Cryptococcal meningitis due to Cryptococcus gattii in an immunocompetent patient: uncommon presentation of a thunderclap headache.

Thunderclap headache is a medical emergency presented as the worst headache ever, is characterised by an abrupt onset and maximal intensity within seconds to minutes. However, cerebrovascular causes are among the most common causes of thunderclap headache, and other non-vascular life-threatening aetiologies should be considered in evaluating a patient. We describe a 23-year-old previously healthy Latino woman who presented to our hospital after a month of repetitive severe, abrupt-onset headaches. Her prior medical history was unremarkable. After a normal brain MRI with angio-MRI, a lumbar puncture was performed with normal opening pressure, hypoglycorrhachia, increased proteins and a leucocyte; India ink staining was positive for encapsulated yeast, cultures were positive for Cryptococcus gattii The patient received appropriate antifungal treatment with a good response. This case highlights the particular presentation of cryptococcal meningitis due to C. gattii among immunocompetent patients.

Thunderclap Headache in Children and Adolescents.

PURPOSE OF REVIEW: This work aimed to review the epidemiology, clinical criteria, and primary and secondary diagnoses of pediatric thunderclap headache and to compare to adult thunderclap headache. RECENT FINDINGS: Thunderclap headache among children aged 6-18 years are rare; this headache presented in 0.08% of the patients admitted to a pediatric emergency department in a tertiary pediatric center. In that recent single-center study, thunderclap was a headache of grade 10 on the pain scale and conferred a benign course. Contrary to adults, in children, most thunderclap headaches are due to either a primary thunderclap headache or another type of primary headache. A number of case reports have attributed pediatric thunderclap to reversible vasoconstriction syndrome and bleeding due to intracranial aneurysm. However, 3-year data from a pediatric emergency department of one center did not find these reasons to be causes of secondary thunderclap headache. This may be due to the rarity of these diagnoses in children compared to adults. Four of the 19 patients with thunderclap headache reported in that single study had secondary thunderclap; the causes were infection in three and malignant hypertension in one. All the patients had a benign course. Although urgent imaging and lumbar puncture are required in the workup of pediatric thunderclap, severe causes are very rare. More research is needed to investigate pediatric thunderclap headache.

Reversible Cerebral Vasoconstriction Syndrome in the Setting of COVID-19 and Pleomorphic Sarcoma: A Case Report.

INTRODUCTION: Reversible cerebral vasonstriction syndrome (RCVS) is an increasingly recognized clinical and radiologic syndrome. However, it has been rarely reported in the setting of the novel coronavirus disease-2019 (COVID-19) infection or sarcomatous tumors. RCVS might be the initial manifestations of COVID-19 infection or noncatecholamine producing masses including sarcoma. CASE REPORT: A 44-year-old male who developed COVID-19-related symptoms followed by rapid onset of severe headaches in the setting of persistently elevated blood pressure (BP). Brain imaging showed multifocal arterial narrowing in the anterior and posterior circulation consistent with RCVS. Serial imaging demonstrated resolution of the arterial narrowing after BP control was achieved with improvement in the patient's headaches. Further investigation for secondary causes of the patient's elevated BP revealed a right renal mass, and the patient underwent right nephrectomy, and the biopsy results confirmed the diagnosis of pleomorphic sarcoma. CONCLUSION: Our case suggests a possible association between severe acute respiratory syndrome coronavirus 2 with development of RCVS, but further studies are needed to validate this observation, establish a causal relationship and define a pathophysiological mechanism. Considering tumors other than catecholamine-producing masses as a potential risk factor for developing RCVS might lead to earlier detection and treatment of any underlying malignancy in patients whom the main and sole presentation could be RCVS.

Reversible cerebral vasoconstriction syndrome (RCVS): an interesting case report.

BACKGROUND: Reversible Cerebral Vasoconstriction Syndrome is a condition of transient cerebral vascular spasms, which usually presents with recurrent thunderclap headaches and recovers within 3 months. Several probable triggers and underlying factors, such as sex hormones, vasoactive drugs, head trauma or surgery, and tumors, have been implicated. CASE PRESENTATION: In this paper, we present a 53-year-old woman with thunderclap headaches and normal lab tests who was radio-clinically diagnosed with reversible cerebral vasoconstriction syndrome and treated accordingly. Then, she experienced the recurrence of RCVS after about 2 years and headaches after 1 year in association with high blood pressure, high blood sugar, hypothyroidism, hyperlipidemia, and a urine metanephrine level of 5 times higher than the normal limit, suggesting a diagnosis of pheochromocytoma. After confirmation of the diagnosis with further imaging studies, surgical removal of the tumor resolved all the signs and symptoms. CONCLUSION: Often underdiagnosed, pheochromocytoma could be an important condition associated with RCVS. It is important for clinicians to bear this diagnosis in mind while dealing with similar cases of recurrent thunderclap headaches.

