Sudden Onset Of Severe Headache Research Articles

Extensive central nervous system involvement in Merkel cell carcinoma: a case report and review of the literature

IntroductionMerkel cell carcinoma is a rare malignant cutaneous neoplasm that is locally invasive and frequently metastasizes to lymph nodes, liver, lungs, bone and brain. The incidence of Merkel cell carcinoma has increased in the past three decades.Case presentationA 65-year-old Caucasian man presented with a sudden onset of severe headache and a three-month history of balance disturbance. Magnetic resonance imaging revealed a large meningeal metastasis. The radiologic workup showed retroperitoneal and inguinal lymph node metastases. Biopsy of the inguinal lymph nodes showed metastases of Merkel cell carcinoma. Biopsy from three different suspected skin lesions revealed no Merkel cell carcinoma, and the primary site of Merkel cell carcinoma remained unknown. Leptomeningeal metastases, new axillary lymph node metastases, and intraspinal (epidural and intradural) metastases were detected within six, seven and eight months, respectively, from the start of symptoms despite treating the intracranial metastasis with gamma knife and the abdominal metastases with surgical dissection and external radiotherapy. This indicates the aggressive nature of the disease.ConclusionTo the best of our knowledge, this is the first report in the literature of an intracranial meningeal metastasis of Merkel cell carcinoma treated with gamma knife and of intraspinal intradural metastases of Merkel cell carcinoma. Despite good initial response to radiotherapy, recurrence and occurrence of new metastases are common in Merkel cell carcinoma.

Use of the pipeline embolization device for the treatment of recurrent intracranial aneurysm after previous stent-assisted embolization

Aneurysmal subarachnoid haemorrhage remains an important cause of stroke mortality and morbidity. With the advance of endovascular treatment, increasingly more ruptured intracranial aneurysms are being treated with endovascular embolization. Endovascular treatment has a unique form of recurrence and complication, and the recurrence of intracranial aneurysm treated by stent-assisted embolization is a significant challenge. The pipeline embolization device (PED), a dedicated flow-modifying device, has been available in Hong Kong since September 2008. The PED (eV3, Irvine, CA, USA) is a woven wire mesh tube made of 25% platinum and 75% cobalt-nickel alloy; it is designed to provide 30–35% metal coverage of the inner surface of the target vessel with a pore size of 0.02–0.05 mm (approximately three times more than other commonly used intracranial stents). Here, we illustrate the PED treatment of a recurrent intracranial aneurysm after stent-assisted embolization. A 46-year-old man presented with the sudden onset of severe headache; computed tomography of his brain showed diffuse basal cistern subarachnoid haemorrhage. Computed tomographic angiography showed a left internal carotid artery aneurysm (5.4 mm height and 2.9 mm neck). He was treated with endovascular embolization, which was complicated with coil protrusion. Emergency stent (Neuroform3; Boston Scientific, Natick, MA, USA) deployment to avoid further coil dislodgement was undertaken and embolization continued to occlude the aneurysm. On 1-year follow-up angiography, the aneurysm had recurred with a wide neck (3.5 mm height, 3.8 mm neck). After discussing different treatment and management options, the patient decided to undergo treatment with the PED. He was given 75 mg clopidogrel and 160 mg aspirin daily for 5 days before the procedure. Under general anaesthesia and right femoral arterial puncture, a guiding catheter was inserted into the cavernous segment of his left internal carotid artery. A 3-F microcatheter was placed across the neck of the aneurysm under microguidewire guidance. The PED, mounted on a delivery wire and constrained within a sheath, was inserted into the rotating haemostatic valve and introduced into the hub of the microcatheter. Under heparinization, by pushing the delivery wire, the PED was advanced through the whole length of the microcatheter. The PED delivery wire was then held in place while the microcatheter retracted, supplemented by delivery wire rotation, to initiate deployment. Mainly through forward pressure on the delivery wire, the device was further deployed to create a tight mesh. Finally, the microcatheter was advanced to compress the proximal end of the PED, and the delivery wire was retracted. Check angiography showed aneurysm contrast stasis and layering within the dependent portion, forming an eclipse sign on the subtracted venous phase image. The patient did not experience new focal neurological deficit after the procedure. He was prescribed 75 mg clopidogrel and 160 mg aspirin daily for 3 months and 160 mg aspirin for another 3 months. On 3-month follow up angiography, the patent aneurysm sac had decreased in size. The aneurysm is expected to gradually thrombose after 6 to 12 months. In conclusion, the availability of the PED enhanced the set of tools available for the endovascular treatment of recurrent wide-necked intracranial aneurysm.

