A Case Report: The CSF Characteristics of HaNDL Syndrome.

BACKGROUND: Headache and Neurologic Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) is a rare and underdiagnosed syndrome that mimics recurrent stroke, migraine with aura, and encephalitis. OBJECTIVE: Describe the presentation, clinical characteristics, and cerebrospinal fluid (CSF) findings in a patient with HaNDL and provide insight into the importance of the recognition of this syndrome. METHODS: The authors describe a unique case of (HaNDL) during which the patient underwent three lumbar punctures over 26 days. RESULTS: The authors demonstrate the natural course of CSF characteristics of a patient with HaNDL, with rising and falling lymphocyte counts. Additionally, the authors provide an example of the clinical presentation of HaNDL, with episodic attacks over the course of 1 month of migraine headache, hemibody paresthesias, hemibody weakness, and encephalopathy. CONCLUSIONS: HaNDL is a headache syndrome mimicking viral encephalitis, migraine with aura, and recurrent cerebral ischemic events. While HaNDL is a diagnosis of exclusion, the syndrome's association with characteristic clinical and laboratory findings are important to recognize. Furthermore, a monophasic pattern of CSF lymphocytosis in HaNDL may be observed. IMPLICATIONS FOR NURSING: Increased recognition of this syndrome may help prevent unnecessary tests and treatments when patients present with recurrent episodes.

An Unusual Cause of Chronic Headache in an Adolescent Boy: A Case Report.

Spontaneous intracranial hypotension (SIH) is an under-diagnosed cause of headache in children and adolescents. SIH results from cerebrospinal fluid (CSF) leak due to breach in the dura mater and the etiology for dural breach is often diverse. We report an adolescent boy who presented with chronic episodic headache that later progressed to daily headache. There was a typical history of worsening of headache on upright position and relief of headache on lying down. He was treated with migraine prophylaxis in another hospital but there was no response. Marfanoid features and brisk deep tendon reflexes were observed on clinical examination. Brain magnetic resonance imaging (MRI) revealed sagging of the brain stem, pachymeningeal enhancement, and tonsillar herniation. MRI of spine myelogram confirmed multiple levels of CSF leak. He was initially managed with supportive measures and fluoroscopic-guided fibrin glue injection. Although child remained symptom-free for the next 6 months, he again developed headache. MRI and computed tomography spine myelogram revealed a meningeal diverticulum in the lumbar spine. He was managed with an autologous epidural blood patch and he has been well since then. In this report, we highlight the clinical and radiological pointers to the presence of SIH in children with recurrent headache.

Borrelia lusitaniae Infection Mimicking Headache, Neurologic Deficits, and Cerebrospinal Fluid Lymphocytosis.

Headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL) is a rare headache syndrome included in the Classification of Headache of the International Headache Society as a "headache attributed to non-infectious inflammatory intracranial disease." We report one 15-year-old patient with clinical history and cerebrospinal fluid findings compatible with the diagnosis of HaNDL in whom Borrelia lusitaniae was identified in cerebrospinal fluid by polymerase chain reaction.

Isolated cerebrospinal fluid hypertension in chronic headache: diagnostic innovations and clinical implications.

Isolated cerebrospinal fluid hypertension (ICH) is a condition of increased cerebrospinal fluid (CSF) pressure in the cranial-spinal compartment without an identifiable cause. Isolated headache is the most common symptom of ICH, while missing may be signs such as papilledema or sixth nerve palsy. This fact makes difficult the clinical diagnosis of headache attributable to ICH in headache sufferers. Another source of confusion stems from the CSF pressure measurement. It has been observed that a single-spot CSF opening pressure measurement may be insufficient to identify elevated CSF pressure in headache sufferers. A new method of CSF pressure measurement has been able to identify pressure-related features of isolated CSF hypertension (ICH). In fact, nocturnal or postural headache and abnormal pressure pulsations are the more common pressure-related features of ICH in patients with chronic headache. The compressive action of these abnormal pressure pulsations causes the periventricular white matter microstructure alterations leading to the focal diffusion tensor imaging findings in patients with ICH. Abnormal pressure pulsations are a marker of ICH in chronic headache. The identification of the CSF pressure-related features may be useful for differentiating headache sufferers with ICH from those with primary headache disorder in clinical practice. The therapeutic strategy in these headache sufferers with ICH includes the CSF removal and a medical treatment.

Angiomatosis leptomeníngea temporo-occipital de diagnóstico en edad adulta / Adult diagnosis of temporo-occipital leptomeningeal angiomatosis

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New daily persistent headache: an update.

