Evaluation of acute headaches in adults

Classifying headaches as primary (migraine, tension-type or cluster) or secondary can facilitate evaluation and management A detailed headache history helps to distinguish among the primary headache disorders. "Red flags" for secondary disorders include sudden onset of headache, onset of headache after 50 years of age, increased frequency or severity of headache, new onset of headache with an underlying medical condition, headache with concomitant systemic illness, focal neurologic signs or symptoms, papilledema and headache subsequent to head trauma. A thorough neurologic examination should be performed, with abnormal findings warranting neuroimaging to rule out intracranial pathology. The preferred imaging modality to rule out hemorrhage is noncontrast computed tomographic (CT) scanning followed by lumbar puncture if the CT scan is normal. Magnetic resonance imaging (MRI) is more expensive than CT scanning and less widely available; however, MRI reveals more detail and is necessary for imaging the posterior fossa. Cerebrospinal fluid (CSF) analysis can help to confirm or rule out hemorrhage, infection, tumor and disorders related to CSF hypertension or hypotension. Referral is appropriate for patients with headaches that are difficult to diagnose, or that worsen or fail to respond to management     

Jolt Accentuation of Headache: The Most Sensitive Sign of CSF Pleocytosis

We prospectively examined the clinical signs of 54 febrile patients associated with recent-onset headache. They underwent lumbar puncture (LP) on suspicion of meningitis. The relation of each sign to cerebrospinal fluid (CSF) pleocytosis was estimated. Among 34 patients with pleocytosis, 33 had jolt accentuation (sensitivity: 97.1%), while only 5 of them had neck stiffness or Kernig's sign. Among 20 patients without pleocytosis, 12 had no jolt accentuation (specificity: 60%). We found jolt accentuation to be the most sensitive sign of CSF pleocytosis. If jolt accentuation is noted in a febrile patient associated with recent onset headache, the CSF should be examined even in the absence of neck stiffness or Kernig's sign.     

Symptomatische Kopfschmerzen

A patient is suspected of suffering from a potentially life threatening headache according to the following typical warning signs: sudden onset headache, worst headache, neurological findings, fever, neck stiffness, epileptic seizures and deterioration of vigilance. It is far more dangerous not to recognize potentially life threatening headaches like subarachnoid bleeding, meningitis, encephalitis, arterial dissection or epidural hematoma than to over diagnose it. Immediate and specific neurological and radiological investigations (CT, MRI, CSF) are necessary in each patient presenting with those findings. The morbidity and mortality of those patients mainly depend on the beginning of the therapy. This article focuses on the diagnosis of potentially life threatening headache disorders.     

Clues in the Differential Diagnosis of Primary vs Secondary Cough,Exercise, and Sexual Headaches

Activity‐related headaches can be provoked by Valsalva maneuvers (“cough headache”), prolonged exercise (“exertional headache”) and sexual excitation (“sexual headache”). These entities are a challenging diagnostic problem as can be primary or secondary and the etiologies for secondary cases differ depending on the headache type. In this paper we review the clinical clues which help us in the differential diagnosis of patients consulting due to activity‐related headaches. Cough headache is the most common in terms of consultation. Primary cough headache should be suspected in patients older than 50 years, if pain does not predominate in the occipital area, if pain lasts seconds, when there are no other symptoms/signs and if indomethacin relieves the headache attacks. Almost half of cough headaches are secondary, usually to a Chiari type I malformation. Secondary cough headache should be suspected in young people, when pain is occipital and lasts longer than one minute, and especially if there are other symptoms/signs and if there is no response to indomethacin. Every patient with cough headache needs cranio‐cervical MRI. Primary exercise/sexual headaches are more common than secondary, which should be suspected in women especially with one episode, when there are other symptoms/signs, in people older than 40 and if the headache lasts longer than 24 hours. These patients must have quickly a CT and then brain MRI with MRA or an angioCT to exclude space‐occupying lesions or subarachnoid hemorrhage.     

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.     

