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[Neuropsychiatric disorders induced by MDMA ("Ecstasy")].

If neurotoxicity of MDMA (ecstasy) is now well documented in animals, it is not the same in humans. MDMA intoxication puts the problem of its possible link with the serotonin syndrome and the neuroleptic malignant syndrome. Neuropathological consequences following MDMA intake have been reported, including hemorrhaging and cerebral infarction, cerebral venous sinus thrombosis, and acute inflammatory CNS disease. However, the physiopathology of these complications remains unclear. In the same way, there have been various reports that have attributed MDMA to precipitating the onset of a wide range of psychiatric disorders including sleep disorders, cognitive disorders, panic attacks, depression, flashbacks, psychosis and severe paranoia. Findings suggest that these psychiatric manifestations might be consequences of MDMA induced brain serotonin neurotoxic lesions. All these data are examined from a critical review of the literature.

Cerebral Hemorrhage↗

[Investigation of fat in the dural sinus].

Detection of fat in the cranium usually indicates the presence of a fat-containing tumor such as lipoma, dermoid cyst or teratoma. However, since 1982, Hasso et al demonstrated with CT the presence of normal adipose tissue in the cavernous sinus, the mere existence of fat in the cranium does not necessarily mean the presence of a fatty tumor. The author first described fat deposition in the superior sagittal sinus and torcular Herophili following a CT study performed in 1986. The purpose of this study was to investigate the distribution, frequency, and anatomical correlations of fat in the dural sinus as demonstrated on CT. Fat was detected in the cavernous sinus in 20% of all cases (492/2408), and occurred more frequently (25%) in those older than 50 years. Fat was less frequent in the other dural sinuses (3%; 75/2296). The most common location was the torcular Herophili, followed in decreasing order of frequency by the straight sinus, inferior sagittal sinus, superior sagittal sinus and transverse sinus. Pathological examination was performed in three cases. Fat deposition was composed of normal adipose tissue and was devoid of fibrous encapsulation or infiltration. In one case, the fat seemed to be partly exposed to the subarachnoid space on CT, whereas on autopsy, thin dura mater covering the fat nodule was confirmed. Fat in the dural sinus must be differentiated from cavernous nodule or sinus thrombosis. The Hounsfield unit may be helpful in making a definitive diagnosis.

Adipose Tissue↗

A childhood cavernous conundrum.

A 9-year-old boy presented with a left-sided "tooth ache." Subsequently, he developed new onset vertical diplopia. He was found to have a left cranial nerve three palsy. Cranial magnetic resonance imaging showed bilateral cavernous sinus lesions that were initially interpreted as possible "cavernous sinus thrombosis." Subsequent blood smear and bone marrow biopsy disclosed Burkitt lymphoma. He was treated with chemotherapy and made a dramatic recovery. Burkitt lymphoma rarely presents with a third nerve palsy but should be considered in the differential diagnosis of childhood cavernous sinus syndrome.

Antineoplastic Combined Chemotherapy Protocols↗

[Venous embolization of dural arterio-venous fistulas. Transcranial approach under neuronavigation].

OBJECT: Transvenous embolization is an effective treatment for dural arterio-venous fistulas. In the event of dural sinus thrombosis, embolization can be performed by direct puncture via craniectomy. We report our experience with transcranial venous embolization in two patients. METHODS: Two patients underwent transcranial venous embolization of a type-III dural arterio-venous fistula of the transverse sinus. The craniectomy was performed with neuronavigation assistance. RESULTS: The surgical approach consisted in a minimal exposure of the sinus with a drill. Neuronavigation allowed accurate placement of the craniectomy. The endovascular treatment was performed in a second stage, enabling complete and stable occlusion of the fistula in both patients. CONCLUSION: This combined treatment of dural AVFs associating craniectomy and transcranial puncture of the sinus is simple and efficient when retrograde navigation through the internal jugular vein cannot be achieved. Neuronavigation is helpful in accurately positioning the craniectomy.

Aged↗

Treatable intracranial hypertension in patients with lupus nephritis.