Acoustic neuroma presenting as a hypnic headache.

Hypnic headache (HH) is a rare, primary headache syndrome that invariably occurs during sleep and wakes the patient. Acoustic neuroma (AN) is a benign tumour that uncommonly presents with isolated headache. Here, we describe a patient with AN that presented with an HH-like syndrome. A 40-year-old woman presented with 4 months of generalised, throbbing, nocturnal headaches that woke her from sleep. Neurological examination was unremarkable. Retrospectively, she reported a 4-year history of mild, bilateral tinnitus. Neuroimaging demonstrated a large, left-sided AN in the cerebellopontine angle without obstructive hydrocephalus. Gamma knife radiosurgery controlled tumour growth. One year after radiosurgery, she became nocturnal headache-free. AN has not previously been described as presenting with an HH-like syndrome. There are four previous reports of an HH-like syndrome secondary to intracranial masses. In all cases, patients became headache-free following surgery. This advocates for neuroimaging to exclude structural causes.

Reversible cerebral vasoconstriction syndrome associated with hyperosmolar hyperglycaemic state: A case report and literature review.

Reversible cerebral vasoconstriction syndrome (RCVS) is an uncommon disorder characterised by thunderclap headache and self-resolving angiographic vasospasm in the presence or absence of neurological deficit. We present the first case of RCVS likely precipitated by a complex array of confounding factors including a hyperosmolar hyperglycaemic state (HHS), induction chemotherapy with cyclophosphamide, non-Hodgkin's lymphoma, pancytopenia and previous blood transfusions. However, the clinical presentation in this case of altered conscious state followed by thunderclap headache was highly suggestive of HHS being the crucial inciting factor. This report of RCVS associated with HHS lends unique insight into key underlying pathophysiological mechanisms, and warns of the need to maintain a high index of suspicion for this elusive condition given the dynamic and transient nature of its clinical and radiological features.

Pneumocephalus Associated with Inappropriate Valsalva Technique.

BACKGROUND: Pneumocephalus is intracranial air normally occurring as a consequence of trauma or cranial surgery, but rarely has been reported following the habitual use of the Valsalva maneuver. We were unable to locate any similar cases of pneumocephalus associated with high performance aircraft flight, and this case report highlights an important consequence of the inappropriate use of Valsalva on ascent to altitude.CASE REPORT: A healthy 29-yr-old F-22 instructor pilot presented with recurrent pneumocephalus following the use of the Valsalva maneuver. These episodes started after an initial use of a forceful Valsalva during an aggressive unrestricted climb to altitude. His symptoms included facial pressure and a bubbling sensation in his head followed by a severe headache for several days. After upright CT evaluation, three small sphenoid sinus defects were discovered and repaired. There was no recurrence of pneumocephalus following provocative testing after surgical repair of the sphenoid sinus defects, and the aviator returned to flying high performance aircraft.DISCUSSION: Spontaneous or Valsalva-associated pneumocephalus is an uncommon occurrence in healthy individuals. Awareness of the pathognomonic succussion splash associated with this diagnosis as well as a presentation of facial pressure and headache following flight will lead to appropriate diagnostic investigation and management. This case highlights the importance of recognizing rare diagnoses like pneumocephalus in the flying population, and future education of aerospace physiology technicians and aviators should emphasize appropriate Valsalva technique to promote flying safety and prevent similar presentations.Woodside SS, Metzler MM. Pneumocephalus associated with inappropriate Valsalva technique. Aerosp Med Hum Perform. 2019; 90(6):566-569.

RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome.

