The transient syndrome of headache with neurological deficits, cerebrospinal fluid pleocytosis and acute confusional state: a case report

The syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) now referred to in the IHS ICHD–II code 7.8, is only infrequently accompanied by a confusional state and severe agitation. We report the case of a 34–year–old man who suffered from three episodes of headache with transient focal neurological deficits that were consistent with HaNDL but that were accompanied by an intense, confusional agitated state that required admission, in the first episode, in a psychiatric unit.     

Cerebral Vasomotor Changes in the Transient Syndrome of Headache With Neurologic Deficits and CSF Lymphocytosis (HaNDL)

We report two patients with the recently described transient syndrome of headache with neurologic deficits and CSF lymphocytosis (HaNDL). Transcranial Doppler sonography performed during and after attacks of HaNDL showed asymmetrical decreases or increases in blood flow velocity of the middle cerebral artery, accompanied by increases or decreases in pulsatility suggesting fluctuations of arteriolar tone. The findings demonstrate focal vasomotor disturbances that link the transient headaches and deficits of HaNDL with attacks of migraine.     

Reversible Cerebral Vasoconstriction Syndrome Without Typical Thunderclap Headache

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe headache and diffuse segmental intracranial arterial constriction that resolve within three months. Stroke, which is the major complication of RCVS, can result in persistent neurological disability, and rarely causes death. Diagnosis of RCVS early in the clinical course might improve outcomes. Although recurrent thunderclap headache is the clinical hallmark of RCVS, the absence of such a pattern should not lead to discard the diagnosis. Our literature review shows that RCVS can also manifest as an unspecific headache, such as a single severe headache episode, a mild or a progressive headache. Moreover, a subset of patients with severe RCVS presents without any headache, but frequently with seizures, focal neurological deficits, confusion or coma, in the setting of stroke or posterior reversible encephalopathy syndrome. These patients may be aphasic or in comatose state, explaining their inability to give their own medical history. They may have forgotten the headache they had a few days before more dramatic symptoms, or may have a variant of the classical RCVS. By consequence, an RCVS should be suspected in patients with any unusual headache, whether thunderclap or not, and in patients with cryptogenic stroke or convexity subarachnoid hemorrhage, whether the patient also has headache or not. Diagnosis in such cases relies on the demonstration of reversible multifocal intracranial arterial stenosis and the exclusion of other causes.     

Bifocal nummular headache: a series of 6 new cases

(Headache 2011;51:1161‐1166) Objective.— We aimed to report 6 new cases of bifocal nummular headache (NH), showing their clinical characteristics and comparing them with those formerly described. Background.— NH is a focal head pain felt in a small, well‐circumscribed, coin‐shaped area. Among all the reported cases (over 200), 6 patients localized their pain in 2 or more separate areas. Methods.— We reviewed all patients diagnosed with NH at the headache clinics of 2 tertiary hospitals, searching for cases with head pain in 2 different areas. Results.— Six patients (4 female, 2 male; age at onset 40.8 ± 19.1, range 24‐69 years) presented with bifocal NH. The shape and size of both painful areas were identical in each patient. They were located at symmetrical points of either side in 3 patients, while 2 patients had both symptomatic areas on the same side of the head. The chronological pattern was synchronous in 2 patients, and the other 4 showed an additive pattern with onset intervals between the 2 areas ranging from 2 months to 30 years. Pain intensity was slightly different in each area in 4 of the cases. Four patients were treated with a preventive (gabapentin or carbamazepine) with good clinical response. Conclusion.— Although not frequently found, some patients may have bifocal or multifocal NH.     

Reversible segmental cerebral vasoconstriction (Call-Fleming syndrome): are calcium channel inhibitors a potential treatment option?

The Call-Fleming syndrome is characterized by sudden onset of thunderclap-like headache and focal neurological deficits. The pathophysiological correlate is a reversible segmental cerebral vasoconstriction frequently associated with focal cerebral ischaemia. The syndrome has been described in a variety of clinical conditions, and recently an association between the syndrome and exposure to vasoactive drugs was observed. Effective treatment options are not known. A 63-year-old female developed sudden 'worst ever' headache. Initial neurological examination, laboratory blood tests, CSF examination and brain magnetic resonance imaging (MRI) were normal. Previous medical history was unremarkable and she did not take vasoactive drugs. Eleven days after the onset of headache she developed visual field impairment and a right-sided hemiparesis. Brain MRI revealed bilateral posterior and left parietal ischaemic strokes. Cerebral catheter angiography showed segmental arterial vasoconstriction. A vasodilative therapy with calcium channel inhibitors was started and serial transcranial Doppler ultrasonography demonstrated resolution of cerebral arterial vasoconstriction. The present case illustrates that calcium channel inhibitors may be an effective therapy for segmental cerebral arterial vasoconstriction. However, more clinical data are needed to prove this observation.     