Idiopathic intracranial hypertension is a disorder of intracerebral pressure regulation and patients run the risk of permanent visual loss. Intracranial hypertension (IH) has been reported rarely in systemic lupus erythematosus (SLE). We reviewed the medical records of 127 patients with lupus nephritis (LN) who were followed up from 1987 to 1996 in our unit. There were six patients with IH which gave a disease prevalence of 4.7% in those with LN. All were females giving a disease prevalence of 5.2% for that sex, a high rate of occurrence of IH in patients with LN. Their age ranged from 22 to 34 y (27.8 +/- 3.6 y). Headache, vomiting and diplopia were the common presenting symptoms and had started 7.3 +/- 4.4 weeks prior to the diagnosis of IH. The cerebrospinal (CSF) opening pressure (413.3 +/- 77.0 mmH2O) was raised in all cases. Biochemical and cytological analyses of CSF were normal. The only abnormal radiological finding was partially empty sella in one patient on magnetic resonance imaging (MRI) (performed in three patients) or computed tomography (CT) (performed in all patients). All patients had serological evidences of active lupus disease at the time of diagnosis of IH. The renal histology was WHO type IV in four cases and III and V in one each indicating severe renal involvement. Laboratory evidences of procoagulant activity were found in the form of positive anticardiolipin antibody (aCL) in two patients, lupus anticoagulant (LA) in two and an otherwise unexplained isolated prolongation of activated partial thromboplastin time (APTT) in the other two. Clinically, one or more episodes of symptomatic venous or arterial thrombosis had occurred in all subjects. In addition to symptomatic measures, all subjects were treated with prednisolone, azathioprine, cyclophosphamide and plasmapheresis according to the protocol of our unit. One patient who did not receive plasmapheresis and cyclophosphamide had a relapse while all others recovered completely. None received anticoagulant therapy. Young females with serologically active lupus, severe forms of renal lesions, past history of venous or arterial thrombosis and laboratory evidences of procoagulant activity, appear to be at increased risk of IH. Thrombotic occlusion of the cerebral arteriolar or venous vascular bed eventually affecting the arachnoid villi and impeding CSF absorption is favoured compared to cerebral venous or sinus thrombosis as the pathogenic mechanism. Combined immunosuppression and plasmapheresis appeared to be beneficial in short and long term follow-up. We propose that patients with SLE and IH have definable risk and pathogenetic factors and are no more to be considered 'idiopathic'. The conditions calls for aggressive intervention which leads to an excellent outcome.

Acetazolamide↗

Subarachnoid hemorrhage: a rare presentation of cerebral venous thrombosis.

Because of its large spectrum of clinical manifestations, diagnosis of cerebral venous thrombosis may be very difficult. Since appropriate treatment influences prognosis, early recognition of this condition is extremely important. We report a subarachnoid hemorrhage as a rare initial manifestation of cerebral venous thrombosis. A 58-year-old woman was admitted with severe headache of sudden onset, neck stiffness, dysarthria, and ataxia. Computed tomography scan showed a subarachnoid hemorrhage in the right posterior fossa. Magnetic resonance imaging coupled with magnetic resonance angiography revealed right transverse/sigmoid sinus thrombosis with hemorrhagic infarction of the right cerebellar hemisphere leading to a pseudotumoral appearance and displacing the fourth ventricle. Anticoagulant treatment resulted in rapid clinical recovery and in resolution of the radiological signs of infarction and of the subarachnoid and subdural hemorrhages.

Cerebral Veins↗

Deep cerebral venous thrombosis in adults.

OBJECTIVE: To investigate the pathogenesis, clinical features, radiographic findings and therapeutic outcomes of non-acute intracranial deep venous thrombosis in adults. METHODS: Five patients who presented with increased intracranial pressure were examined with computed tomography, magnetic resonance and angiography, diagnosed as having non-acute intracranial deep venous thrombosis, and treated with thrombolytic therapy. They were reviewed retrospectively. RESULTS: There were 3 men and 2 women, aged from 22 to 49 years. Symptom duration ranged from 1 month to 7 months, and 4 of the 5 patients were associated with venous sinus thrombosis. Two patients developed cold and fever before the onset of disease, and 3 patients had no evident predisposing factors. After the infusion of thrombolytic and systemic anti-coagulant therapy, the neurological symptoms and signs of the patients were alleviated. CONCLUSIONS: Digital subtraction angiography (DSA) is more sensitive and accurate than MRI on diagnosing intracranial deep venous thrombosis. It may play an important role in the assessment of the treatment of intracranial deep venous thrombosis. Thrombolysis and anticoagulation of intracranial deep venous thrombosis appears to be a safe and efficacious treatment not only in the acute stage but also in the non-acute stage.

