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.     

Vertebral artery dissection onset mimics migraine with aura in a graphic designer

Headache is the most common symptom in patients with cervical artery dissection. This symptom, however, rarely occurs in isolation, and more commonly is associated with other neurological symptoms and signs. Visual symptoms associated with vertebral artery dissection (VD) have also been observed, but do not typically mimic the migraine aura. Here, we report a young patient who presented VD, embolic ischemic lesions, and visual symptoms with the features of aura, followed by migraine headache. The suggestion of VD dissection should be kept in mind in those cases complaining of the first attack of headache that mimics migraine with aura. In these cases, an extensive neuroimaging study is advisable.     

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.     

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.     

Confusional state in the syndrome of HaNDL

(Headache 2011;51:1285‐1288) Background.— The association of headache with transient neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL) is recognized as a distinct benign, self‐limited headache syndrome. Aphasic, sensory and motor disturbances predominate the clinical picture and to our knowledge, only 2 detailed cases of confusion and agitation have been previously described. Case.— We present our recent experience with 2 cases of the HaNDL syndrome who, in addition to the focal neurological deficits, developed confusional state of variable degree, with no signs of aphasia that could jeopardize the clinical picture. An extensive laboratory and neuroimaging work‐up excluded all other possible entities and both patients treated conservatively showed an excellent functional recovery. Conclusion.— We suggest that, although the HaNDL syndrome has a focal plateau, explaining the focal deficits; diffuse manifestations in the form of confusion may well be part of the clinical spectrum of this disorder.     

结核性脑膜炎神经体征转归的相关因素分析

目的：评价影响结核性脑膜炎神经功能缺失体征转归的临床因素。方法：对我院神经内科1998～2003年收治的45例入院时有神经功能缺失体征的结核性脑膜炎患者的临床、生化特征、治疗1a后的神经功能恢复情况进行观察研究。结果：45例患者中神经功能缺失体征完全或部分恢复22例（48．9％），加重或无变化者23例（51．1％）。加重或无变化病例中14例（31．1％）仍留有明显神经系统残疾，9例（20．0％）死亡。多因素统计结果显示，患者开始抗结核治疗时间与神经功能缺失体征转归密切相关（OR=1．843．P＜0．05），其他变量（性别、年龄、肺结核病史、脑积水、脑梗死、脑脊液自细胞数、脑脊液蛋白含量、激素应用等）的差异无显著性。结论：患者发病后开始抗痨治疗时间是影响本组患者神经功能缺失体征转归的重要因素．因此延误诊断和治疗仍是目前引起结核性脑膜炎神经系统残疾和死亡的重要原因。     

Headache as a surrogate marker of the molecular mechanisms implicated in progressing stroke

The mechanism for headache in patients with acute ischaemic stroke are not completely understood. We analysed the relationship between headache and the early worsening of neurological symptoms in patients with acute ischaemic stroke, and we studied the possible biochemical mechanisms implicated. Headache at the onset of ischaemic stroke predicted progression with a sensitivity, specificity, and positive predictive value of 56%, 99%, and 98%, respectively. CSF concentrations of glutamate, Interleukin-6, and NO-m were significantly greater in patients with progressing stroke than in patients with nonprogressing stroke, and these biochemical markers were also significantly higher in patients with headache than in those without headache. Results of this study suggest that headache at the onset of ischaemic stroke is an independent predictor of neurological worsening and we hypothesize that headache might be a surrogate marker of the molecular mechanisms involved in neurological worsening after acute stroke.     

Clinical and Angiographic Features of Thunderclap Headache

Thunderclap headache is an acute high intensity headache similar to that seen in the setting of ruptured saccular aneurysm. We report four patients without subarachnoid hemorrhage who presented with thunderclap headache. Three patients had transient neurologic signs or symptoms. Cerebral angiography revealed diffuse segmental intracerebral arterial vasoconstriction which was reversible in the one patient in whom angiography was repeated. The headaches resolved spontaneously in all cases and after 1 week did not recur. These cases highlight the specific clinical and angiographic features and the self-limited course of patients with thunderclap headache and suggest that thunderclap headache may represent a unique headache category. On the basis of these cases and others reported in the literature, we propose diagnostic criteria for thunderclap headache.     