Pilocytic Astrocytoma With Histological Malignant Features Without Previous Radiation Therapy -Case Report-

Pilocytic astrocytoma sometimes transforms to a malignant type, and previous radiation therapy is considered to be a key factor. We report a case of pilocytic astrocytoma with histological malignant features without previous radiation therapy. A 21-year-old man presented a sudden onset of severe headache. Neuroimaging had detected a cystic mass in the posterior fossa at the age of one year without therapeutic intervention. On admission, computed tomography depicted a brain tumor in the posterior fossa with cystic components, intratumoral hemorrhage, and upward herniation. Urgent surgery was performed, and histological examination revealed some features of pilocytic astrocytoma but also broad necrosis, high cellularity, and MIB-1 labeling index of more than 20%. The histological diagnosis was pilocytic astrocytoma with malignant features. This tumor had continued a benign clinical course for 20 years, but had eventually transformed to a malignant type. Therefore, pilocytic astrocytoma may undergo spontaneous malignant transformation during its natural clinical course.

Intraoperative Magnetic Resonance Imaging in the Successful Surgical Treatment of an Arteriovenous Malformation-Case Report-

A 44-year-old female presented with left occipital arteriovenous malformation (AVM) manifesting as sudden onset of severe headache. Magnetic resonance (MR) imaging and conventional angiography showed the left occipital AVM with hemorrhage. Intraoperative MR imaging (iMR imaging) and intraoperative time-resolved imaging of contrast-kinetics (iTRICKS) at 1.5 T revealed complete removal of the nidus of the AVM without conventional catheter angiography. Conventional catheter angiography is commonly used in preoperative and intraoperative examination of AVMs, and for documentation of the surgical outcome, but less-invasive techniques are desirable for both preoperative screening and intraoperative examination. iMR imaging with iTRICKS is less invasive and safer than conventional angiography for both brain tumor surgery and AVM surgery.

Pituitary Apoplexy Causing Internal Carotid Artery Occlusion -Case Report-

A 50-year-old man presented with pituitary apoplexy resulting in internal carotid artery occlusion manifesting as sudden onset of severe headache, right ptosis, and left hemiparesis, associated with visual impairment. Computed tomography showed a nodular mass, located in the sellar and suprasellar regions with early signs of acute cerebral ischemia. Magnetic resonance (MR) imaging indicated that the mass compressed the bilateral cavernous sinuses, resulting in obliteration of the cavernous portion of the right internal carotid artery. Right middle cerebral artery territory infarction was also found. Conservative therapy with steroids was given in the acute stage and repeated MR imaging showed recanalization of the internal carotid artery with reduction of the tumor size. The tumor was removed through the transsphenoidal approach to obtain a definitive diagnosis in the chronic stage. The histological diagnosis was consistent with non-functioning pituitary adenoma. Eye movement of this patient showed full recovery after the operation. Pituitary apoplexy resulting in internal carotid artery occlusion is rare. Surgical decompression through the transsphenoidal approach is appropriate, but the optimal timing should consider severe disturbance of visual acuity and visual field in the acute stage.