The Child With Headache in a Pediatric Emergency Department

Objectives.— To investigate clinical features of a pediatric population presenting with headache to a pediatric emergency department (ED) and to identify headache characteristics which are more likely associated with serious, life‐threatening conditions in distinction from headaches due to more benign processes. Background.— Although headache is a common problem in children visiting a pediatric ED, a few studies thus far have attempted to identify the clinical characteristics most likely associated with suspected life‐threatening disease. Methods.— A retrospective chart review of all consecutive patients who presented with a chief complaint of headache at ED over a 1‐year period was conducted. Etiologies were classified according to the International Headache Society diagnostic criteria 2nd edition. Results.— Four hundred and thirty‐two children (0.8% of the total number of visits) aged from 2 to 18 years (mean age 8.9 years) were enrolled in our study. There were 228 boys (53%) and 204 girls (47%). School‐age group was the most represented (66%). The most common cause of headache was upper respiratory tract infections (19.2%). The remaining majority of non‐life‐threatening headache included migraine (18.5%), posttraumatic headache (5.5%), tension‐type headache (4.6%). Serious life‐threatening intracranial disorders (4.1%) included meningitis (1.6%), acute hydrocephalus (0.9%), tumors (0.7%). We found several clinical clues which demonstrated a statistically significant correlation with dangerous conditions: pre‐school age, recent onset of pain, occipital location, and child's inability to describe the quality of pain and objective neurological signs. Conclusions.— Differential diagnosis between primary and secondary headaches can be very difficult, especially in an ED setting. The majority of headaches are secondary to respiratory infectious diseases and minor head trauma. Our data allowed us to identify clinical features useful to recognize intracranial life‐threatening conditions.     

Idiopathic Ophthalmodynia and Idiopathic Rhinalgia: Two Topographic Facial Pain Syndromes

(Headache 2010;50:1286‐1295) Objective.— To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Background.— Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Methods.— Through a 10‐year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well‐characterized facial pain syndromes. Results.— In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n = 11) or in the nose (n = 7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Conclusions.— Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.     

Hypnotic relaxation vs amitriptyline for tension-type headache: let the patient choose

(Headache 2012;52:785‐791) Background.— Although both pharmacological and behavioral interventions may relieve tension‐type headache, data are lacking regarding treatment preference, long‐term patient compliance, and feasibility of behavioral intervention in a standard neurological outpatient clinic setting. Objective.— To describe patient choice, long‐term compliance, and clinical outcome in a neurological clinic setting where patients are given the choice of the approach they wish to pursue. Design.— Patients presenting to the headache clinic with a diagnosis of tension‐type headache that justified prophylactic therapy (frequent episodic tension‐type headache or chronic tension‐type headache) were given the choice of amitriptyline (AMT) treatment or hypnotic relaxation (HR), and were treated accordingly. Patients were given the option to cross‐over to the other treatment group at each visit. HR was performed during standard length neurology clinic appointments by a neurologist trained to perform hypnosis (Y.E.). Follow‐up interviews were performed between 6 and 12 months following treatment initiation to evaluate patient compliance, changes in headache frequency or severity, and quality‐of‐life parameters. Results.— Ninety‐eight patients were enrolled, 92 agreed to receive prophylactic therapy of some kind. Fifty‐three (57.6%) patients chose HR of which 36 (67.9%) actually initiated this treatment, while 39 (42.4%) chose pharmacological therapy with AMT of which 25 (64.1%) patients actually initiated therapy. Patients with greater analgesic use were more likely to opt for AMT (P = .0002). Eleven of the patients initially choosing AMT and 2 of the patients initially choosing HR crossed over to the other group. Seventy‐four percent of the patients in the HR group and 58% of patients in the AMT group had a 50% reduction in the frequency of headaches (P = .16). Long‐term adherence to treatment with HR exceeded that of AMT. At the end of the study period, 26 of 47 patients who tried HR compared with 10 of 27 who tried AMT continued receiving their initial treatment. Conclusions.— HR treatment was a more popular choice among patients. Patients choosing HR reported greater symptom relief than those choosing AMT and were found to have greater treatment compliance. Patients receiving HR were less likely to change treatments. HR practiced by a neurologist is feasible in a standard neurological outpatient clinic setting; HR training should be considered for neurologists involved in headache treatment.     

