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PubMed1986

Neurological manifestations of intracranial dural arteriovenous malformations.

The authors describe their experience with four cases of dural arteriovenous malformation (AVM) which led them to analyze the clinical aspects of these lesions in an attempt to understand their pathophysiology. An additional 191 previously reported cases of dural AVM's were reviewed with special attention to the mechanism of intradural, central, and peripheral nervous system manifestations. Apart from the peripheral cranial nerve symptoms, which are most likely due to arterial steal, the central nervous system (CNS) symptoms appear to be related to passive venous hypertension and/or congestion. Generalized CNS symptoms can be related to cerebrospinal fluid malabsorption due either to increased pressure in the superior sagittal sinus, to venous sinus thrombosis, or to meningeal reaction resulting from minimal subarachnoid hemorrhages. These phenomena are not related to the anatomical type of venous drainage. On the other hand, focal CNS symptoms are specifically indicative of cortical venous drainage. Seizures, transient ischemic attacks, motor weakness, and brain-stem and cerebellar symptoms can be encountered depending on the territory of the draining vein or veins. Therefore, the localizing value of focal CNS symptomatology relates to the venous territory and not to the nidus or to the arterial supply characteristics of dural AVM's. Furthermore, the venous patterns of various dural AVM's at the base of the skull are expressed by differences in their clinical presentation. Dural AVM's of the floor of the anterior cranial fossa and of the tentorium are almost always drained by the cortical veins and, therefore, have a high risk of intradural bleeding. The remarkable similarities in the manifestations of dural and brain AVM's and the differences in the manifestations of dural and spinal dural AMV's are pointed out. High-quality angiograms and a multidisciplinary approach to the study of dural AVM's will provide the best understanding of their symptoms and, therefore, the most appropriate treatment strategy.

Adult↗
PubMed1992

Endovascular treatment of intracranial dural arteriovenous fistulas with spinal perimedullary venous drainage.

Intracranial dural arteriovenous (AV) fistulas with spinal perimedullary venous drainage are rare lesions that have distinctive clinical, radiological, and therapeutic aspects. Five patients presented with an ascending myelopathy, which extended to involve the brain stem in three cases. Myelography and magnetic resonance imaging showed slightly dilated spinal perimedullary vessels. Spinal angiograms were normal in the arterial phase. Diagnosis was only possible after cerebral angiography, which demonstrated posterior fossa AV fistulas fed by meningeal arteries and draining into spinal perimedullary veins. Endovascular treatment alone resulted in angiographic obliteration of the lesion in three patients. Two patients required surgery in addition to endovascular therapy. One patient died postoperatively, and in one a transient complication of embolization was observed. Improvement after treatment was good in two cases and fair in two. Transverse sinus thrombosis was observed in three cases and was probably the cause of the aberrant venous drainage of the fistula into the spinal perimedullary veins. The pathophysiology is related to spinal cord venous hypertension. These lesions were classified as Type 5 in the Djindjian and Merland classification of dural intracranial AV fistulas. Endovascular therapy is a safe effective method in the treatment of these fistulas and should be tried first.

Adult↗
PubMed1996

Clinical significance of diffuse dural enhancement detected by magnetic resonance imaging.

This study was performed to determine the clinical significance of diffuse dural enhancement (DDE) detected by magnetic resonance (MR) imaging and to typify enhancing patterns related to inflammatory or metastatic causes. The authors retrospectively evaluated the clinical, imaging, and laboratory characteristics of 20 consecutive patients with DDE. Those with DDE and an underlying neoplastic disease (13 patients) were compared to 11 consecutive patients with cytological evidence of neoplastic leptomeningeal metastasis evaluated by MR imaging. The DDE was often associated with an underlying malignancy (13 (65%) of 20 patients) but it coexisted with leptomeningeal metastasis in only one patient. Skull metastases were evident in 10 (77%) of 13 patients and cranial nerve palsies in six (46%) of 13. Other causes of DDE were related to cerebrospinal fluid (CSF) leak or shunting (five (25%) of 20), with or without symptoms of intracranial hypotension, and to dural sinus thrombosis and pachymeningitis. Dural biopsies obtained in two patients with DDE showed a narrow rim of granulation-like tissue adherent to the dural surface facing the inner skull table. Magnetic resonance subtraction, diffusion, and perfusion studies revealed unique characteristics in patients with metastatic causes as compared to those with DDE secondary to CSF leak. None of the patients with proven leptomeningeal metastasis had DDE, but four of them presented with focal dural enhancement and two displayed apparent leptomeningeal enhancement. The findings indicate that DDE is not a radiographic hallmark of leptomeningeal metastasis in spite of the similarities in clinical manifestations (for example, headache and cranial polyneuropathy). Nonetheless, DDE is most frequently associated with metastatic malignancies and particularly with skull metastases and CSF leak. Special MR techniques can discern the underlying cause and elucidate the disparity in the pathophysiological mechanisms leading to DDE.