Headaches and Arnold-Chiari Syndrome: When to Suspect and How to Investigate

Migraine and tension-type headache are common clinical problems, occurring even at a young age. When patients report headache as a symptom, it is necessary to exclude a secondary headache induced by an organic disease. Proper diagnosis and management of headache depends on a thorough history review and comprehensive clinical examination. A Chiari malformation is one organic cause that should not be overlooked. A thorough clinical screening is always recommended, including a complete neurological, mental status and physical examination. However, when the symptom pattern suggests a Chiari malformation, neuroimaging is warranted to identify correctly the pathologic condition and the most appropriate therapeutic approach. This paper reviews this condition, the signs and symptoms suggestive of its presence and how to arrive a the proper diagnosis.     

The significance of age, sex, and cerebrospinal fluid pressure in post-lumbar-puncture headache

In this study of 300 neurological inpatients aged between 18 and 60 years the incidence of post-lumbar-puncture headache (PPH) was 37.3%. The more severe the headache, the more frequently it was associated with dizziness, nausea, vomiting, and tinnitus. If PPH occurred during the first day after lumbar puncture (LP), it was more severe, and lasted longer than PPH, which started later. The incidence of PPH and associated symptoms decreased with increasing age, and was much higher in females than males. The sex difference was nearly exclusively explained by a marked preponderance of PPH in females below 40 years of age, i.e. women in the fertile age. Furthermore, there was a decreased incidence of PPH and associated symptoms in patients with an initial higher than average cerebrospinal fluid (CSF) pressure (162 mm H2O). All these differences were statistically significant. Particularly high frequencies of PPH were found in young women with an initial CSF pressure lower than mean.     

Cerebrospinal fluid and serum neuron-specific enolase in acute benign headache

We determined the cerebrospinal fluid (CSF) and serum neuron-specific enolase (NSE) concentrations in 19 patients with acute benign headache. All patients had normal neurological examination, CSF and head computed tomography scan. The final diagnoses were: primary thunderclap headache ( n  = 7), primary exertional headache ( n  = 3), primary cough headache ( n  = 1), migraine without aura ( n  = 4), headache unspecified ( n  = 2), probable infrequent episodic tension-type headache ( n  = 1), headache attributed to hypertensive crisis without hypertensive encephalopathy ( n  = 1). A group of 108 healthy subjects served as controls. CSF NSE concentration was 14.16 ng/ml [95% confidence interval (CI) 11.86, 16.47)] in the headache sample (controls 17.19 ng/ml, 95% CI 16.23, 18.15). Serum NSE concentration was 7.50 ng/ml (95% CI 5.20, 9.80) in the headache sample (controls 8.45 ng/ml, 95% CI 7.67, 9.23). CSF/serum ratio was 2.81 (95% CI 2.21, 3.40) in the headache sample (controls 2.23, 95% CI 2.03, 2.42). Acute benign headache is not associated with neuronal damage as estimated by means of CSF and serum NSE concentration.     

Thunderclap headache attributed to reversible cerebral vasoconstriction: view and review

Thunderclap headache attributed to reversible cerebral vasoconstriction (THARCV) is a syndrome observed in a number of reported cases. In this article we reviewed this new headache entity (idiopathic form) using the clinical-radiological findings of 25 reported patients. In this series of patients 72% were women, the mean age at the onset of first headache episode was 39.4 +/- 2.3 years. In addition to the sine qua non condition of being abrupt and severe (thunderclap) at the onset, the headache was usually described as being explosive, excruciating, or crushing. The feature of pulsatility, accompanied or not by nausea was described by 80% of the patients. Forty percent of the cases manifested vomiting and 24% photophobia. Usually the headache was generalized, and in three cases it was unilateral at least at the onset. In 21 of 25 patients (84%) there was at least one recurrence or a sudden increase in the intensity of the headache. A past history of migraine was present in 52% of the patients. Precipitating factors were identified in 56% of the patients. Sexual intercourse was described by six patients. Of the 25 patients with THARCV syndrome studied, 12 (48%) developed focal neurological signs, transitory ischemic attack (n = 1), or ischemic stroke (n = 11, 44%), and two (8%) of them manifested seizures. The THARCV syndrome is a neurological disturbance perhaps more frequent than expected, preferentially affecting middle aged female migraineurs, and having an unpredictable prognosis, either showing a benign course or leading to stroke.     