New daily persistent headache is a primary headache disorder marked by a unique temporal profile which is daily from onset. For many sufferers this is their first ever headache. Very little is known about the pathogenesis of this condition. It might be a disorder of abnormal glial activation with persistent central nervous system inflammation and it may be a syndrome that occurs in individuals who have a history of cervical hypermobility. At present there is no known specific treatment and many patients go for years to decades without any improvement in their condition despite aggressive therapy. This article will present an up-to-date overview of new daily persistent headache on the topics of clinical presentation, treatment, diagnostic criteria, and presumed pathogenesis. It will also provide some of the authors own treatment suggestions based on recognized triggering events and some suggestions for future clinical trials.

Inflammatory profile in cerebrospinal fluid of patients with headache as a manifestation of neuropsychiatric systemic lupus erythematosus.

OBJECTIVE: The objective of this study was to define the cytokine and chemokine profiles in cerebrospinal fluid (CSF) from patients with headache as neuropsychiatric systemic lupus erythematosus (NPSLE). METHODS: In a post hoc analysis, seven patients hospitalized because of headache were included. Patients were evaluated at hospitalization and 6 months later and a CSF sample was obtained. As controls, CSF from 27 patients with other NPSLE syndromes, 16 SLE patients without a history of NP manifestations (non-NPSLE) and 25 patients with non-autoimmune diseases were studied. Soluble molecules including cytokines (IL-2, IL-4, IL-6, IL-10, TNF-α, IFN-α and IFN-γ) and chemokines [monocyte chemotactic protein-1, RANTES (regulated on activation normal T cell expressed and secreted), IL-8, monokine induced by IFN-γ (MIG), and IFN-γ-induced protein 10 (IP-10)] were measured with the use of cytometric bead array kits or luminometry. RESULTS: Patients with headache had increased CSF values in the following molecules compared with non-NPSLE and non-autoimmune diseases patients, respectively: IL-6 (208.5, 3.0, 3.0 pg/ml, P < 0.004 and P < 0.001), IL-8 (406.6, 30.0, 19.7 pg/ml, P < 0.05 and P < 0.004), IP-10 (4673, 329.7, 113.6 pg/ml, P = 0.02 and P < 0.002), RANTES (7.5, 2.5, 2.2 pg/ml, P < 0.003 for both) and MIG (944.7, 11.4, 3.5 pg/ml, P = 0.02 and P = 0.001). No clear difference was observed between patients with headache and other NPSLE. Higher levels of inflammatory molecules were found in patients with headache from intracranial hypertension and intractable non-specific headache than patients with migraine. Six months later, when the headache had resolved, all the elevated molecule levels had decreased significantly. CONCLUSION: Headache from intracranial hypertension and intractable non-specific headache, but not migraine, share the inflammatory profile in CSF observed in other NPSLE syndromes.

Documentation of cerebrospinal fluid opening pressure and other important aspects of lumbar puncture in acute headache.

BACKGROUND: Measuring cerebrospinal fluid (CSF) opening pressure by lumbar puncture (LP) is an essential tool in the investigation of patients with acute headache. AIM: To assess documentation of opening CSF pressure in those with acute headache undergoing LP. General documentation of the procedure and CSF investigations was also assessed. METHODS: Retrospective review of medical records of patients admitted to a teaching hospital Acute Medical Admissions Unit over a three-month period with a presenting complaint of headache. RESULTS: A total of 106 patients presented with headache of whom 48 patients had at least one LP attempted. Only 41 patients (85%, 95% CI 72-94) had their LP documented. Of 47 patients that had a successful LP, 22 (47%) had a recorded opening pressure. Eighteen (32%) of all patients had their position recorded, with seven (15%) patients having had position and opening pressure documented. Twenty patients (43%) had the appropriate results documented. Twelve patients (31%) had paired serum glucose measured. CONCLUSIONS: Documentation of a LP for headache in the acute setting was generally poor. CSF opening pressure measurement was frequently omitted and no appropriate action taken if high. Paired serum glucose was rarely measured. Acute physicians may benefit from a proposed protocol and documentation sticker.

Glutamate levels in cerebrospinal fluid and triptans overuse in chronic migraine.

OBJECTIVE: Chronic migraine (CM) is a common disorder, affecting 2% to 3% of the general population. Glutamate is implicated in cortical spreading depression, trigeminovascular activation, central sensitization, and may be linked to migraine chronification. Triptans brought a novel option for the acute migraine treatment. As the development of central sensitization impacts upon the effectiveness of triptan therapy, we hypothesized that glutamate might be related to triptan response mechanisms. METHODS: We studied 19 patients diagnosed with CM according to the International Headache Society (2004) criteria. Patients were divided in those overusing analgesics (NSAIDs); those without overuse, and those overusing triptans. RESULTS: Cerebrospinal fluid (CSF) glutamate levels were similar in patients overusing acute medications (0.335 +/- 0.225 micromol) compared to those without overuse (0.354 +/- 0.141 micromol), P= NS). In contrast, patients overusing triptans had CSF glutamate levels significantly lower than that observed in nonoverusers (0.175 +/- 0.057 vs 0.354 +/- 0.141 micromol, P= 0.015), and significantly higher than controls (0.175 +/- 0.057 vs 0.109 +/- 0.066 micromol, P= 0.039). In triptan overusers, CSF glutamate levels, although lower, were not significantly different from patients overusing other types of analgesics. CONCLUSIONS: Our study showed lower glutamate levels in CSF of CM patients overusing triptans. Glutamate may be implicated in triptan response mechanisms, triptans may work in part by reducing extracellular glutamate levels in the brain.