Aged↗
PubMed2001

Spontaneous retroclival hematoma presenting as a thunderclap headache. Case report.

An excruciating headache of instantaneous onset, or thunderclap headache, may be caused by a variety of serious disorders, including aneurysmal subarachnoid hemorrhage, cerebral venous sinus thrombosis, pituitary apoplexy, and carotid artery or vertebral artery dissection. The authors describe a patient with this type of headache who was found to have a spontaneous retroclival hematoma. A 49-year-old woman experienced an instantaneous excruciating headache. Results of computerized tomography (CT) scans of the head were normal, but on examination of the cerebrospinal fluid xanthochromia was found. Magnetic resonance (MR) imaging of the cervical spine revealed a retroclival hematoma. Three cerebral angiographic studies did not reveal the source of the hemorrhage and a repeated MR image demonstrated resolution of the hematoma. The patient made an uneventful recovery. Spontaneous retroclival hematoma is an exceedingly rare type of intracranial hemorrhage and may be associated with normal findings on CT scans. Spontaneous retroclival hematoma should be included in the differential diagnosis of thunderclap headache.

Cerebral Angiography↗
PubMed1999

Variations of the extreme-lateral craniocervical approach: anatomical study and clinical analysis of 69 patients.

OBJECT: The aim of this study was to describe six variations of the extreme-lateral craniocervical approach, their application, and treatment results. METHODS: During a 4-year period 69 patients underwent surgery in which six variations of the extreme-lateral craniocervical approach were performed. The variations included: the transfacetal approach (TFA), performed to treat four lesions in the upper cervical spine anterior or anterolateral to the spinal cord; the retrocondylar approach, to treat five intradural lesions located anterolateral to the medulla oblongata and six vascular lesions to expose the extradural segment of the vertebral artery (VA); the partial transcondylar approach (PTCA), to treat 18 intradural lesions located anterior to the medulla oblongata; the complete transcondylar approach (CTCA), to treat 13 extradural lesions that involved the lower clivus and anterior upper cervical spine; the extreme-lateral transjugular approach, to treat 14 jugular foramen tumors; and the transtubercular approach with or without division of the sigmoid sinus, to treat complex VA and vertebrobasilar junction aneurysms. An anatomical prosection was performed to study the surgical exposure of each of the six variations of the extreme-lateral craniocervical approach. Total removal was achieved in 35 (69%) of the patients with tumor; subtotal resection was achieved in 16 (31%) of those patients. In the 12 patients with VA aneurysms, seven underwent clipping, three underwent trapping and a vein graft bypass procedure, and two underwent trapping without the use of a bypass procedure. In five other patients, different cystic, inflammatory, and other vascular lesions were successfully treated. Fifty percent of the patients who underwent surgery via the TFA, 83% via the of the CTCA, and 11% via the PTCA required an occipitocervical fusion procedure. The mean Karnofsky Performance Scale score was 74.7 preoperatively and 76.4 postoperatively. Major complications were hydrocephalus (nine patients), cerebrospinal fluid leakage (seven patients), worsened cranial nerve function (seven patients), vertebrobasilar vasospasm (one patient), and sigmoid sinus thrombosis (one patient). CONCLUSIONS: To treat lesions in the region of the foramen magnum and surrounding areas, the approach should be tailored to each specific lesion to provide the needed exposure without unnecessary operative steps.

Adult↗
PubMedDate not supplied

Diabetes insipidus as a consequence of neurologic involvement in Behcet's syndrome.

OBJECTIVE: To describe a case of central diabetes insipidus that was associated with Behçet's syndrome. METHODS: We present a case report, including clinical, laboratory, and radiologic data. The pertinent literature is reviewed relative to diabetes insipidus and Behçet's syndrome, and a discussion about a possible association is presented. RESULTS: A 32-year-old man presented with diplopia and severe headaches and was found to have transverse sinus thrombosis. He reported having recurrent mouth and genital ulcers for 3 months before the current consultation. On the basis of the clinical picture, Behçet's syndrome was diagnosed. During hospitalization of the patient, polyuria and polydipsia developed, along with hypernatremia and hypotonic urine, indicative of diabetes insipidus. After desmopressin treatment was initiated, considerable improvement in clinical status and laboratory data was evident and persisted during follow-up. CONCLUSION: Behçet's syndrome, probably through vasculitic mechanisms, may result in central diabetes insipidus. This case report emphasizes that Behçet's syndrome should be considered in the differential diagnosis of diabetes insipidus.

