in patients with MWA compared to other classes of headache and control group (P < or = .01). CONCLUSION: The present study supports the hypothesis of a possible clinical continuum between some types of MWA and epileptic syndromes as entities due to altered neuronal excitability with similar genetic substrates.     

Dental presentations of cluster headaches

Cluster headache has been defined by the International Headache Society (IHS) as one of the primary headaches. A primary headache is a headache that has no other known cause, such as infection or trauma. Cluster headache is also listed as one of the trigeminal autonomic cephalalgias. These headaches are mediated by the trigeminal nerve with accompanying autonomic symptoms that may range from conjunctival injection, lacrimation, nasal congestion, rhinorrhea, forehead and facial sweating, miosis, and ptosis to eyelid edema. The IHS has described cluster headache as "attacks of severe, strictly unilateral pain that is orbital, supraorbital, temporal or in any combination of these sites, lasting 15 to 180 minutes." In the author’s practice, as a dentist treating orofacial pain, patients with cluster headache have dental or midfacial complaints as a primary presentation. This paper introduces such presentations based on interviews with cluster headache patients, with the main purpose of having midfacial complaints considered as an important presentation to be added to the IHS diagnostic criteria for cluster headache.     

Rare pathomorphologic findings in complicated migraine

137 patients suffering from classical or complicated migraine were investigated in the Neurology Department of the University of Vienna between 1971 and 1984. 13 of these patients were found to have pathological alterations and their case histories are presented. Clinically, 11 patients suffered from migraine accompagnée (in 2 cases accompanied by epileptic seizures), 1 patient had ophthalmoplegic migraine and 1 had a subarachnoid haemorrhage imitating migraine. The underlying pathological findings were: 1 tumour, 4 arteriovenous malformations, 4 aneurysms, 1 arterio-venous shunt, 1 pathological vascular network, 1 Moya-Moya syndrome and 1 intracerebral haemorrhage without detectable source of bleeding. 8 of the patients underwent successful surgery and most of them showed subsequent clinical improvement. The family history was positive in only 2 patients. The time interval between the occurrence of the first symptoms and the establishment of the final diagnosis was up to 25 years. The neurologist should undertake extensive investigation of the patient, including cerebral angiography, if the following criteria apply: hemicrania consistently on the same side; change in type of headache after a number of years; uniform complicating neurological symptoms; additional occurrence of epileptic seizures; manifestation of neurological symptoms after the prodromal phase; persistent neurological signs without remission; negative family history; persisting diffuse or locally accentuated EEG changes; pathological CAT results.     

A report of cranial autonomic symptoms in migraineurs

The presence of cranial autonomic symptoms in migraine is well known and thought to represent activation of the trigeminal parasympathetic reflex pathway similar to trigeminal autonomic cephalalgias. However, studies regarding the prevalence of these symptoms are few. The characteristics of migraineurs with cranial autonomic symptoms and the association of cranial autonomic symptoms with laterality of headache have never been studied in a clinic population. Seventy-eight consecutive subjects with migraine were recruited from the Headache Clinic of the Department of Psychiatry after exclusion of subjects with secondary headache. Their demographic data and detailed history of headache were noted and leading questions were asked regarding cranial autonomic symptoms. chi(2) test and Fisher's exact test was used for categorical variables, whereas an independent sample t-test was applied on numerical data. Spearman's correlation was used for correlational analysis of categorical variables. Female subjects (78.2%) outnumbered males and the average duration of illness in the whole sample was 3.81 years. Migraine without aura (53.8%) was the commonest diagnosis, followed by migraine with aura (24.4%). Cranial autonomic symptoms were present in 73.1% of subjects and, commonly, they were ipsilateral to headache. Moreover, strictly unilateral cranial autonomic symptoms were reported by only 32% of patients. The anatomical side of headache did not affect the presence of autonomic symptoms. Those with or without autonomic symptoms did not differ with respect to gender, diagnosis, laterality of headache or associated symptoms except phonophobia, which was more common in subjects with autonomic symptoms (P = 0.05). Those with autonomic symptoms had longer duration of illness (P = 0.03) and longer headache episodes (P = 0.04). In addition, sleep was ineffective in relieving their headache (P = 0.02). Cranial autonomic symptoms are frequent in migraineurs and are common in subjects with long duration of illness and longer headache episodes. Clinical evidence in the present study suggests that subjects with cranial autonomic symptoms have a hyperactive efferent arm of trigeminal autonomic reflex. The connections of trigeminal nucleus with the locus coeruleus and dorsal raphe nucleus may account for the observed phenotypic differences between the two groups. Further research, however, is required to elucidate the underlying neural mechanisms of cranial autonomic symptoms in migraine.     