Adult↗

[Imaging methods in diagnosis of cerebrovascular complications with L-asparaginase therapy].

During or immediately following L-asparaginase (L-asp) therapy especially intracranial thromboembolic or hemorrhagic complications due to hemostatic imbalance have been observed. We report about 6 children who had intracranial thrombosis or hemorrhage after 3 to 8 doses of L-asp. Initial symptoms of the cerebral events were similar--seizure, coma, hemiparesis and disorientation. All patients were examined by cerebral computerized tomography (CT) or/and magnetic resonance imaging (MRI). Three patients had a dural sinus thrombosis, one had an intracranial hemorrhage and two a hemorrhagic infarction with typical findings in the cerebral CT or MRI. Two other patients were interpreted as having peripheral thrombosis. They showed nontypical hypodense cortical and subcortical areas without any contrast medium enhancement in CT and hyperintense areas in T2-weighted MRI scan. All patients recovered from neurological symptoms, and showed obvious regression of CT and MRI findings which correlate with the good prognosis of these complications. Both CT and MRI are useful in diagnosis and follow-up of cerebrovascular complications of L-asp therapy. The CT and MRI findings of the reported cases seem to reflect different appearances of the same entity, i.e. thrombosis of venous vessels of different size with or without congestive edema and hemorrhagic complication.

Adolescent↗

Transcranial approach for venous embolization of dural arteriovenous fistulas.

OBJECT: Transvenous embolization is effective in the treatment of an intracranial dural arteriovenous fistula (DAVF). Access to the fistula via the internal jugular vein (IJV) may be limited by associated dural sinus thrombosis; a transcranial approach has been developed for venous embolization in such a situation. The authors report their experiences with the use of a transcranial approach for venous embolization of DAVFs. METHODS: Ten patients with DAVFs underwent craniectomy and embolization procedures in which direct sinus puncture was performed. The DAVFs were located inside the dura mater that constituted the walls of the transverse sinus in five cases, the superior sagittal sinus in four cases, and the superior petrosal sinus in one case. All DAVFs drained directly into a sinus with secondary reflux into leptomeningeal veins. In all cases, the fistula could not be accessed from the IJVs. Craniectomy was performed in an operating room and, in seven cases, subsequent enlargement of the craniectomy was required. Sinus catheterization was performed after the patient had been transferred to the angiography room. The DAVFs were embolized using coils only in five patients, glue only in two patients, and both coils and glue in three patients. Angiographic confirmation that embolization of the fistula was successful was obtained in all cases. A transient complication occurred during the first case after sinus catheterization was attempted in the operating room. CONCLUSIONS: The transcranial approach allows straightforward access to DAVFs located on superficial dural sinuses that are inaccessible from the IJVs. The effectiveness of this approach is similar to that of the standard retrograde venous approach. The correct location and adequate extent of the craniectomy are essential for success to be achieved using this technique.

Adult↗

Classification of spontaneous dural arteriovenous fistulas with regard to their pathogenesis.

PURPOSE: A classification of dural arteriovenous fistulas (DAVFs) according to their pathogenesis has not been established. The purpose of this study was to examine the hypothesis that the different angiomorphologies of DAVFs depend on the location of the venous recipient, and that a territorial classification of spontaneous DAVFs can be created based on their acquired development. MATERIAL AND METHODS: The clinical and radiographic findings of 96 patients with DAVFs were reviewed, especially with regard to their venous characteristics. RESULTS: DAVFs were subdivided into 5 groups: Type 1--DAVFs of the dural sinuses (n = 39); Type 2--DAVFs of the cavernous sinus (n = 29); Type 3--DAVFs of Galen's system (n = 10); Type 4--DAVFs of the venous plexus at the base of the skull (n = 9); Type 5--DAVFs of the parasinusal cortical veins (n = 9). The documentation of a causal sinus thrombosis depends on the location of the DAVF: in 72% of the cases with type 1 DAVFs there was a thrombosis at the time of the investigation, but no thrombosis could be proved in cases with type 4 DAVFs. CONCLUSION: The morphological development of DAVFs seems to depend on the flow volume of the venous recipient. A pronounced development of pathological AV shunts takes place at the level of the large basal dural sinuses. A delayed development of pathological AV shunts with a low shunt volume occurs in a venous recipient with a low AV pressure gradient.