Headache resembling tension-type headache as the single manifestation of Lyme neuroborreliosis

We present two patients with monosymptomatic headache resembling chronic tension-type headache as the first manifestation of Lyme neuroborreliosis. The headache developed over a few days in both cases and lasted for three months in the first case and for two and a half years in the second case before the diagnosis of Lyme neuroborreliosis was made. Neuroimaging and many laboratory investigations did not lead to the diagnosis, which was only established after lumbar puncture. The CSF in both cases showed high protein, lymphocytic pleocytosis and Borrelia burgdorferi-specific intrathecal antibody synthesis. The headache disappeared completely after treatment with penicillin G. In patients suffering from daily headaches which have developed subacutely, Lyme neuroborreliosis should be considered even in the absence of signs of meningeal irritation. A lumbar puncture should be performed more often than is presently customary and the CSF should be examined for pleocytosis as well as Borrelia burgdorferi -specific intrathecal antibody synthesis.     

Sudden onset headache: a prospective study of features,incidence and causes

Sudden onset headache is a common condition that sometimes indicates a life-threatening subarachnoid haemorrhage (SAH) but is mostly harmless. We have performed a prospective study of 137 consecutive patients with this kind of headache (thunderclap headache=TCH). The examination included a CT scan, CSF examination and follow-up of patients with no SAH during the period between 2 days and 12 months after the headache attack. The incidence was 43 per 100 000 inhabitants >18 years of age per year; 11.3% of the patients with TCH had SAH. Findings in other patients indicated cerebral infarction (five), intracerebral haematoma (three), aseptic meningitis (four), cerebral oedema (one) and sinus thrombosis (one). Thus no specific finding indicating the underlying cause of the TCH attack was found in the majority of the patients. A slightly increased prevalence of migraine was found in the non-SAH patients (28%). The attacks occurred in 11 cases (8%) during sexual activity and two of these had an SAH. Nausea, neck stiffness, occipital location and impaired consciousness were significantly more frequent with SAH but did not occur in all cases. Location in the temporal region and pressing headache quality were the only features that were more common in non-SAH patients. Recurrent attacks of TCH occurred in 24% of the non-SAH patients. No SAH occurred later in this group, nor in any of the other patients. It was concluded that attacks caused by a SAH cannot be distinguished from non-SAH attacks on clinical grounds. It is important that patients with their first TCH attack are investigated with CT and CSF examination to exclude SAH, meningitis or cerebral infarction. The results from this and previous studies indicate that it is not necessary to perform angiography in patients with a TCH attack, provided that no symptoms or signs indicate a possible brain lesion and a CT scan and CSF examination have not indicated SAH.     

Viral encephalitis

Viral encephalitis presents with fever, headache, focal and generalized neurologic symptoms and signs, seizures, and CSF pleocytosis. Herpes simplex Virus (HSV) 1 and arboviruses (flaviruses) are the most common causes of encephalitis in Switzerland. The initial work-up in a suspected encephalitis includes CSF analysis, EEG, and brain CT or MRI. The identification of the responsible agent usually occurs with polymerase chain reaction or serology. The differential diagnosis to other infectious and non-infectious acute CNS-disorders may initially be arduous. A specific treatment is possible only in encephalitis caused by viruses of the herpes group. Active immunization should be considered in subjects at high risk for tick-borne encephalitis. With early treatment the prognosis may be satisfactory also in HSV encephalitis.     

Repeat CT or MRI in Posttraumatic Headache

Repeat CT or MRI of the brain should be considered in posttraumatic headache. We describe two patients with posttraumatic headache who had negative CT scans on initial presentation. One patient later had bilateral subdural hematomas on CT, and the other had temporal lobe hemorrhage on MRI. We recommend considering repeat CT or MRI for persisting posttraumatic headache and mental status change.     