Dissection Aneurysm of the Radiculomedullary Branch of the Artery of Adamkiewicz With Subarachnoid Hemorrhage -Case Report-

A 60-year-old female presented with sudden onset of severe headache and back pain, followed by nausea. The initial head computed tomography (CT) scan revealed posterior fossa subarachnoid hemorrhage (SAH). Spinal T(2)-weighted magnetic resonance imaging demonstrated SAH, and a homogeneous and slightly low signal intensity mass at T11. Spinal angiography in the early arterial phase revealed a small pearl and string-like aneurysm of the proximal radiculomedullary artery on the left side at the T12 level. Forty days after the onset of SAH, CT angiography demonstrated complete occlusion of the dissecting aneurysm and the preserved anterior spinal artery. The present case of ruptured dissecting aneurysm of the radiculomedullary branch of the artery of Adamkiewicz with SAH underwent subsequent spontaneous occlusion, indicating that the wait-and-see strategy may be justified and will provide adequate treatment.

Intracranial hypotension secondary to spinal arachnoid cyst rupture presenting with acute severe headache: a case report.

IntroductionHeadache is a common presenting complaint and has a wide differential diagnosis. Clinicians need to be alert to clues that may suggest an underlying secondary aetiology. We describe a novel case of headache secondary to intracranial hypotension which was precipitated by the rupture of a spinal arachnoid cyst.Case reportA 51-year-old Indian female presented with sudden onset severe headache suggestive of a subarachnoid haemorrage. Investigations including a computed tomography brain scan, cerebrospinal fluid examination and a magnetic resonance angiogram were normal. The headache persisted and magnetic resonance imaging revealed bilateral thin subdural collections, a spinal subarachnoid cyst and a right-sided pleural effusion. This was consistent with a diagnosis of headache secondary to intracranial hypotension resulting from spinal arachnoid cyst rupture.ConclusionsSpinal arachnoid cyst rupture is a rare cause of spontaneous intracranial hypotension. Spontaneous intracranial hypotension is a common yet under-diagnosed heterogeneous condition. It should feature significantly in the differential diagnosis of patients with new-onset daily persistent headache.

Dural arteriovenous fistula post sinus thrombosis in puerperium

A 28-year-old woman had a normal delivery (primipara) on January 15, 2009. On March 20, 2009, she suffered sudden onset of severe headache. Protein-S and protein-C levels were within the normal ranges. Computed tomography on admission showed no subarachnoid hemorrhage, and the patient was discharge with analgesic treatment. Four days later the patient presented again to our emergency department with increased headache, nauseas, vomiting and bruit of the head. In view of the history and clinical evolution, an urgent computerized tomography (CT) brain scan with intravenous contrast was done showing the empty delta sign; as magnetic resonance imaging (MRI) scan was not available at that time, computed tomography in venograms (Fig. 1a,b) was performed revealing occlusion of the superior sagittal sinus and the bilateral transverse sinus. The patient was referred to the Department of Neurosurgery where a cerebral angiography confirmed sinus thrombosis (ST) and revealed a dural arteriovenous fistula (DAVF) involving the right transverse sinus fed by the branches of the right middle meningeal artery, and the right occipital artery draining through transverse and sigmoid sinuses to the straight sinus (Fig. 2a,b). Transarterial embolization with N-butylcyanocrylate and detachable coils was performed, achieving occlusion of the fistula (Fig. 3a,b). Fig. 1 Computed tomography venograms showing occlusion of the superior sagittal sinus (a) (arrows) and the bilateral transverse sinues (b) (arrows) Fig. 2a, b Computed reconstruction 3D imaging showing dural arteriovenous fistula (arrows) Fig. 3 Lateral right external carotid angiogram before the embolization (a) (arrow) and after the embolization (b) showing nearly complete occlusion of the fistula (arrow) There is a clear-cut association between ST and DAF, although it may be difficult in some cases to ascertain if the thrombosis was a primary or secondary event [1]. The hypothesis proposed for the pathogenesis of DAVF is based on the physiological arteriovenous shunts between the meningeal arterial networks and the dural venous sinuses[2].