Adult↗

[Color-coded Doppler sonography of orbital blood vessels with special reference to the central retinal artery and vein].

When certain conditions are met, parameters correlating with orbital and retinal perfusion can be investigated non-invasively with the use of Doppler ultrasonography. Color coding allows more rapid vessel localization and more precise definition of sample volume localization. Flow direction and blood velocity in the central retinal artery and vein, as well as in the ophthalmic artery, were evaluated under normal and pathological conditions using current B-scan Doppler instruments with color coding. Fourteen patients with central artery occlusion, 9 with central vein occlusion, and 9 with orbital arteriovenous fistulas were compared with a control group. In the patients with retinal venous prethrombosis and thrombosis, the blood velocities were not significantly decreased. Patients with retinal arterial occlusion had contradictory findings, presumably since recanalization had already taken place. Echographic flow imaging might be most useful when, particularly after treatment, flow velocity patterns in the orbital vessels are evaluated, and when angiographic procedures can be avoided. Illustrative cases such as spontaneous dura-cavernous sinus fistulas versus cavernous sinus thrombosis show that difficult diagnostic decisions can be made easier.

Adult↗

[The neuroleptic malignant syndrome].

HISTORY AND CLINICAL FINDINGS: A 22-year-old oligophrenic patient had on several occasions over several months been given various neuroleptics (haloperidol, benperidol, levomepromazine) for exacerbations of paranoid schizophrenia. For a few days before hospitalization he had become quiet and withdrawn, but on the day of admission 100 mg chlorprothixene was administered intramuscularly when he had become agitated. At admission he was somnolent, his general condition was disturbed. He had hyperhidrosis and hypersalivation, as well as tachycardia (112/min) with a normal body temperature of 37.8 degrees C. He also exhibited the cogwheel phenomenon of the limbs and neck, as well as tremor of the hands. The differential diagnosis included inflammatory disease of the brain, sinus thrombosis and, especially, malignant neuroleptic syndrome and febrile catatonia. INVESTIGATIONS: The activities of creatine kinase (3840 U/l), GOT (75 U/l) and GPT (88 U/l) were all increased. Serum myoglobin was 77 micrograms/l. CSF contained blood and there was pleocytosis of 50/3 cells. The ECG showed sinus tachycardia. EEG, chest radiogram, computed tomography, magnetic resonance imaging and cerebral angiography showed no abnormalities. Febrile catatonia could not be excluded. TREATMENT AND COURSE: 4 hours after admission the patient began to respond with normal orientation. Shortly afterwards he was able to walk a few steps, i.e. there was no catatonia. But rigor, tremor, hypersalivation and tachycardia persisted and 12 hours later he developed a fever (up to 39.2 degrees C). Blood pressure varied with peak pressures up to 190/110 mm Hg. After 2 days muscle tone had clearly increased so much that voluntary movement was hardly possible. After amantadine administration (200 mg daily) the rigor improved and for the first time body temperature became normal again. There were no signs pointing to psychotic symptoms. CONCLUSION: Neuroleptic malignant syndrome is difficult to distinguish from febrile catatonia and the diagnosis can often only be made through the clinical course.

Adult↗

[Cerebral venous thrombosis, complication of the nephrotic syndrome (author's transl)].

The occurence of arterial and, more frequently, venous thrombosis in patients with the nephrotic syndrome is well documented. Several factors are involved among which the fall in plasma albumin, the rise of several coagulation factors, platelet hyperreactivity, decrease of antithrombin III. We report here the first case, to our knowledge, of a dural sinus thrombosis, complicating a nephrotic syndrome with minimal glomerular changes. The difficulties of such a diagnosis and the key value of carotid angiography to establish it are emphasized. The use of anticoagulants can prevent this complication.

Cerebral Angiography↗

Acquired dural fistulae in benign intracranial hypertension: a short case report.

Venous sinus thrombosis has been regarded as a known cause of intracranial hypertension. We report a case of long-standing raised intracranial hypertension (ICT) that presented with deteriorating vision in both eyes. Magnetic resonance (MR) imaging of the brain and cerebral angiography showed blockage of superior saggital sinus and sigmoid sinuses with bilateral dural arteriovenous fistulae (DAVF) formation.

Adult↗

[Extensive cerebral venous thrombosis resistant to heparin: local fibrinolysis with urokinase].