Headaches due to cerebrovascular disease

Subarachnoid hemorrhage causes sudden, severe headache and requires immediate medical and surgical diagnosis and treatment. A CT scan is the first choice for correct diagnosis. In order to prevent rebleeding, delays in treatment should be avoided. Intraparenchymal cerebral hemorrhage is now often recognized by means of CT scanning and sometimes is a cause of headache. Cerebellar hemorrhage commonly causes occipital headache and is an indication for immediate surgical intervention, although small cerebellar hemorrhages can be treated conservatively. Ischemic cerebrovascular disease is frequently accompanied by headache, but its etiology remains uncertain. Thrombosis of the cerebrovenous system is a less frequent cause of head pain than that of the arterial system, but it usually shows characteristic neurologic signs. Following carotid endarterectomy or superficial temporal artery-middle cerebral artery bypass surgery, the patient may have moderate to severe unilateral headaches, probably as a result of platelet aggregation and serotonin release.     

Trendelenburg position: a tool to screen for the presence of a low CSF pressure syndrome in daily headache patients

Objective.— To test the hypothesis that the Trendelenburg position is an accurate screening investigation for the presence of a low cerebrospinal fluid (CSF) pressure syndrome in patients with daily headache. Background.— The Trendelenburg position causes a rapid increase in intracranial CSF pressure. In a patient with a known CSF leak who overtime had less improvement in the supine position, being placed in Trendelenburg rapidly alleviated her daily headache. This suggested that the Trendelenburg position might be a good screening tool for low CSF pressure syndromes. Methods.— Case reports. All patients were placed in the Trendelenburg position (10°‐20° head‐down tilt) for 5 minutes. A patient was considered to have a positive Trendelenburg test if they experienced complete pain freedom or substantial improvement in baseline head pain in the Trendelenburg position. Results.— Case patients are presented for 3 clinical scenarios: Scenario 1: Daily headache with or without a positional component with a positive response to the Trendelenburg position and subsequent evidence of an underlying low CSF pressure syndrome. Scenario 2: Daily headache with a strong positional component but no improvement in the Trendelenburg position and a negative evaluation for a low CSF pressure syndrome. Scenario 3: Trendelenburg position proves the existence of a post‐lumbar puncture headache in patients with near‐daily headaches. Conclusion.— The Trendelenburg position appears potentially useful as a clinical tool to screen for the presence of a low CSF pressure syndrome in patients with daily headache.     

What Differences Exist in the Appropriate Treatment of Congenital Versus Acquired Adult Chiari Type I Malformation?

Chiari type I malformation is found in 1 out of 20 magnetic resonance imaging (MRI) studies. Isolated tonsillar herniation is of limited utility and should be considered within the clinical context because these patients can be asymptomatic. Cine MRI showing compression of the cerebrospinal fluid (CSF) spaces in the foramen magnum area is a crucial technique for making treatment decisions. Congenital malformation is thought to be due to a volumetric small posterior fossa. The most common symptom in these patients is cough headache. Posterior fossa reconstruction is mandatory in patients with progressive symptoms/signs, hydrocephalus, or syringomyelia, but not in patients who are asymptomatic or those with stable and tolerable symptoms. Acquired tonsillar descent can be secondary to a variety of disorders conditioning disproportion between the volume of the cranial cavity and that of the intracranial contents, or to CSF hypovolemia, which is the most common cause for acquired herniation. CSF hypovolemia can be spontaneous or secondary to CSF removal. Treatment of acquired tonsillar herniation depends on the responsible etiology.     

The Transient Syndrome of Headache With Neurologic Deficits and CSF Lymphocytosis. Report of a Case Without Severe Headache

It has been recently reported that the occurrence of severe headache associated with temporary neurologic deficits and CSF lymphocytic pleocytosis is highly suggestive of the so-called "transient syndrome of headache with neurologic deficits and CSF lymphocytosis." In particular, in almost all of the 40 patients reported in the literature to date, the head pain was severe and of a type not previously experienced by the patient. In the present case report, we describe a patient who fulfilled almost all the proposed diagnostic criteria, except for the lack of a severe headache. Probably, a severe headache is not a compulsory feature of this syndrome. Some patients have rather mild headache accompanying their episodes of neurologic symptoms, and some attacks occur without any accompanying headache. It is possible that in some cases the absence of a severe headache, and thus the lack of CSF analysis, lead to misdiagnosis. Therefore, the prevalence of this syndrome could be underestimated.     

