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Changing pattern of headache pointing to cerebral venous thrombosis after lumbar puncture and intravenous high-dose corticosteroids.

OBJECTIVE: To emphasize the diagnostic importance of change in the headache pattern which pointed to cerebral venous thrombosis in two patients after lumbar puncture and high-dose intravenous methylprednisolone for suspected multiple sclerosis. RESULTS: Both patients had a diagnostic lumbar puncture for suspected multiple sclerosis and were treated with high-dose intravenous methylprednisolone. Both developed a postlumbar puncture headache that was initially postural, typical of low cerebrospinal fluid pressure. Three days later, the headache became constant, lost its postural component, and was associated with bilateral papilledema. Magnetic resonance imaging of the brain disclosed superior sagittal and lateral sinuses thrombosis. The diagnostic difficulties of such cases and the potential role of lumbar puncture and corticosteroids as risk factors for cerebral venous thrombosis are discussed. CONCLUSIONS: When a typical postdural puncture headache loses its postural component, investigations should be performed to rule out cerebral venous thrombosis, particularly in the presence of other risk factors.

Adult↗

Traumatic carotid-cavernous sinus fistula.

Traumatic carotid-cavernous sinus fistula is an uncommon situation resulting from severe trauma, with less than 40 cases having been reported. It is usually associated with a skull base, frontal or mid-facial fracture, but it may also be a spontaneous phenomenon of congenital, infective or degenerative origin. The blood shunts from the internal carotid to the cavernous sinus resulting in pulsating exophthalmos, orbital headache, pain, orbital or frontal bruit, loss of visual acuity, diplopia, ophthalmoplegia and the differential diagnosis should include superior orbital fissure syndrome, orbital apex syndrome and cavernous sinus thrombosis. Several modes of treatment have been proposed. We were recently faced with such a case, who had sustained fractures of the facial skeleton and developed the fistula despite an initial non-contributory angiogram. The patient remained in a permanent coma.

Accidents, Traffic↗

DDD pacemaker implantation in a patient with persistent left superior vena cava and absent right superior vena cava: a four-year follow-up report.

Persistent left superior vena cava coexisting with the absence of right superior vena cava is an uncommon anomaly, and sometimes requires permanent pacemaker implantation due to the relatively high incidence of conduction disturbances and arrhythmias. This anomaly makes the implantation of pacemaker leads more difficult; therefore, accurate preintervention diagnosis of this anomaly is valuable for the interventionalist. A patient in which the diagnosis of persistent left superior vena cava coexisting with absent right superior vena cava was made echocardiographically before permanent pacemaker implantation is presented. Acetylsalicylic acid was prescribed after pacemaker implantation to prevent a potentially fatal complication of coronary sinus thrombosis, and no complication occurred during the four-year follow-up. Some clues for the noninvasive diagnosis of this anomaly and techniques of pacemaker implantation are also described briefly.

Aspirin↗

Concomitant heterozygous factor V Leiden mutation and homozygous prothrombin gene variant (G20210A) in patient with cerebral venous thrombosis.

BACKGROUND: Factor V Leiden mutation represents the most common genetic risk factor of venous thrombosis in Caucasian population. A common mutation in prothrombin gene, which is due to G-->A transition at position 20210, is also associated with elevated prothrombin concentration and thrombosis. Both this mutations may constitute concomitant risk factors for deep venous thrombosis. CASE REPORT: A 29-years old woman was admitted in the Emergency Department because of severe headache with vomiting that she suffered for a few days without any neurological deficits. In the Emergency she presented first in her life tonic-clonic seizures followed by right hemiparesis and aphasia and than was admitted to hospital. CT and MR scan showed a large lesion in the left fronto-parietal region with extent edema, which was first diagnosed as tumor. Following MR showed more lesions and typical signs of sinus thrombosis. She improved quickly after stroke without any anticoagulant treatment. Genetic study revealed factor V Leiden mutation and homozygous mutation G20210A in prothrombin gene. CONCLUSIONS: Both mutations found in this case, alone, are not a high risk factors for venous thrombosis but together may increase 5-10 fold risk of venous thrombosis. Venous stroke must be considered always in acute neurological events with organic brain lesions, especially in young

Adult↗

Lemierre's syndrome: two cases of postanginal sepsis.