OBJECTIVE: To develop a method to distinguish reversible cerebral vasoconstriction syndrome (RCVS) from other large/medium-vessel intracranial arteriopathies. METHODS: We identified consecutive patients from our institutional databases admitted in 2013-2017 with newly diagnosed RCVS (n = 30) or non-RCVS arteriopathy (n = 80). Admission clinical and imaging features were compared. Multivariate logistic regression modeling was used to develop a discriminatory score. Score validity was tested in a separate cohort of patients with RCVS and its closest mimic, primary angiitis of the CNS (PACNS). In addition, key variables were used to develop a bedside approach to distinguish RCVS from non-RCVS arteriopathies. RESULTS: The RCVS group had significantly more women, vasoconstrictive triggers, thunderclap headaches, normal brain imaging results, and better outcomes. Beta coefficients from the multivariate regression model yielding the best c-statistic (0.989) were used to develop the RCVS2 score (range -2 to +10; recurrent/single thunderclap headache; carotid artery involvement; vasoconstrictive trigger; sex; subarachnoid hemorrhage). Score ≥5 had 99% specificity and 90% sensitivity for diagnosing RCVS, and score ≤2 had 100% specificity and 85% sensitivity for excluding RCVS. Scores 3-4 had 86% specificity and 10% sensitivity for diagnosing RCVS. The score showed similar performance to distinguish RCVS from PACNS in the validation cohort. A clinical approach based on recurrent thunderclap headaches, trigger and normal brain scans, or convexity subarachnoid hemorrhage correctly diagnosed 25 of 37 patients with RCVS2 scores 3-4 across the derivation and validation cohorts. CONCLUSION: RCVS can be accurately distinguished from other intracranial arteriopathies upon admission, using widely available clinical and imaging features. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that the RCVS2 score accurately distinguishes patients with RCVS from those with other intracranial arteriopathies.

[Reversible cerebral vasoconstriction syndrome following smoking cessation and treatment with bupropion]. / Sindrome de vasoconstriccion cerebral reversible tras abandono de habito tabaquico y tratamiento con bupropion.

TITLE: Sindrome de vasoconstriccion cerebral reversible tras abandono de habito tabaquico y tratamiento con bupropion.

Reversible cerebral vasoconstriction syndrome.

Reversible cerebral vasoconstriction syndrome (RCVS) is a relatively rare, non-progressive angiopathy frequently heralded by severe thunderclap headache. It is characterised by vasoconstriction of cerebral arteries which usually resolves within three months of onset. Transient focal neurological signs may occur, and persistent deficits associated with haemorrhagic comorbidities have been reported in a small percentage of individuals. In this paper we report the case of RH, a 36-year-old woman who presented at a university teaching hospital in Sydney with a clinical history and radiological evidence consistent with RCVS. There were no haemorrhagic events during the course of her illness, and vasoconstriction resolved within a few days, following treatment with verapamil. Neuropsychological evaluation 16 months later revealed significant deficits in autobiographical memory, verbal and non-verbal new learning and active delayed recall, cognitive flexibility, abstraction and (to a lesser extent) immediate attention span and information processing speed. RH's case was unusual because the "trigger" for RCVS (Ear, Nose and Throat surgery) has not been previously reported, and because despite there being no haemorrhagic complications during the course of RCVS and no subsequent radiological abnormalities, she had significant cognitive impairment. To date, persistent neuropsychological deficits have not been recognised as a feature of RCVS.

The Headache That Changed History: The Most Famous Thunderclap Headache.

BACKGROUND: Franklin Delano Roosevelt's fatal brain hemorrhage on April 12, 1945 provides an excellent platform for the study of the potential causes of one of the most famous headaches in history. IMPRESSION: The differential diagnosis of thunderclap headache, including hypertension and melanoma, is discussed, as are Roosevelt's health problems in the context of how serious diseases of our leaders can have a tangible effect upon historical interpretation. DISCUSSION: A large body of evidence is presented in support of a probable cover-up of Roosevelt's health problems, perpetrated by his doctors and close confidantes. The necessity of challenging conventional thinking about the health of our leaders and the fundamental importance of understanding their health issues is also emphasized.

[A Case of Juvenile Cerebral Infarction due to Reversible Cerebral Vasoconstriction Syndrome].

A 19-year-old woman had a thunderclap headache, followed by left hemiparesis and left homonymous hemianopsia. Laboratory tests showed no signs of infection and immunological test results were unremarkable. MRI revealed a cerebral infarction in the right posterior cerebral artery territory, and digital subtraction angiography(DSA)showed right posterior cerebral artery stenosis on day 2. The first follow-up DSA demonstrated an irregular, bead-like appearance on day 9, but the stenotic lesion returned to normal on day 21. Reversible cerebral vasoconstriction syndrome should be suspected in cases of rapid resolution of symptoms.

Infective Endocarditis Presenting with Intracranial Bleeding.