Chronic daily headache: When to suspect meningitis

Millions of patients see physicians each year for headache, most of which are primary headaches. However, serious secondary headaches, such as meningitis, represent about 5% of children and 1% to 2% of adults seen in the emergency department for headache. A primary care or emergency department physician may initially miss individuals with bacterial meningitis. Considering meningitis as a headache cause is important because delay in the diagnosis may have adverse consequences. A careful history and physical examination are central in identifying individuals at high risk for meningitis. This article lists information that can be obtained from the patient that may be indicative of meningitis. Performing a lumbar puncture with appropriate examination of the cerebrospinal fluid (CSF) is the key to establishing the diagnosis of meningitis. This article also includes the types of meningitis that should be considered when the CSF demonstrates a pleocytosis.     

The radiology of headache

The patient who presents with a severe and acute headache should be evaluated radiographically with CT. The key diagnosis to make in this situation is hemorrhage, either subarachnoid or intraparenchymal. Computed tomography is more sensitive to acute hemorrhage than is MRI. When the patient is stable, MRI frequently contributes information to narrow the diagnostic possibilities, because vascular malformations and certain parenchymal lesions have a characteristic appearance on MRI. Hydrocephalus may also present acutely and is easily seen on CT or MRI. In a patient may show WMF and atrophy. The patient with trigeminal neuropathy may demonstrate central or peripheral lesions. In temporomandibular joint dysfunction, conventional tomography and MRI are frequently used. Magnetic resonance imaging shows excellent detail of the disk and surrounding soft tissues, whereas tomography better demonstrates bony changes. When a history of trauma is present, MRI may show a subacute subdural hematoma. These collections are easily seen on MRI, even when isodense on CT. Evidence of old shear injury is also well seen on MRI. Finally, neoplastic, inflammatory, congenital, and idiopathic sources of headache may be demonstrated by either MRI or CT, depending on presentation. MRI will generally show superior characterization.     

艾滋病并发隐球菌脑膜炎14例临床分析

目的分析艾滋病(AIDS)并发隐球菌脑膜炎临床特点,为临床诊治提供信息。方法对近3年北京佑安医院收治的14例AIDS并发隐球菌脑膜炎患者的临床资料进行回顾性分析。结果 14例患者均以头痛为主诉入院,10例(71.4%)有发热、呕吐等主要伴随症状,脑膜刺激征及视神经乳头水肿的发生率也较高,分别为7例(50.0%)和8例(57.1%),抽搐及意识障碍等脑实质受损的表现发生率较低,分别为3例和2例。85.7%(12/14)的患者CD4+T淋巴细胞<100/μL。85.7%(12/14)的患者颅内压增高,其中一半患者脑脊液压力大于35 cmH2O,13例(92.9%)患者的脑脊液细胞数和9例患者(64.3%)的脑脊液蛋白水平轻度增高,6例(42.9%)脑脊液葡萄糖水平减低,8例(57.1%)氯化物水平减低。11例(78.6%)脑脊液隐球菌涂片阳性,6例(42.9%)脑脊液隐球菌培养阳性,12例(85.7%)脑脊液隐球菌荚膜多糖抗原检测阳性。4例(28.6%)头颅CT或核磁共振检查表现异常,包括脑实质损伤2例,脑积水1例及占位性病变1例。经治疗2例(14.3%)死亡,1例自动出院,11例病情好转出院,平均住院天数为60 d。结论 AIDS并发隐球菌脑膜炎的患者多以头痛为主诉,伴发热及颅内压增高等表现多见,而脑实质损伤的表现较少。患者的CD4+细胞数多低于100/μL,脑脊液压力大多明显增高,隐球菌抗原检测灵敏度较高,而影像学检查很少有特异性发现。该病目前的诊断及治疗仍相对困难,临床医师应当予以更多的重视。     

Atypical headache after prolonged treatment with nasal continuous positive airway pressure