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.     

Diagnosis of subarachnoid hemorrhage in the emergency department

To decide which patients with headache ought to be evaluated for SAH, physicians should focus on specific elements of the patient history, such as onset, severity, and quality of the headache and associated symptoms. These questions should be asked and the responses documented for every patient with a headache. The physical examination should be compulsive with regard to vital signs, HEENT. and neurologic signs. Then, the physician should form an explicit differential diagnosis and have reasons for diagnosing migraine, tension, or sinus headache and other benign causes. If there is no clear-cut alternative hypothesis, the patient should be evaluated by CT and LP (if the CT is negative, equivocal, or technically inadequate). Physicians should understand the limitations of this diagnostic algorithm. In addition, the CSF should be carefully analyzed, including measuring the opening pressure. In patients whose CT scans and CSF analyses are normal, further testing is rarely indicated.     

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.     

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.     

The risks of sumatriptan administration in patients with unrecognized subarachnoid haemorrhage (SAH)

Administration of sumatriptan in subarachnoid haemorrhage (SAH) patients, misdiagnosed as migraine patients, may induce symptomatic cerebral vasospasm with potentially dangerous consequences. Over a 5-year period, we observed three patients with a 3-15-year history of migraine, who received sumatriptan for acute headache. Two patients received 6 mg sumatriptan subcutaneously on days 4 and 6, and one patient 3 x 100 mg sumatriptan orally on day 1 after an acute headache episode. In all three cases, an alleviation of headache intensity from severe to moderate was observed. When headache recurred and meningeal signs appeared, SAH was diagnosed by computed tomography in all three cases. No neurological deficits occurred during the further course of the disease. In both patients with a SAH caused by an aneurysm, transcranial Doppler sonography demonstrated vasospasm of the basal cerebral arteries. An antinociceptive effect of sumatriptan can be observed in SAH patients in good clinical condition, which suggests a specific craniovascular antinociceptive action. This may lead to misdiagnosis as migraine and delayed appropriate diagnosis and treatment.     

Idiopathic spontaneous subdural hemorrhage

We describe three middle-aged and previously healthy patients in whom idiopathic spontaneous subdural hemorrhage occurred, giving rise to headache with minimal or no neurological signs and resolving with conservative management. Previous reports of this condition describe an associated severe neurological impairment, a poor prognosis, and a necessity for surgical evacuation. Our eases indicate that mild and self-limiting spontaneous subdural hemorrhage may occur and is perhaps underdiagnosed in our patients' age group because it presents non-specifically with headache.     

Detection of Toxoplasma gondii by polymerase chain reaction in cerebrospinal fluid from human immunodeficiency virus-1-infected Japanese patients with focal neurological signs

We aimed to determine the effectiveness of using the polymerase chain reaction (PCR) to detect Toxoplasma gondii in cerebrospinal fluid (CSF) specimens from Japanese patients infected with human immunodeficiency virus (HIV)-1. Twenty-six HIV-positive individuals presenting with focal neurological signs and a possible diagnosis of T. gondii encephalitis (TE) were enrolled in the study between April 1997 and March 2003. Eight patients were diagnosed as having TE using the accepted diagnostic criteria; PCR amplified the T. gondii B1 gene in CSF samples from five of these eight patients. CSF samples from the 18 patients without TE were negative for T. gondii DNA. The sensitivity, specificity and positive and negative predictive values for detecting T. gondii in CSF using PCR were 62.5%, 100%, 100% and 85.7%, respectively. These results suggest that PCR might be a clinically useful technique for detecting T. gondii DNA in patients infected with HIV showing focal neurological signs. Improvements in sensitivity are needed, however.     

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。