Lemierre's disease consists of suppurative thrombophlebitis of the IJV in the presence of oropharyngeal infection and can be complicated by septic pulmonary emboli. If a patient has an oropharyngeal or deep neck infection and neck pain suspicious for IJV thrombosis, a CT or MRI is warranted to establish the diagnosis. Blood cultures should be obtained to establish the responsible organism. In most cases F. necrophorum, an anaerobic bacterium, is responsible for the sepsis. Once the diagnosis of Lemierre's disease is made, long-term, high-dose intravenous antibiotics with beta-lactamase anaerobic activity should be initiated. In cases with persistent sepsis and emboli despite appropriate medical management, ligation or excision of the IJV should be performed. Finally, if there is clinical or radiologic evidence of retrograde cavernous sinus thrombosis, the use of anticoagulants should be considered.

Adult↗

The role of C.T. scans in managing the orbital complications of ethmoiditis.

Thirty-three cases of ethmoidities with orbital complications were reviewed to determine the accuracy of clinical diagnosis and the benefit of CT scans in planning treatment. Patients were classified according to the Schramm et al. 1982 criteria of orbital involvement with ethmoiditis: periorbital cellulitis with chemosis (PCC)-9, and orbital cellulitis (OC)-11, subperiosteal abscess (SPA)-9, and orbital abscess (OA)-4. These patients received CT scans acutely for diagnostic purposes and demonstrated an 84% accuracy with the final clinical groupings. Of 33 patients, 9 had a shift in clinical classification based on the CT scan result interpreted by the Radiology Department or surgical findings. There were no false positives in the periorbital or orbital cellulitis patients, and no false negatives in the subperiosteal and orbital abscess patients. Thus classification changes caused no change from medical to surgical treatment in any of the cases. The conclusion is that a knowledgeable clinical exam established the correct grouping in 70% of the patients versus 82% with the CT scan. An urgent CT scan is advised for patients in clinical groups SPA, OA, or cavernous sinus thrombosis (CST) to determine the imminent need for surgery, as the cases may be underestimated. Patients with PC or PCC can be managed medically with elective CT scans not routinely indicated.

Abscess↗

Parasellar syndromes.

The parasellar compartments are located lateral to and on either side of the sella turcica. The cavernous sinuses are the most prominent anatomic feature of the parasella. Each sinus consists of a plexus of veins through which runs the intracavernous portion of the internal carotid artery. Ocular motor nerves three and four travel within the dural covering of the cavernous sinus to the superior orbital fissure, and cranial nerve six travels through the carotid sinus itself, giving rise to parasellar syndromes, which have distinctive clinical features. Ophthalmoplegia occurs as a result of damage to these ocular motor nerves and variable involvement of oculosympathetic nerves. Facial pain, dysesthesia, and paraesthesia are caused by damage to one or more of the divisions of the fifth cranial nerve, travelling in the dural wall of the cavernous sinus. Tumors, such as meningiomas, frequently cause parasellar syndromes, as do aneurysms of the intracavernous portion of the internal carotid artery, carotid-cavernous fistulas, and cavernous sinus thrombosis. Inflammatory conditions such as Tolosa-Hunt syndrome, ischemia to small vessels supplying the cavernous portion of the cranial nerves, and infections can cause this syndrome. Magnetic resonance imaging is the investigation of choice and therapy is specific to the cause of the parasellar syndrome, but now includes more aggressive endoscopic and microsurgical intervention, and radiosurgery.

Adult↗

Predictive factors of bleeding and thrombosis during induction therapy in acute promyelocytic leukemia-a single center experience in 34 patients.

In this retrospective study, the hemorrhagic and thrombotic events are reported at presentation and during induction in 34 consecutive acute promyelocytic leukemia (APL) patients treated in a single referral center. The most consistent hemostatic abnormality was decreased fibrinogen level (<150 mg/dL) found in 21 patients (61%), partial thromboplastin time (PTT) was normal almost in all patients. A mildly prolonged prothrombin time (PT) was observed in 14 patients (44%). Median platelet count was 30.10(9)/L (range 3-191.10(9)/L). Life-threatening bleeding manifestations occurred in 10 patients (29%). By multivariate analysis, severe bleeding complications did not correlate with hemostatic parameters but did correlate with white cell count at presentation. Four patients (12%) had severe thrombotic events, two cerebral sagital sinus thrombosis, one pulmonary embolism, and one subclavian vein thrombosis. Two other patients had pseudotumor cerebri. Three out of six patients with thrombotic events were found to have thrombophilia. These results may suggest an association between thrombophilia and thrombosis in APL patients. Two patients suffered from combined severe bleeding and thrombosis. Hemostatic parameters are not helpful in predicting neither hemorrhage nor thrombosis.

Adolescent↗

Bilateral superior ophthalmic vein enlargement associated with diffuse cerebral swelling. Report of 11 cases.