Symptomatic occipital epilepsy misdiagnosed as migraine

Two young patients with symptomatic occipital lobe epilepsy due to discrete lesions of cysticercosis were misdiagnosed and treated for 2 years as migraine with visual aura. The patients suffered from frequent visual seizures often followed by migraine-like headache. Seizures manifested with colored and mainly circular elementary visual hallucinations of up to 1 minute duration. Headache, often severe and of long duration, was frequently associated with nausea, photophobia, and phonophobia. Both patients became seizure-free with appropriate treatment of the underlying disease and epileptic seizures.     

Juvenile myoclonic epilepsy presenting as a new daily persistent-like headache

New daily persistent headache (NDPH) is a recognized subtype of chronic daily headache with a unique presentation of a daily headache from onset typically in individuals with minimal or no prior headache history. Various secondary mimics of NDPH have now been documented but at present there has been no association made between primary epilepsy syndromes and new daily persistent-like headaches. A case patient is presented who developed a daily continuous headache from onset who 3 months after headache initiation had her first generalized tonic-clonic seizure. Further investigation into her history and her specific EEG pattern suggested a diagnosis of juvenile myoclonic epilepsy (JME). Her NDPH and seizures ceased with epilepsy treatment. Clinically relevant was that the headache was the primary persistent clinical symptom of her JME before the onset of generalized tonic-clonic seizures. The current case report adds another possible secondary cause of new daily persistent-like headaches to the medical literature and suggests another association between primary epilepsy syndromes and distinct headache syndromes.     

Meningeal Hyperperfusion Visualized by MRI in a Patient With Visual Hallucinations and Migraine

A 41-year-old patient with a history of migraine but with no history of seizures had intermittent prolonged and variable complex visual hallucinations and illusions lasting 9 days, accompanied by unilateral headache. Electroencephalography during these visual symptoms revealed occipital epileptic discharges. Distinction between focal migrainous attacks and ictal phenomena was difficult. Magnetic resonance imaging showed a lesion in the right visual cortex probably related to low perfusion and hyperemia of meningeal vessels, representing the rarely described transient MRI changes associated with migraine. Continued treatment with antiepileptic drugs and calcium-channel blocking agents completely resolved the headache and visual symptoms, while minor EEG changes persisted. After discontinuation of treatment, a second attack occurred with a similar and reversible pattern on EEG.     

Dorsolateral medullary ischemic infarction causing autonomic dysfunction and headache: a case report

Stroke can present, among other signs, with headache. Here, we describe the case of a man suffering from severe orbitary pain and autonomic dysfunction secondary to dorsolateral medullary ischemia. The anatomical relationship between lesion and symptomatology could be an indirect sign of hypothalamospinal tract involvement in the genesis of autonomic dysfunction and headache resembling a trigeminal autonomic cephalalgia.     

Posterior reversible encephalopathy syndrome (PRES) in critically ill obstetric patients

Objective To describe clinical, neuroradiological and evolutionary findings in obstetric patients with posterior reversible encephalopathy syndrome (PRES).Design Retrospective case series.Setting University intensive care unit (ICU).Patients Four critically ill patients. Two patients experienced PRES in late postpartum without the classical pre-eclamptic signs. All patients showed impairment of consciousness and epileptic seizures; two of them presented cortical blindness and headache, too. True status epilepticus (SE) occurred in two cases. In all patients MRI showed the typical feature of gray-white matter edema, mainly localized to the temporo-parieto-occipital areas.Interventions Normalization of high blood pressure (BP) and treatment of seizures. Two patients with SE and severe impairment of consciousness were treated with an intravenous valproate (ivVPA) bolus followed by continuous infusion.Measurements and results In three cases, neurological and MRI abnormalities completely resolved in about a week. Another patient died due to subarachnoid hemorrhage.Conclusion Posterior reversible encephalopathy syndrome is a well described clinical and neuroradiological syndrome characterized by headache, altered mental status, cortical blindness and seizures, and a diagnostic MRI picture; usually reversible, PRES can sometimes result in death or in irreversible neurological deficits, thus requiring early diagnosis and prompt treatment. PRES can have various etiologies, but pregnancy and postpartum more frequently lead to this condition. Treatment of seizures deserves special attention since the anti-epileptic drugs currently used in SE management may worsen vigilance as well as autonomic functions. Extensive research is needed to assess the role of ivVPA in this condition.     