Adult↗
PubMed1998

[Deep venous cerebral thrombosis. Report of one case].

We report a 39 years old female presenting with headache, progressive clouding of sensorium and long pathway deficit signs. Brain CAT scan showed a process involving basal ganglia, thalami and Herophilus press. A thrombosis of this vessel was suspected. The first magnetic resonance imaging was informed as a diffuse inflammatory process compatible with an encephalitis. The patient aggravated and a second magnetic resonance imaging demonstrated a partial thrombosis of sinus rectus, Galenus vein, Herophilus press and both internal cerebral veins. Intravenous heparin was started with a progressive recovery of the patient. Two months later she had an almost complete recovery with a persistent mild right brachial paresis and a memory deficit for recent events.

Adult↗
PubMed1999

Arachnoid granulations--computerized tomography and magnetic resonance imaging features: a case report.

A 71-year-old woman had arachnoid granulations found in the straight and transverse sinuses on computerized tomography (CT) and magnetic resonance imaging (MRI). The arachnoid granulations appeared as low-density masses on CT. They gave low signals on T1-weighted MR images and high signals on T2-weighted images. Arachnoid granulations are usually of no clinical significance. They have characteristic CT and MRI manifestations and should not be mistaken for sinus thrombosis or intrasinus tumors.

Aged↗
PubMed1999

[Acute severe headache: a subarachnoidal hemorrhage?].

Five patients, three women aged 87, 50, and 31 years, and two men aged 31 and 32 years, presented with severe headache of sudden onset. A sudden onset of unusually severe headache is suggestive of an intracranial haemorrhage or other serious disease, even in the absence of focal neurologic deficits. The diagnoses were subdural haematoma, cerebral venous sinus thrombosis, idiopathic thunderclap headache, subarachnoid haemorrhage, and viral meningitis, respectively. There are no characteristics from history or examination that accurately discriminate among all these causes; idiopathic thunderclap headache and subarachnoid haemorrhage are commonest. Consultation of a neurologist and further ancillary investigations are necessary for proper diagnosis and treatment.

Adult↗
PubMed2000

Intracranial angioplasty and thrombolysis.

With careful patient selection, timely neuroendovascular therapy can improve the clinical outcome of patients suffering from cerebrovascular ischemic disease. The current indications, techniques, recent experiences, limitations, and future directions of balloon angioplasty and local intra-arterial/intravenous thrombolysis in the treatment of intracranial vasospasm, atherosclerotic disease, thromboembolism, and dural sinus thrombosis are reviewed.

Angioplasty↗
PubMed1999

[Neurologic manifestations of Behçet's disease].

Neurological involvement in Behçet's disease, the cause of the disease's severe functional sequellae, is reported in 5.3 to 30% of cases. Coagulation disorders have been reported but they cannot explain the different thrombotic manifestations which are probably the consequence of an abnormal response of the vascular endothelial cells. Neurological manifestations include: a) dural sinus thrombosis which can be diagnosed by angio-MRI and whose prognosis is improved with the use of anticoagulants; b) exceptional lesions to arteries supplying the brain; c) aseptic meningitis and meningo-encephalitis; and d) exceptionally, solitary spinal cord involvement and peripheral disease. Neurological involvement can occur early or late after development of skin and mucosal signs and when inaugural make mislead diagnosis. The spinal tap usually gives objective evidence of lymphocyte meningitis. MRI is nonspecific, but the T2 and Flair sequences can evidence hypersignal areas, preferentially in the brain stem, basal nuclei, and subtentorial white matter with no preference for the periventricular regions. Spontaneous aggravation is the rule and the neurological prognosis is severe (dementia, pseudo-bulbar syndrome, loss of independence). Treatment is similar to that used for vasculitis and is aimed initially at reducing the inflammation with corticosteroids and at preventing relapse with the adjunction of an immunosuppressor. Results are better when treatment begins early; restitutio ad integrum has been observed. Duration of treatment is poorly defined: immunosuppressors have been proposed for a minimal duration of 2 years; corticosteroid therapy can be tapered off but interruption would expose to relapse. A maintenance therapy is advisable and, in our opinion, should be proposed indefinitely combining colchicine (1 to 2 mg/d), anti-aggregate doses of aspirin, and low-dose corticosteroids (1/10 mg/kg/d).