Bilateral superior ophthalmic vein (SOV) enlargement has rarely been shown to occur in patients with septic and aseptic cavernous sinus thrombosis, Graves' disease due to obstruction of the SOV by enlarged extraocular muscles, or carotid-cavernous fistulas caused by retrograde flow. The authors describe 11 patients with bilateral SOV enlargement associated with cerebral swelling as detected by computerized tomography scanning. The bilaterally enlarged SOVs returned to a normal size following resolution of cerebral swelling and elevated intracranial pressure. To the authors' knowledge, this is the first report of bilateral SOV enlargement associated with diffuse cerebral swelling that subsequently resolved after treatment of the cerebral edema. The authors believe that the bilateral SOV enlargement was caused by mechanical cavernous sinus venous stagnation due to cerebral swelling, a syndrome that occurs more commonly than currently appreciated.

Adolescent↗

Sinusitis and ischemic stroke.

Acute sinusitis is a prevalent and generally uncomplicated infection that is normally resolved by medical therapy. However, severe neurological complications are known, and comprise of cerebral abscess, cavernous sinus thrombosis, meningitis, and epidural or subdural empyema. We report a case about a 10-year-old girl with a severe acute pansinusitis and ischemic stroke in the right lentiform nucleus and the anterior part of the right internal capsule. Possible explanations for this rare combination are discussed.

Acute Disease↗

[Thrombophlebitis of the lateral sinus. A more frequent complication than expected].

At present, the frequency of intracranial complications of middle ear infections has decreased sharply as a result of the use of more effective antibiotics. We report two cases of lateral sinus thrombosis, in a 2-year-old male and a 14-year-old male respectively, after middle ear infection that progressed to mastoiditis with osteitis. The severity of these complications underlines the importance of clinical awareness and early diagnosis by angiographic magnetic resonance imaging so that suitable treatment can be begun.

Adolescent↗

Indirect carotid cavernous fistula appeared after balloon embolization of direct CCF.

The authors describe a case of indirect carotid cavernous fistula (CCF) appearing five months after embolization for traumatic direct CCF, which was treated six months after the trauma. Long-term (six months) venous hypertension to the affected cavernous sinus due to direct CCF and cavernous sinus thrombosis following a balloon embolization were considered as an etiology of the de novo dural arteriovenous fistula. The recurrent symptoms of CCF are usually related to detached balloon disorder, but delayed recurrence may be caused by the de novo dural AVF, if the direct CCF was treated in the chronic state.

Arteriovenous Fistula↗

[Thrombosis of intracraneal sinuses in homocystinuria (author's transl)].

The case of a child affected with piridoxinresistant homocystinuria who developed early on the disease an aseptic thrombosis of intracraneal sinuses, of chronic evolution is presented. Pathogenesis and therapy of the thrombosis are discussed emphasizing the value of measures directed to avoid the aggregation of platelets.

Aspirin↗

MR findings in rhinocerebral mucormycosis.

A case of rhinocerebral mucormycosis evaluated by magnetic resonance (MR) imaging is presented, with inflammatory changes in the paranasal sinuses and orbit. Extension of the infection into the cavernous sinus, with cavernous sinus thrombosis and internal carotid artery narrowing, was well demonstrated on pre- and postgadolinium MR images. Infarctions in the anterior choroidal artery distribution suggested intracranial invasion of basal arteries, and watershed distribution infarctions attested to the tenuousness of flow through a narrowed cavernous carotid artery. Magnetic resonance effectively demonstrated the wide array of findings possible in rhinoorbitocerebral mucormycosis.

Adult↗

Spontaneous cardiomyopathy and exophthalmos in cotton rats (Sigmodon hispidus).

Exophthalmos and clinical signs of heart failure occurred sporadically in 3- to 12-month-old cotton rats (Sigmodon hispidus) in a colony originally derived from three male and four female littermates. Macroscopic lesions in severely affected animals included subcutaneous edema, hydrothorax, right ventricular dilatation, unilateral or bilateral atrial thrombosis, and exophthalmos. Hearts from 17 cotton rats that were found dead or were euthanatized because of exophthalmos or dyspnea and 33 control cotton rats were examined microscopically. Myocardial lesions were present in 46 of 46 cotton rats > or = 1 month of age and consisted of multifocal cardiac myocyte necrosis, mineralization, and mononuclear inflammatory cell infiltration. Cotton rats > 5 months of age also had foci of interstitial fibrosis and myocyte atrophy. Twelve of 24 (50%) necropsied cotton rats had chronic pulmonary congestion, and livers from eight of 24 (33%) had chronic periacinar congestion and atrophy. Thrombi were present in one or both cardiac atria in nine of 50 (18%) hearts, and in at least one orbital venous sinus in 14 of 24 (58%) necropsied cotton rats and in 12 of 14 (86%) with exophthalmos. Exophthalmos in this colony of cotton rats appears to have resulted predominantly from orbital venous sinus thrombosis caused by stasis of venous blood secondary to right heart failure associated with a heritable cardiomyopathy.