A 37 year-old man had headaches for 10 days, then a single tonic-clonic seizure and coma due to an extensive cerebral venous thrombosis. In spite of full-dose heparin treatment for 7 days, the clinical picture worsened along with increasing edema on CT-Scan. Direct thrombolytic treatment was then performed using transvenous catheterization and instillation of Urokinase (2.6 MU over 4 days). A near complete repermeabilization of the sinuses was obtained and the patient improved dramatically in a few days. The only adverse effect of Urokinase was hematuria. Based on our experience and review of the literature which includes 26 previous cases, direct thrombolytic therapy appears to be a relatively safe procedure. This treatment should be considered in a patient with extensive dural sinus thrombosis which fails to respond to heparin treatment.

Adult↗

Deep cerebral venous thrombosis: imaging in eight cases.

Deep cerebral venous thrombosis (DCVT) is a rare, potentially fatal disease. We carried out a retrospective analysis of eight patients presenting with DCVT, using CT and MRI data. Digital subtraction angiography (DSA), MR angiography (MRA) (2D phase-contrast), and angiography were performed in four, four and two patients, respectively. Direct (venous abnormalities) and indirect (parenchymatous involvement) signs of thrombosis were assessed. Follow-up MRI and MRA were performed on two patients. CT was initially normal in two patients, demonstrated a "cord sign" in deep veins in five and deep venous infarcts in three. When performed within a week of admission (seven patients), MRI showed thrombosis as into intense or high signal in deep veins on sagittal T1-weighted spin-echo images in all patients, and on axial T2-weighted spin-echo images in two. Deep venous infarcts were found in five patients. Direct or indirect signs of sagittal or lateral sinus thrombosis were present on CT in two patients and on MRI in five. CT angiography, MRA and DSA were concordant with MRI findings. CT venography showed persistent flow in thrombosed veins. MRI follow-up demonstrated progressive deep venous recanalisation.

Adolescent↗

[Neurologic manifestations in trichinosis].

Neurologic manifestations are present in about 10-20 percent of patients with trichinosis. They could be a serious diagnostic problem in the absence of corresponding epidemiological data and typical symptoms and signs of the disease. In untreated patients the mortality rate is about 50%. Several pathogenic mechanisms are responsible for the neurological complications in trichinosis: obstruction of brain blood vessels by larvae, cysts or granulomas, toxic vasculitis with secondary thrombosis and haemorrhages, granulomatous inflammation of the brain parenchyma and allergic reaction. Neurotrichinosis is manifested with clinical symptoms and signs of meningitis, encephalitis, polyradiculoneuritis, poliomyelitis, myastenia gravis, paresis and paralysis, with the clinical picture of systemic disease of the connective tissue involving the nervous system and, extremely rare, as a sinus thrombosis. Thus, the broad spectrum of neurological lesions in trichinosis is, probably, the results of the fact that Trichinella spiralis larvae, during haematogenic dissemination has no special affinity for particular parts of the nervous system. We present five patients with encephalitis and focal cerebral lesions in trichinosis. In one patient the neurologic manifestations were the only sign of the disease. We believe that all pathogenic mechanisms mentioned above, were involved in the onset of neurological manifestations in our patients. The diagnosis of the disease was based on the clinical picture, epidemiological data, microscopic identification of larvae in the muscular tissue, the presence of antibodies against Trichinella spiralis in cerebrospinal fluid (with preserved blood brain barrier) and in serum confirmed by IIF method, computerised tomography and magnetic resonance imaging of the brain, eosinophilia in the peripheral blood picture. One patient died, and in the remaining patients the course of the disease was favourable; they were discharged from the hospital with minimal neurologic sequelae.

Adult↗

Primary cerebral venous thrombosis in patients with cancer--a rarely diagnosed paraneoplastic syndrome. Report of three cases and review of the literature.

Idiopathic thrombosis of the cerebral venous structures is a rare complication of malignancy. The clinical, radiologic, pathologic, and laboratory findings in the 15 previously reported cases are reviewed, and three additional cases are reported. The clinical syndrome that develops in such patients is similar to that seen in other patients with cerebral venous or sinus thrombosis. However, the diagnosis is often not made clinically because cerebral involvement by neoplastic disease provides a more common and readily acceptable explantation of the observed phenomena. If venous thrombosis involving the brain is suspected, angiography provides the only certain method of diagnosis. The relationship of this thrombotic process to a cancer-associated hypercoagulable state is discussed.

Adenocarcinoma↗