Hyperperfusion Syndrome After Neck Clipping of a Ruptured Aneurysm on a Dolichoectatic Middle Cerebral Artery

A 40-year-old female presented with sudden onset of severe headache and vomiting due to subarachnoid hemorrhage. Angiography demonstrated a saccular aneurysm on a dolichoectatic left middle cerebral artery (MCA) and delayed filling of the MCA. Magnetic resonance imaging showed a partially thrombosed giant aneurysm on the dolichoectatic MCA. An intentionally delayed operation was performed, during which the neck of the aneurysm was successfully clipped. The patient exhibited aphasia 48 hours after surgery. Single-photon emission computed tomography revealed hyperperfusion in the territory of the left MCA. The patient's blood pressure was maintained normotensively, and her symptoms gradually improved. She returned to work 1 month after surgery. The saccular aneurysm was formed on the dolichoectatic MCA, presumably due to an abnormal arterial wall and hemodynamic stress. The preoperative hypoperfusion might have been caused not only by the giant aneurysm, but also to some degree by the dolichoectatic MCA. After neck clipping, the increase in blood flow might have caused hyperperfusion.

Aneurysmatische Subarachnoidalblutung

The most characteristic symptom of an aneurysmal subarachnoid haemorrhage (aSAH) is a sudden onset of severe headache, which can be accompanied by meningism, impaired consciousness, and vegetative symptoms. A cerebral CT scan is the initial diagnostic tool of choice if aSAH is suspected. The localisation and morphology of the aneurysm is depicted in a CT-angiography and/or conventional panangiography. Until definitive exclusion of the aneurysm by either microsurgical clipping or endovascular coiling, rebleeding should be avoided implicitly. The postoperative phase, especially in patients with severe aSAH is embossed with the occurrence of disease-specific complications (cerebral vasospasm, intracranial hypertension, seizure disorder, hydrocephalus) and consecutively increased risk for secondary brain injury. Furthermore, many patients present systemic complications such as electrolyte disturbances and cardiac dysfunctions. Early identification and therapy of cerebral and systemic complications are very important in order improve functional outcome.

Rathke cleft cyst apoplexy: a newly characterized distinct clinical entity

Although most Rathke cleft cysts (RCCs) remain asymptomatic, some present with compression of surrounding structures and pituitary hormone dysfunction. A rare, but distinct presentation of the RCC includes hemorrhage into the cyst--a presentation that mimics the clinical syndrome of pituitary tumor apoplexy. The objective of this article is to present the authors' experience on the prevalence and the clinical, biochemical, pathological, and imaging characteristics of hemorrhage into an RCC and review published reports on this entity. An institutional database for patients with pituitary masses was reviewed and data on cases involving surgery for a subsequently documented RCC were identified. Patients with documented hemorrhage within the RCC were included in the analysis. Documentation included intraoperative visualization of hemorrhage by the surgeon and/or pathological confirmation of hemorrhage within the RCC. The clinical, biochemical, pathological, and imaging characteristics of these patients were extracted from the database. All published data on RCC were also reviewed and confirmed cases of hemorrhage were extracted and included in the review. A total of 11 cases with hemorrhage within an RCC were identified to have been managed at the authors' institution over a 10-year span. This figure represented 20% of all surgically treated cases of RCC from that period. Among published reports, a total of 10 confirmed cases were evaluated. The clinical presentation of patients with hemorrhage within an RCC are classical and include sudden onset of severe headache--or a sudden increase in headache severity--associated with visual disturbances, impairment in pituitary function, and occasionally alterations in mental status. Leakage of the cystic contents into the subarachnoid space can occur rarely and may lead to associated chemical meningitis. There are no known precipitating factors for this phenomenon. The clinical presentation of such patients is difficult to distinguish from that of patients with pituitary tumor apoplexy. However, patients with RCC apoplexy often present with less severe symptoms, have a lower prevalence of pituitary dysfunction, and generally have smaller mass lesions than those with pituitary tumor apoplexy. Management of patients with RCC apoplexy is similar to that of those with pituitary tumor apoplexy. Surgery can provide definitive diagnosis and relief of symptoms and often leads to recovery from hypopituitarism. All patients with this syndrome require long-term follow-up to treat any residual sequelae. This article provides the first thorough review and characterization of patients with hemorrhage within an RCC. Based on the clinical manifestations of this presentation, we recommend using the term "Rathke cleft cyst apoplexy" to describe the syndrome.