[Spontaneous intracranial hypotension]. / Síndrome de hipotension intracraneal espontánea.

Spontaneous intracranial hypotension is an infrequent clinical entity characterized by orthostatic headache, low cerebrospinal fluid pressure, and magnetic resonance imaging findings of diffuse pachymeningeal gadolinium enhancement without previous history of head trauma or lumbar puncture. A 24 year-old healthy woman was referred after having daily headaches for two weeks. She described a headache that occurred soon after assuming an upright position and disappeared after resuming a recumbent position. The cerebrospinal fluid showed pleocytosis primarily lymphocitic, raised protein content and an opening pressure of 20 mm H2O. Magnetic resonance imaging of the head showed diffuse pachymeningeal gadolinium enhancement. Radioisotope cisternography using 99mTc-DTPA confirmed the cerebrospinal fluid leak in the thoracic region. With conservative treatment the patient improved in a few days and the headache disappeared. Radiological findings, together with a compatible clinical condition, help to establish the diagnosis of spontaneous intracranial hypotension and avoid the use of unnecessary clinical investigations.

Cerebrospinal fluid biomarkers in primary headache disorders.

OBJECTIVE: The object of this review is to examine the published literature for cerebrospinal fluid laboratory measures of primary headache disorders to identify biomarkers and provide recommendations for future biomarker discovery. BACKGROUND: Biomarkers may distinguish deviation from a normal state, provide insight into mechanisms of pathophysiology, quantify the degree of change, discriminate what may be clinically overlapping disorders, and allow monitoring and/or selection of specific treatment. High-throughput, discovery technologies fuel the ability to reveal more biomarkers than past hypothesis-driven studies. DESIGN OR METHODS: Publications were identified in PubMed, ISI web of knowledge (both Web of Science and BIOSYS), and SciFinder, using the key words for cerebrospinal fluid (CSF) and migraine, headache, or biomarkers. Additional references were sought from the papers identified in these searches. Data were assessed relating to all primary headache types for clinical and scientific methods and results. RESULTS: Fifty-five out of 82 biomarkers were found from 55 publications, though none have been validated for clinical utility. Data for site (ventricular, cervical, lumbar) and timing of CSF collection, headache state, and diagnostic description were patchy, and controls were often poorly defined. Most routinely performed CSF measurements were within normal limits. Most levels of pain-related molecules were reduced, and concentrations of most neurotransmitters, neuropeptides, proteins, and small molecules were increased. Though few studies assessed the specificity of biomarkers for primary headaches, it is clear that there are differences in CSF biomarkers between migraine, cluster headache, tension-type headache, and trigeminal neuralgia. CONCLUSIONS: The high proportion (67%) of biomarkers identified from laboratory measures tested thus far predicts that many more biomarkers will be identified for primary headaches when more candidates are evaluated. In order to discover and evaluate more biomarkers, especially those that may have clinical application for headache management, 3 recommendations are encouraged: prospective design of care-independent studies; evaluation of more clinical variables; and evaluation of substantially more candidates by using discovery-based research methods. Outlines of approaches to pursue these aims are proposed.

Utility of CSF pressure monitoring to identify idiopathic intracranial hypertension without papilledema in patients with chronic daily headache.

The aim of the present study was to report on the utility of continuous Pcsf monitoring in establishing the diagnosis of idiopathic intracranial hypertension without papilledema (IIHWOP) in chronic daily headache (CDH) patients. We report a series of patients (n = 10) with refractory headaches and suspected IIHWOP referred to us for continuous Pcsf monitoring between 1991 and 2000. Pcsf was measured via a lumbar catheter and analysed for mean, peak, highest pulse amplitude and abnormal waveforms. A 1-2 day trial of continuous controlled CSF drainage (10 cc/ h) followed Pcsf monitoring. Response to CSF drainage was defined as improvement in headache symptoms. Patients with abnormal waveforms underwent a ventriculoperitoneal (VPS) or lumboperitoneal (LPS) shunt insertion. All patients had normal resting Pcsf (8 +/- 1 mmHg) defined as ICP < 15 mmHg. During sleep, all patients had B-waves and 90% had plateau waves or near plateau waves. All patients underwent either a VPS or LPS procedure. All reported improvement of their headache after surgery. Demonstration of pathological Pcsf patterns by continuous Pcsf monitoring was essential in confirming the diagnosis of IIHWOP, and provided objective evidence to support the decision for shunt surgery. Increased Pcsf was seen mostly during sleep and was intermittent, suggesting that Pcsf elevation may be missed by a single spot-check LP measurement. The similarity between IIHWOP and CDH suggests that continuous Pcsf monitoring in CDH patients may have an important diagnostic role that should be further investigated.