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.     

Non-Migrainous Headache and Transient Neurological Deficits

SYNOPSIS
Two young patients experienced transient and recurrent neurological deficits, associated with headache, without fever, signs of meningeal irritation or systemic disease.
This situation in a young patient is usually a manifestation of migraine, but none of our patients had a history f vascular headache. Lumbar puncture was performed, and CSF pleocytosis found in both cases. These cases may represent a poorly recognized benign syndrome of transient neurological signs of headache and a sterile inflammatory reaction in the CSF.     

Neurological manifestations of systemic lupus erythematosus: a prospective study.

A prospective study of the neurological manifestations in all patients with systemic lupus erythematous (SLE) was conducted between February 1985 to January 1989. Excluding herpes zoster infection of peripheral or cranial nerves, post-herpetic neuralgia and migraine, 36 neurological episodes occurred in 33 patients. The presenting symptoms were mental confusion (10), psychosis (five), seizures (six), focal neurological deficit (three), coma (two), headache (five), blurring of vision (three), neuropathy (one) and myelopathy (one). Of these manifestations, only eight episodes were due to primary involvement by SLE: psychosis (two), seizure (two), multiple cerebral infarcts (one), papillitis (one), neuropathy (one) and myelopathy (one). Infection was the most common secondary cause of neurological episodes: all 10 episodes of mental confusion (fungal seven, pyogenic two, tuberculous one, nocardial one); two of six seizures (tuberculous one, pyogenic one); all five headaches (tuberculous meningitis three, cryptococcal meningitis two). The other secondary causes included steroid psychosis (two), hypertensive encephalopathy with seizure (one) and hypertensive retinopathy (one). Three of five cases of focal neurological deficit were due to macrovascular disease rather than to vasculitic infarction. We concluded that cerebral psychosis was a relatively rare presentation in our patients with SLE. In patients who presented with a neurological problem, especially mental confusion, efforts should be made to ascertain the underlying cause, especially if this may be an infection.     

Cluster Headache as the Index Event in MS: A Case Report

We report a 42‐year‐old woman who presented with cluster headache (CH) in association with other neurological symptoms as the index event of new onset multiple sclerosis (MS). Her initial symptoms were left‐sided headache with ipsilateral lacrimation and nasal congestion associated with ipsilateral facial numbness. A subsequent similar headache attack was also associated with ipsilateral arm ataxia and gait ataxia. She had many additional short headache attacks without focal neurological symptoms. Her cluster‐like headache attacks have not recurred since intiation of dimethyl fumarate. Our patient illustrates that cluster‐like headache attacks can occur as a first symptom of MS, in our patient in association with other neurological symptoms. A striking finding in our patient was a large demyelinating lesion in the brachium pontis ipsilateral to the headaches, although additional supratentorial demyelinating lesions were also present. Although CH associated with MS is rare, our patient and the two other reported patients with MS and CH with similar ipsilateral brachium pontis lesions suggest that the lesions in this location may have played a role in the generation of the cluster‐like attacks.     

Nontraumatic Headaches in the Emergency Department: Evaluation of a Clinical Pathway