BACKGROUND: Infective endocarditis (IE) can be complicated by intracranial bleeding (ICB) caused by different pathologic mechanisms. The occurrence of ICB in patients with IE significantly influences therapeutic decisions and has a negative impact on outcome. CASE REPORT: We describe the clinical courses of 3 patients with aortic prosthetic valve IE presenting with ICB. Patients 1 and 2 experienced subarachnoid hemorrhage (SAH) and intracerebral hemorrhage (ICH), respectively, caused by rupture of an intracranial infectious aneurysm (IIA). Both underwent endovascular treatment of IIA with good outcome. In patient 3, ICB was the hemorrhagic conversion of an acute ischemic lesion from septic brain embolization. In the subacute phase of the disease, aortic valve replacement was performed, with excellent outcome. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: ICB is a relevant complication and sometimes the first clinical feature of IE. Imaging of brain vessels should be performed to investigate the pathologic mechanism underlying ICB. The prevalence of IIA is probably underestimated and may influence the therapeutic strategy. Cerebrovascular imaging may therefore also be considered in asymptomatic subjects with left-sided IE. Withdrawal of anticoagulant treatment and delay of cardiac surgery are recommended in all cases of IE complicated by ICB. Because of the impact of ICB on IE management and outcome, a high level of clinical suspicion and prompt recognition and treatment of this complication are necessary.

Clinical and diagnostic findings in patients with elevated cerebrospinal bilirubin.

INTRODUCTION: Cerebrospinal fluid (CSF) spectroscopy can identify subarachnoid haemorrhage (SAH) when CT is negative in patients presenting with acute severe headache. The primary objective of this study was to evaluate the clinical use and usefulness of CSF spectrophotometry. Secondary objectives were to identify other causes of elevated CSF bilirubin, to analyse headache descriptions and to compare clinical features in patients with an elevated CSF bilirubin among those with and without an intracranial vascular cause of SAH (avSAH). METHODS: Consecutive patients admitted to two hospitals in Enniskillen and Londonderry between 1 January 2004 and 30 September 2014 with CSF spectroscopy bilirubin results were identified from a clinical chemistry laboratory dataset. Patients with elevated CSF bilirubin were studied. Clinical demographics, delays to investigation and final diagnoses were recorded. Patients with avSAH were compared with patients without avSAH. RESULTS: Among 1813 patients with CSF spectrophotometry results, requests increased more than threefold during the study (p<0.001). Fifty-six patients had elevated CSF bilirubin. Ten (17.9%) had avSAH, of which 8 (14.3%) had aneurysmal SAH. Non-vascular causes of elevated CSF bilirubin included meningitis, spontaneous intracranial hypotension and carcinomatous meningitis. Headache descriptions varied. Time from headache onset to admission, CT scan and lumbar puncture did not differ significantly for patients with avSAH and non-avSAH. CSF red cell counts were higher among patients with avSAH than patients with non-avSAH (p=0.005). CONCLUSIONS: CSF bilirubin measurement has an important role in identifying avSAH in CT-negative patients presenting with a thunderclap headache. Better clinical selection of patients is required as CSF spectrophotometry, although sensitive, is not specific for SAH.

Thunderclap-like headache triggered by micturition and angina as an initial manifestation of bladder pheochromocytoma. A case report / Cefaleia trovoada-símile desencadeada pela micção e angina como manifestação inicial de feocromocitoma de bexiga. Um relato de caso

CONTEXT: Pheochromocytoma is a catecholamine-producing tumor characterized by hypertension, headache, tachycardia, excessive diaphoresis and angina. The thunderclap headache is so named because the pain strikes suddenly and severely. Although the symptoms of bladder pheochromocytoma are rather evident, the diagnosis of this rare neuroendocrine tumor can be missed. CASE REPORT: This study reports the case of a woman diagnosed with bladder pheochromocytoma who experienced thunderclap headache triggered by micturition and angina as an initial manifestation. CONCLUSION: This case study suggests that thunderclap headache and angina occurring concurrently with sudden blood pressure elevation during or immediately after micturition are important diagnostic clues for bladder pheochromocytoma. .

CONTEXTO: Feocromocitoma é um tumor produtor de catecolaminas, caracterizado por hipertensão, cefaleia, taquicardia, sudorese excessiva e angina. A dor de cabeça trovão é assim chamada porque a dor ataca de repente e intensamente. Embora os sintomas de feocromocitoma de bexiga sejam bastante evidentes, o diagnóstico deste tumor neuroendócrino raro pode ser perdido. RELATO DE CASO: Este estudo relata o caso de uma mulher diagnosticada com feocromocitoma na bexiga que sentiu a dor de cabeça trovão, desencadeada pela micção e angina como manifestação inicial. CONCLUSÃO: Este estudo de caso sugere que a dor de cabeça trovão e angina ocorrendo simultaneamente com a elevação da pressão de sangue repentina durante ou imediatamente após a micção são dicas importantes de diagnósticos de feocromocitoma na bexiga. .