Glutamate and nitric oxide pathway in chronic daily headache: evidence from cerebrospinal fluid.

A central sensitization has been advocated to explain chronic daily headache (CDH) due to sustained peripheral sensitization of allogenic structures responsible for sustained trigeminovascular system activation. Several mechanisms have been suggested to underlie central sensitization, but have been poorly investigated in CDH. They involve N-methyl-D-aspartate (NMDA) receptor activation and nitric oxide (NO) production and supersensitivity and increased and maintained production of sensory neuropeptides. The present study supports the above pathogenic mechanisms demonstrating a significant increase in glutamate and nitrite levels in the CSF of CDH patients, without a significant difference between patients without and those with analgesic overuse headache (P < 0.0001 and P < 0.002). The increase in CSF nitrites was accompanied by a significant rise in the CSF values of cyclic guanosine monophosphate (cGMP) in patients in comparison with controls (P < 0.0001). A statistically significant correlation emerged between visual analogic scale (VAS) values and glutamate, nitrites and cGMP. Although substance P (SP) and calcitonin gene-related peptide (CGRP), and to a lesser extent neurokinin A, were significantly increased in CSF compared with control subjects, their values did not correlate with glutamate, nitrites and cGMP levels in CSF in the patient group. The present study confirms the involvement of glutamate-NO-cGMP-mediated events underlying chronic head pain that could be the target of a new therapeutic approach which should be investigated.

Spontaneous CSF leaks mimicking benign exertional headaches.

Spontaneous CSF leaks are increasingly recognized, and a broader clinical and imaging spectrum of the disorder is emerging. The headaches of CSF leaks are typically orthostatic, but sometimes especially with chronicity the orthostatic features are blurred into lingering chronic daily headaches. Additional types of headache are also increasingly recognized. Two patients with spontaneous CSF leaks presented with intermittent transient severe headaches provoked by Valsalva-type manoeuvres. Orthostatic features were absent and the patients were asymptomatic if they avoided the provoking manoeuvres. One patient had been treated for 6 years for benign exertional headaches and had failed many medical treatments, including courses of indomethacin. He was found to have a leak from cribriform plate. The second patient had been symptomatic for several months, had diffuse pachymeningeal gadolinium enhancement on head magnetic resonance imaging, spinal meningeal diverticula, and CSF leak at the thoracic spine level. Headaches that mimic benign exertional headaches are yet another mode of the still broadening clinical presentation of spontaneous CSF leaks.

The impact of HIV infection on primary headache. Unexpected findings from retrospective, cross-sectional, and prospective analyses.

Headache is one of the most important factors influencing the quality of life in patients infected with the human immunodeficiency virus type 1 (HIV). However, only symptomatic headache but not changes or primary headache types during HIV infection have been studied to date. Therefore, we aimed to determine the impact of an HIV infection on frequency and semiology of different primary headache types. Patients with confirmed HIV type 1 infection underwent a neurological examination, neuroimaging or EEG, and a standardized interview. Time pattern and symptoms of headaches (cross-sectional analysis), changes of headaches preexisting to their infection (longitudinal retrospective analysis), and changes of primary headaches during a 2-year follow-up (longitudinal prospective analysis) were evaluated as were the correlations between these headache patterns and different markers of HIV infection. One hundred thirty-one consecutive HIV-infected patients without evidence of a cerebral manifestation except mild encephalopathy were enrolled. The point prevalence of migraine was 16.0% (confidence interval (CI) 10.1-25.4%), of headache with a semiology of tension-type headache 45.8% (CI 33.7-62.2%), and of other headache types 6.1% (CI 3.0-12.5%). During the natural course of infection, the migraine frequency significantly decreased in the retrospective and in the prospective analyses, whereas the frequency of the headache with a semiology of tension-type headache significantly increased in all three analyses. In 20% of all patients, the tension-type headache could be considered as symptomatic due to the infection but not due to focal or general cerebral lesions. Changes of primary headache were significantly associated with different stages of the infection and with the presence of mild encephalopathy but not with antiretroviral treatment or CD4 cell count. HIV infection seems to be associated with a progressive decrease in migraine frequency and intensity which probably is related to the immunological state of the patients. Tension-type headache becomes more frequent during HIV infection. However, this can in part be related to secondary headache caused by the HIV in less than 50% of patients with tension-type headache. The progressing immunological deficiency of HIV-infected patients seems to influence pain processing of primary headache types in different ways.