Objective.— To determine the impact and efficacy of a clinical pathway in the management of patients with nontraumatic and afebrile headache (NTAH) in the emergency department (ED). Background.— Nontraumatic and afebrile headache is one of the most common neurological symptoms in the ED. However, data about the application of an evidence‐based operative protocol are lacking. Methods.— A before–after intervention study comparing adult patients presenting to the ED with atraumatic headache was conducted during a 6‐month period from April to September 2005 and with the same type of patients in the same period in 2006 after a clinical pathway had been implemented. According to their clinical presentations, patients of the 2006 group were divided into 3 subgroups and managed following the established protocol. Study results were based on analysis of 6 months of clinical outcome, the number of CT head scans in the ED, number of neurological consultations in the ED, number of admissions, and length of stay in the ED. Results.— A total of 686 patients were enrolled in the study, of which 374 were those presenting to our ED with NTAH in 2006 and managed with the aid of the study protocol; the other 312 patients were those who presented in 2005, before the intervention. The study protocol was strictly applied to 247 patients (66%) of the 2006 group. There were fewer neurological consultations after the intervention (41.2% vs 52.5%, difference: ?11.3%, 95% confidence intervals [CI]: ?18.7% to ?3.9%; P = .003); likewise, admissions were significantly reduced after the intervention (9.0% vs 14.7%, difference: ?5.7%, 95% CI: ?10.6% to ?0.8%; P = .02). No significant differences were found between the 2 groups for number of CT head scans (42.2% vs 38.4%, difference: 3.7%, 95% CI: ?3.5% to 11%; P = .3). Mean length of stay in the ED was lower after the intervention, though not significantly (170.6 ± 102 minutes vs 180.5 ± 105 minutes, difference: ?9.8 minutes, 95% CI: ?20.3 to 5.7; P = .09). A 6‐month follow‐up was completed involving 302 (96.7%) patients in the first group and 370 (98.9%) in the second group. There was only one misdiagnosis after the intervention while 2 incorrect diagnoses were made before the intervention (0.27% vs 0.6%, difference: ?0.33%, 95% CI: ?2.1% to 0.9%; P = .5). Conclusions.— Our diagnostic protocol for NTAH appears to be safe and sensitive in diagnosing malignant headaches. In addition, it may improve use of resources by reducing the need for neurological consultations and admissions without increasing the number of CT scans or prolonging length of stay in the ED. Furthermore, when using the protocol ED physicians seem more confident in their evaluations of headache resulting in fewer requests for specialist input.     

Prolonged migraine aura resembling ischemic stroke following CoronaVac vaccination: an extended case series

BackgroundAfter the initiation of the COVID-19 vaccination program in Thailand, thousands of patients have experienced unusual focal neurological symptoms. We report 8 patients with focal neurological symptoms after receiving inactivated virus vaccine, CoronaVac.Case seriesPatients were aged 24–48 years and 75% were female. Acute onset of focal neurological symptoms occurred within the first 24 h after vaccination in 75% and between 1-7d in 25%. All presented with lateralized sensory deficits, motor deficits, or both, of 2–14 day duration. Migraine headache occurred in half of the patients. Magnetic resonance imaging of the brain during and after the attacks did not demonstrate any abnormalities suggesting ischemic stroke. All patients showed moderately large regions of hypoperfusion and concurrent smaller regions of hyperperfusion on SPECT imaging while symptomatic. None developed permanent deficits or structural brain injury.DiscussionsHere, we present a case series of transient focal neurological syndrome following Coronavac vaccination. The characteristic sensory symptoms, history of migraine, female predominant, and abnormal functional brain imaging without structural changes suggest migraine aura as pathophysiology. We propose that pain related to vaccine injection, component of vaccine, such as aluminum, or inflammation related to vaccination might trigger migraine aura in susceptible patients.Supplementary InformationThe online version contains supplementary material available at 10.1186/s10194-022-01385-0.     

Elimination of menstrual-related migraine beneficially impacts chronification and medication overuse

Objectives.— This study seeks to determine whether menstrual‐related migraine (MRM) has a discrete, attributable impact on migraine chronicity and medication overuse. Background.— Menstrual‐related migraine can be a disabling headache on its own; but when seen in headache clinics, it is often enmeshed in the setting of chronic migraine (CM) and medication overuse headache (MOH). Whereas nonspecific migraine preventives bestow their benefit uniformly, hormonal preventives (HPs)—when they are successful—address a discrete hormonal mechanism. They confer no known benefit to migraines that are not hormonally triggered. This selective property of HPs could potentially isolate MRM and segregate its effect on the overall clinical picture. Methods.— This is a retrospective review of 229 consecutive women seen in follow‐up for hormonal prevention of MRM at an academic headache center. Patients kept standardized diaries from which separate menstrual‐week (MW) and non‐menstrual week (nonMW) headache indices were calculated and compared. Resolution of MRM was defined by reduction of the MW headache index to a score not exceeding the nonMW headache indices. Consumption of all acute and preventive agents used in the preceding month was tallied. We performed post‐treatment comparisons of medication usage and headache characteristics among subjects in whom MRM was resolved and those in whom it was not resolved. Results.— At baseline, CM was present in 92% of subjects, 72% of whom also met criteria for MOH. Resolution of MRM was achieved in 81% of subjects who were compliant with HP and was associated with reversion to episodic migraine (59% vs 18%, P < .001) and resolution of medication overuse (54% vs 20%, P < .001). Resolution of MRM was associated with significant decreases in per capita consumption of triptans, opioids, all acute agents, and migraine preventive medications. Conclusions.— Resolution of MRM correlated not only with conversion of CM to an episodic pattern, but also with a significant reduction in medication usage. It offers preliminary evidence that hormonal regimens may have a beneficial role in prevention of MRM.     