Pituitary Apoplexy Manifested as Subarachnoid Hemorrhage Accompanied by Primary Hypothyroidism

A 37-year-old woman presented with sudden onset of severe headache and vomiting. Cerebrospinal fluid examination was consistent with subarachnoid hemorrhage, but no venous sinus thrombosis or aneurysmal bleeding was found by magnetic resonance angiography or computed tomography angiography. Pituitary magnetic resonance imaging revealed a suprasellar mass with intratumoral hemorrhage. The patient's history revealed symptoms of hypothyroidism over the past 10 years. Primary hypothyroidism was confirmed by markedly decreased free T4 and T3 along with elevated thyroid-stimulating hormone levels. The patient was treated conservatively with mannitol to reduce intracerebral pressure, followed by hydrocortisone and levothyroxine. After 3 months of levothyroxine replacement, an magnetic resonance imaging revealed slight shrinkage of the pituitary gland.

Pathological crying as a manifestation of spontaneous haemorrhage in a pontine cavernous haemangioma

A 53-year-old man presented with a sudden onset severe headache associated with ataxia. His family observed labile emotions and frequent episodes of crying. Investigations revealed a pontine cavernoma with evidence of bleeding. The lesion was treated conservatively. Over approximately 6 months, the patient’s ataxia improved and he recovered from the crying episodes. To our knowledge, pathological crying has not been previously associated with brainstem cavernoma.

Pituitary apoplexy: an overview of 186 cases published during the last century

Pituitary apoplexy is a rare and life-threatening complication occurring in 0.6-10.5% of all cases of pituitary adenomas. Although the association between pituitary apoplexy and visual dysfunction has been recognized for a long time, the optimal management of this problem still remains controversial. The purpose of this overview was to present the surgical experience by analyzing the literature on the management of pituitary apoplexy for better treatment of these cases. To establish a new guideline for the surgical treatment of this entity, publications reported during the last century and databases containing medical literature were analyzed. In addition, an illustrative case with pituitary apoplexy presenting with complaints of sudden onset severe headache associated with nausea, vomiting, and a sudden loss of vision was described. In fact, the experience in our complicated patient prompted us to review the available literature on the management of pituitary apoplexy to date. Based on an overview of 186 cases of apoplectic pituitary adenoma presenting with monocular or binocular blindness, we highlight the importance of correct diagnosis and an early, but not necessarily emergency, surgery within the first week of admission to optimize visual outcome of such patients. The illustrative case further exemplifies the value of close interaction between members of the management team for optimal outcome.

Three-Dimensional Digital Subtraction Angiography for Endovascular Treatment of Direct Carotid-Cavernous Fistula -Case Report-

A 63-year-old woman with a direct carotid-cavernous fistula (CCF), manifesting as sudden onset of severe headache and pulsatile tinnitus suffered during coronary angiography, was successfully treated with transarterial embolization based on the anatomical information obtained from volume-rendered three-dimensional digital subtraction angiography (3D DSA) images. 3D DSA is useful for understanding the configuration of such fistulas accurately and devising a therapeutic strategy for the endovascular treatment of direct CCFs.

Anterior Cranial Fossa Dural Arteriovenous Fistula With Bilateral Cortical Drainers -Case Report-

A 58-year-old man presented with sudden onset of severe headache. Computed tomography demonstrated subarachnoid hemorrhage and right acute subdural hematoma. He had no neurological deficits. Cerebral angiography showed an anterior cranial fossa dural arteriovenous fistula (AVF) supplied by the bilateral ethmoidal arteries. A fistula was suggested on the right side, and the dural AVF drained into the superior sagittal sinus via the bilateral frontal cortical veins. Venous varix was observed at both drainage sites. Bifrontal craniotomy with right-side dural incision was performed and the fistula was interrupted. Postoperative angiography demonstrated a persistent fistula draining into the left cortical vein. Nineteen days later, bifrontal craniotomy with left-side dural incision was performed and the draining vein was completely coagulated with the aid of intraoperative angiography. Postoperatively, there was no detectable residual fistula. He was discharged without neurological deficits 2 weeks after surgery. The present case of anterior cranial fossa dural AVF with bilateral cortical drainers shows that drainer occlusion at two points may be needed for complete obliteration of the drainers because the fistulous connection may not be simple.