Group psychotherapy with epileptic adolescents during initial vocational training

When the therapeutic alliance between physician or psychotherapist, and epileptic patient is seriously disrupted (non-compliance) group psychotherapy is capable of bringing about marked changes in coping with the epileptic condition. Even a brief therapy of 20 sessions with epileptic young people undergoing initial vocational training contributes to their learning to perceive problems present in their vocational-social development, as well as various disease phenomena and therapeutic possibilities. Eight young adults aged 18-20 years who had been referred to the Annedore Leber Vocational Training Centre for psychological and neurological examination suffered from frequent seizures, which had in each case been attributable to non-compliance. On completion of therapy, serum levels of anti-epileptic drugs had reached the therapeutic range. None of them failed to complete their vocational training.     

Epileptic seizures caused by low valproic acid levels from an interaction with meropenem

A 55-year-old woman was diagnosed with pneumonia and was treated with meropenem; 5 days later she developed epileptic seizures. She had been treated with valproic acid for 16 years to control her epileptic seizures. Her serum valproic acid concentration was low during treatment with meropenem than previously recorded despite an increase of valproic dose. As soon as administration of meropenem was withdrawn, valproic acid concentration increased to previous levels and her seizures stopped. Meropenem decreases valproic acid concentration, and may promote the development of epileptic seizures in previously controlled epileptic patients. The acute lowering of serum valproate produced by meropenem probably precludes their concomitant use.     

Headache,anosmia, ageusia and other neurological symptoms in COVID-19: a cross-sectional study

BackgroundNeurological symptoms are frequent among patients with COVID-19. Little is known regarding the repercussions of neurological symptoms for patients and how these symptoms are related to one another.ObjectivesTo determine whether there is an association between the neurological symptoms in patients with COVID-19, and to characterize the headache.MethodThis was a cross-sectional study. All hospital inpatients and health workers at the Hospital Universitario Oswaldo Cruz with a PCR-confirmed COVID-19 infection between March and June 2020 were considered for the study and were interviewed by telephone at least 2-months after the acute phase of the disease. These patients were identified by the hospital epidemiological surveillance department. A semi-structured questionnaire was used containing sociodemographic and clinical data and the ID-Migraine.ResultsA total of 288 patients was interviewed; 53.1% were male; with a median age of 49.9 (41.5–60.5) years; 91.7% presented some neurological symptom; 22.2% reported some neurological symptom as the symptom that troubled them most during COVID-19. Neurological symptoms were: ageusia (69.8%), headache (69.1%), anosmia (67%), myalgia (44.4%), drowsiness (37.2%), agitation (20.8%); mental confusion (14.9%), syncope (4.9%) and epileptic seizures (2.8%). Females, those who presented with fever, sore throat, anosmia/ageusia and myalgia also presented significantly more with headache (logistic regression). The most frequent headache phenotype was a non-migraine phenotype, was of severe intensity and differed from previous headaches. This persisted for more than 30 days in 18% and for more than 90 days in 10% of patients. Thirteen percent of those with anosmia and 11% with ageusia continued with these complaints after more than 90 days of the acute phase of the disease. Aged over 50 years, agitation and epileptic seizures were significantly associated with mental confusion (logistic regression).ConclusionHeadache is frequent in COVID-19, is associated with other symptoms such as fever, sore throat, anosmia, ageusia, and myalgia, and may persist beyond the acute phase of the disease.Supplementary InformationThe online version contains supplementary material available at 10.1186/s10194-021-01367-8.     

Hemicrania Continua: A Case Responsive to Piroxicam-Beta-Cyclodextrin

The case of a 53-year-old woman is described, who since the age of 38 years had been suffering from a unilateral headache that was at first remitting, but then evolved into an unremitting course. The headache was burning in quality, located in the left forehead and face, and was accompanied by mild ipsilateral autonomic symptoms. The clinical picture, as well as the absolute response to indomethacin, suggested a diagnosis of hemicrania continua. The presence of mild gastric discomfort on continuous indomethacin administration made us transfer the patient to another NSAID (piroxicam-beta-cyclodextrin). With this drug, complete relief and good tolerability was obtained.