Migrainous Syndrome With CSF Pleocytosis. SPECT Findings

Brain single photon emission computed tomography (SPECT) findings are described in four adult patients with the transient syndrome of headache with neurological deficits and cerebrospinal fluid (CSF) pleocytosis. Focal deficits consisted of right-sided hemisensory changes with or without motor dysphasia or dysarthric speech (n=4) and confusional episodes (n=1). All patients had a CSF pleocytosis (with a mean of 309 cells/mm 3 on the first spinal tap; range 75 to 590) and an elevated total protein (mean 130.5 mg/dL; range 70 to 193). The EEG showed excessive focal slowing (n=2). A technetium Tc 99m hexamethyl propylenamine oxime (HMPAO) brain SPECT was performed during a symptom-free period, within 8 and 25 days after the onset of symptoms (n=4). Three patients showed a decreased tracer uptake in the anterior left hemisphere, topographically consistent with the neurological deficits and EEG slowing. One patient showed no abnormalities. These findings indicate either focally impaired neuronal metabolism or hypoperfusion in regional cerebral blood flow, which could bear some relationship with the clinical features. The possibility that SPECT abnormalities may represent an epiphenomenon was also considered.     

Menarche in Girls and Headache – A Longitudinal Analysis

Background.— Puberty is assumed to influence the occurrence of headache, increasing the risk for recurrent episodes, especially in girls. The increase of headache, in particular recurrent headache, in girls from around the age of 12 on, is often ascribed to the occurrence of menarche as the most stringent indicator of completed puberty. Objectives.— We examined the hypothesis that the occurrence of menarche in girls is predictive of recurrent headache in reference to no or rare headache in the past 6 months. Furthermore, the assumption was tested that headache episodes increase after onset of menarche but remain unchanged in girls not having experienced their first menstruation. We also expected a higher probability of migraine type of headache after menarche. In a further analysis girls with and without menarche were compared with boys, and a difference between gender only for girls with menarche was predicted. Methods.— In an epidemiological study recruiting 8800 families with children 7‐14 years in Southern Lower Saxony (Germany), we conducted 3 yearly panels based on postal questionnaires. Headache data are based on self‐report of children from 9 years on. Parents were asked for information regarding the onset of menarche. Approximately 1100 girls and about the same number of boys constitute the sample for our analyses. Results.— The results of a logistic regression analysis demonstrate that in 11‐ to 16‐year‐old girls having experienced menarche in either the year of the assessment of headache or 2 years before that, the risk for recurrent headache is increased when compared with girls without menarche. Intraindividual longitudinal analyses, however, do not support our hypotheses that after the onset of menarche headaches become more frequent. The expected differences in headache between girls with menarche and boys were found. The risk for migraine‐like headaches is not significantly higher after onset of menarche than before. Conclusions.— Thus, results do not consistently support the hypothesis that puberty is a moderator of headache frequency. Even in cases where the outcome of regression analyses is supportive of our expectations, explained variance is diminutive (maximum 2.2%). Thus, the influence of menarche on headache seems to be only marginal.     

Lack of Efficacy of Manual Therapy in Children and Adolescents With Suspected Cervicogenic Headache: Results of a Prospective,Randomized, Placebo‐Controlled,and Blinded Trial

(Headache 2010;50:224‐230) Objective.— Clinical trials concerning cervical spine manipulation and mobilization in children and adolescents with cervicogenic headache are lacking. Methods.— We performed a multicenter, prospective, randomized, placebo‐controlled, and blinded trial in 52 children and adolescents (21 boys, 31 girls) aged 7‐15. After prospective baseline documentation for 2 months patients were either assigned to placebo or true manipulation with another 2‐month follow‐up. Main outcome measures were defined as: percentage of days with headache, total duration of headache, days with school absence due to headache, consume of analgesics, intensity of headache. Results.— We did not find a significant difference comparing the groups with placebo and true manipulation with respect to the defined main outcome measures. Conclusions.— We were not able to show an efficacy of cervical spine manipulation in 52 children and adolescents.