解離性動脈瘤 特発性破裂中大脳動脈遠位部解離性脳動脈瘤の１例

We present a rare case of a dissecting ruptured aneurysm of the right distal middle cerebral artery associated with subcortical hematoma and subarachnoid hemorrhage. A 57-year-old man with sudden onset of severe headache and left hemiplegia was referred to our hospital. A CT scan showed a subarachnoid hemorrhage in the right sylvian fissure associated with subcortical hematoma in the right temporo-parietal lobe. The right carotid angiogram revealed a fusiform aneurysm in the distal middle cerebral artery. The aneurysm was successfully excised. The postoperative course was uneventful, and his left hemiparesis recovered well. The distal middle cerebral dissecting aneurysm is extremely rare. We present this case with a review of the literature.

Dilated Outer Diameter of the Dissected Artery: Acute Bilateral Anterior Cerebral Artery Dissection Evaluated by Repeat Magnetic Resonance Cisternography -Case Report-

A 42-year-old male patient presented with an anterior cerebral artery (ACA) dissection manifesting as sudden onset of severe headache. Initial computed tomography revealed faint subarachnoid hemorrhage in the frontal region. Initial angiography showed tapering stenosis at the A(2) segment of right ACA. The patient was admitted to our hospital and treated conservatively. Magnetic resonance (MR) imaging and angiography did not detect intramural hematoma, intimal flap, or double lumen at the stenotic right A(2) segment. The ACA dissection was difficult to confirm based on the findings on day 0. ACA dissection was confirmed by improvement of the right ACA stenosis on follow-up angiography on day 14. On the other hand, MR cisternography revealed a fusiform dilatation of the vascular outer contour at the right A(2) on day 0, which had resolved on day 14. Cerebral angiography and MR cisternography similarly suggested asymptomatic contralateral (left) A(2) dissection on day 14. Fusiform dilatation of the vascular outer contour at the affected segment on MR cisternography may be indicative of arterial dissection in the acute phase.

Carotid agenesis with intercavernous anastomosis.

Agenesis of internal carotid artery (ICA) is rare. Association of agenesis with abnormal arterial communication between the cavernous segment of the carotid arteries is extremely rare. We present a case of a middle-aged woman who presented with sudden onset severe holocranial headache. She was investigated and diagnosed to have sub-arachnoid hemorrhage on CT scan. Digital Subtraction Angiogram revealed absent right internal carotid artery. The right middle cerebral artery received blood supply from an incidentally detected intercavernous anastomosis from the left internal carotid artery. No aneurysm, arteriovenous malformation or any other vascular abnormality was detected. The patient was managed conservatively. She has been asymptomatic for five years on follow up.

Intracranial plasmacytoma with apoplectic presentation and spontaneous intracerebral hemorrhage: Case report and review of the literature

Involvement of the nervous system is not uncommon in patients with multiple myeloma, with polyneuropathy and myelopathy predominating. Intracranial involvement producing neurological symptoms, however, is distinctly uncommon. Massive intraparenchymal hemorrhage from a previously unrecognized intracranial plasmacytoma is exceedingly rare. The authors report the case of a 57-year-old male who presented with sudden onset of severe headache, rapid onset of right-sided weakness and deterioration in level of consciousness while at work. Two years earlier the patient had completed treatment for multiple myeloma and was considered to be in remission, with a recent bone marrow biopsy that was negative, and complete normalization of serum protein electrophoresis. Imaging studies revealed a massive intracerebral hemorrhage with the possibility of an underlying lesion, and the patient was taken for emergent hematoma evacuation and tumor resection. The patient made an excellent recovery and was treated with intracranial radiation. Even in patients with multiple myeloma without evidence of systemic disease following successful treatment, the possibility of unrecognized lesions lingers. The onset of new symptoms referable to potential intracranial pathology in this setting should prompt consideration of intracranial plasmacytoma in the differential diagnosis.