We report a case of atraumatic pneumocephalus associated with prolonged use of nasal continuous positive airway pressure. Initial symptoms included headache, ataxia, vertigo, and a "gurgling" sensation in the head; and a CT image showed small air bubbles along the falx of cerebrum and adjacent to the temporal epidural spaces bilaterally. Although no evidence of cerebrospinal fluid (CSF) leak was either reported by the patient or found at initial clinical examination, subsequent nasal discharge tested positive for beta2-transferrin, a finding consistent with CSF leak in the paranasal sinus region or through the cribriform plate. To try to prevent infection from an open communication between the paranasal sinuses and intracranial structures, an attempt should be made to localize the anatomic defect.     

Prevalence of Herniation and Intracranial Shift on Cranial Tomography in Patients With Subarachnoid Hemorrhage and a Normal Neurologic Examination

Objectives: Patients frequently present to the emergency department (ED) with headache. Those with sudden severe headache are often evaluated for spontaneous subarachnoid hemorrhage (SAH) with noncontrast cranial computed tomography (CT) followed by lumbar puncture (LP). The authors postulated that in patients without neurologic symptoms or signs, physicians could forgo noncontrast cranial CT and proceed directly to LP. The authors sought to define the safety of this option by having senior neuroradiologists rereview all cranial CTs in a group of such patients for evidence of brain herniation or midline shift. Methods: This was a retrospective study that included all patients with a normal neurologic examination and nontraumatic SAH diagnosed by CT presenting to a tertiary care medical center from August 1, 2001, to December 31, 2004. Two neuroradiologists, blinded to clinical information and outcomes, rereviewed the initial ED head CT for evidence of herniation or midline shift. Results: Of the 172 patients who presented to the ED with spontaneous SAH diagnoses by cranial CT, 78 had normal neurologic examinations. Of these, 73 had initial ED CTs available for review. Four of the 73 (5%; 95% confidence interval [CI] = 2% to 13%) had evidence of brain herniation or midline shift, including three (4%; 95% CI = 1% to 12%) with herniation. In only one of these patients was herniation or shift noted on the initial radiology report. Conclusions: Awake and alert patients with a normal neurologic examination and SAH may have brain herniation and/or midline shift. Therefore, cranial CT should be obtained before LP in all patients with suspected SAH. ACADEMIC EMERGENCY MEDICINE 2010; 17:423–428 © 2010 by the Society for Academic Emergency Medicine     

Spontaneous Intracranial Hypotension: Headache With a Reversible Arnold-Chiari Malformation

Spontaneous intracranial hypotension is characterized by severe postural headache in the setting of low CSF pressure, usually attributed to a cryptic CSF leak. We report a patient whose prolonged refractory headache was characterized by the clinical symptoms of occipital neuralgia, but was also associated with the radiographic appearance of an Arnold-Chiari malformation, type I and low CSF pressure. After extensive diagnostic evaluation, CT cisternomyelography ultimately demonstrated a CSF leak at the C2 vertebral level. Symptomatic relief was sustained only with long-term theophylline administration. The apparent Arnold-Chiari malformation resolved with treatment of the low CSF pressure.     

脑静脉窦血栓形成的诊断及治疗

目的:探讨脑静脉窦血栓形成(CVST)的临床特点。方法:对20例CVST患者的一般情况、病因、临床表现、脑脊液特点、影像学特征、治疗及预后等资料进行回顾性分析。结果:20例患者多表现为头痛,可伴有癫疒间发作和各种神经功能缺损的症状体征,脑脊液压力明显升高,白细胞数和蛋白质定量可正常或升高,头颅CT示静脉窦高密度改变及脑实质异常信号,MRI示静脉窦异常信号,MRV示静脉窦闭塞或充盈缺损,DSA示静脉窦狭窄、血流中断或不显影。治疗以脱水、抗凝、溶栓为主,19例好转,1例死亡。结论:对于高颅压伴或不伴神经、精神障碍的患者,须高度警惕CVST,应尽早行MRV或DSA检查,治疗以抗凝、溶栓为主。