Animals↗

Redefined role of angiogenesis in the pathogenesis of dural arteriovenous malformations.

To investigate the role of angiogenesis in the pathogenesis of dural arteriovenous malformations (AVMs), 40 rats underwent common carotid artery-external jugular vein (CCA-EJV) anastomosis, bipolar coagulation of the vein draining the transverse sinus, and sagittal sinus thrombosis to induce venous hypertension. Fifteen rats underwent a similar surgical procedure, but venous hypertension was not induced. The 55 rats were divided into seven groups. Four groups, each containing 10 rats, underwent induced venous hypertension. The other three groups, each containing five rats, did not undergo induced venous hypertension. After 1, 2, or 3 weeks, dura mater was obtained from one group of hypertensive rats and from one group of nonhypertensive rats and was assayed for angiogenic activity (rabbit cornea bioassay). The remaining group of 10 hypertensive rats was not assayed to determine if sampling affected dural AVM formation. Unlike rats without CCA-EJV anastomosis, rats with CCA-EJV anastomosis had significantly increased postoperative sagittal sinus pressures (p < 0.0001). Mean angiogenesis indices were significantly greater in rats with venous hypertension than in rats without venous hypertension (p = 0.004). Dural AVMs formed in 42% of the 55 rats and facial AVMs formed in 51%. Angiogenic activity correlated positively with venous hypertension (p = 0.74). Development of dural AVMs correlated positively with both venous hypertension (p = 0.0009) and angiogenic activity (p = 0.04). These data indicate that venous hypertension may induce angiogenic activity either directly or indirectly by decreasing cerebral perfusion and increasing ischemia, and that dural AVM formation may be the result of aberrant angiogenesis.

Animals↗

Giant infectious intracavernous carotid artery aneurysm presenting as intractable epistaxis.

Infectious intracavernous carotid artery aneurysms usually present with ophthalmoplegia and/or signs of cavernous sinus thrombosis. We report an unusual case in which a patient with AIDS presented with intractable epistaxis secondary to rupture of a giant infectious intracavernous carotid artery aneurysm. Culture of the aneurysm grew mycobacterium avium intracellulare (MAI). The patient was treated successfully by excision of the aneurysm and reconstruction of the internal carotid artery with a saphenous vein interposition graft.

AIDS-Related Opportunistic Infections↗

Clinical features of late-onset pseudotumor cerebri fulfilling the modified dandy criteria.

OBJECTIVES: To characterize the clinical features of patients with pseudotumor cerebri (PTC) fulfilling the Modified Dandy Criteria who were diagnosed at or after the age of 44 years. METHODS: We reviewed the medical records between 1987 and 1999 of 14 patients at a single institution who were diagnosed as having PTC at 44 years of age or older according to the Modified Dandy Criteria: neurologic manifestations attributable to generalized increased intracranial pressure, elevated cerebrospinal fluid pressure with normal cerebrospinal fluid composition demonstrated by lumbar puncture, and normal or small ventricles demonstrated by neuroimaging. We documented presenting symptoms and signs, significant medical conditions, and visual field follow-up. RESULTS: There were nine women and five men. Nine patients (64%) were obese. Five patients (36%) were asymptomatic. None presented with headache alone. Four patients (29%) had an identifiable cause of intracranial hypertension, including two with transverse sinus thrombosis, one with severe chronic obstructive pulmonary disease and cor pulmonale, and one with corticosteroid withdrawal after prolonged administration. During a median follow-up of 2 years of 12 patients, visual fields remained stable in 8, improved in 3, and worsened in 1. CONCLUSIONS: In comparison with patients who have idiopathic intracranial hypertension, our small series of 14 patients diagnosed after the age of 44 years were more often men, were less often obese, were less symptomatic, and had identifiable causes of intracranial hypertension in a substantial minority (29%). The visual prognosis in this age group is generally good. Because the nonidiopathic causes of PTC would be overlooked by adhering to the Modified Dandy Criteria, we propose a modification that excludes patients who have dural venous sinus disease demonstrated on magnetic resonance imaging and those who may be exposed to medications or toxins or have systemic disorders that are known to increase intracranial pressure.

Acetazolamide↗