Behcet Syndrome↗
PubMed2000

Hemorrhagic complications in vein of Galen malformations.

The authors report on a series of spontaneous intracranial hemorrhages associated with vein of Galen aneurysmal malformations (VGAMs). Thirty-four children with VGAMs have been treated at this institution since 1986. Eight children (24%) harbored the mural-type malformation, and 26 (76%) had the choroidal-type lesion. Two children (25%) with mural lesions and 1 (4%) with a choroidal lesion suffered hemorrhagic complications. Two presented with acute intracranial hemorrhage. A third child developed acute intracranial hemorrhage due to delayed dural sinus thrombosis after endovascular treatment of his choroidal-type VGAM. The subjects ranged in age from 13 days to 17 months at the time of presentation. Each patient underwent rapid radiological evaluation and treatment with endovascular surgery. Post-procedural arteriography demonstrated complete occlusion of the malformation in each patient. For the 3 patients with hemorrhage, follow-up has taken place over 49-, 107-, and 43-month intervals, respectively. Vein of Galen aneurysmal malformations can present with acute intracranial hemorrhage or develop delayed intracranial hemorrhage but respond to treatment using standard endovascular techniques. The presence of hemorrhage does not de facto portend a poor prognosis.

Cerebral Angiography↗
PubMed2000

[Q fever: not just in sheep].

Four patients, a woman aged 41 years and 3 men aged 12, 47 and 44 years, developed high fever after returning from a farm vacation in the Ardèche (France). They also suffered from severe headache, a painful tightness of the chest, abdominal pain or myalgia. Other symptoms were shivers, tiredness and a cough. While physical examination revealed few abnormalities, the chest X-ray in 3 patients showed an atypical pneumonia. One of these patients developed dural venous sinus thrombosis with increased intraspinal pressure. This patient also showed signs of a slight hepatitis. Serological investigation revealed that all 4 patients were suffering from acute Q fever, a zoonosis caused by the Coxiella burnetii bacterium. All 4 had been present at lambing and sheep-shearing. The patients with pneumonia were treated with antibiotics. All 4 patients recovered well. It is known that animal birth products in particular can contain large numbers of bacteria and therefore be highly infectious.

Acute Disease↗
PubMed2000

[Fatal hypernatremia due to accidental administration of table salt].

Hypernatremia is a common electrolyte abnormality, but it is rarely attributable to excess sodium. Hypernatremia due to exogenous salt intake, caused either by accidental ingestion or as a form of child abuse, is rare, difficult to manage and results in high mortality. Although hypernatremia is easily recognized by laboratory tests, its etiology is often difficult to determine. A surprisingly small amount of salt intake can result in a fatal outcome. We report two cases of severe salt intoxication in two girls, aged 20 and 7 months, who presented with severe hypernatremia. Both had seizures after accidental salt ingestion. In the first case, salt instead of sugar was inadvertently added to two yoghurts, leading to hypernatremia and convulsions. In the second case, a mistake in the preparation of salt-saturated water as an oral rehydration solution provoked seizures, coagulopathy and longitudinal venous sinus thrombosis. Both cases developed encephalic death. We discuss the clinical course and the difficulties in the treatment of these cases in the context of the available literature.

Accidents↗
PubMedDate not supplied

[Cerebrovascular disease in pregnancy and puerperium].

UNLABELLED: Cerebrovascular diseases are the main causes of neurological events occurring during pregnancy and puerperium. METHODS: We retrospectively reviewed the clinical records and imaging scans of females presenting with stroke during pregnancy or puerperium. We selected the cases with abnormal initial CT and/or MRI and a radiological follow-up until stabilization of the neurological condition. RESULTS: Eleven patients with ages ranging from 21-38 years were found. The hospital admission occurred between the second trimester of pregnancy and the first two weeks after delivery. In six cases there was a clinical setting of preeclampsia/eclampsia (PE/E): five with reversible encephalopathy syndromes (hypertensive-like encephalopathy) and one with a hematoma in a HELLP patient. Three other cases had a clinical setting of hypertension, not fulfilling the PE/E criteria, presented with hemorrhagic infarct in one case and lenticular hematoma in the other two. The other two cases were a subarachnoid hemorrhage in a patient with a middle cerebral aneurysm and a cortical venous infarct in a patient with lateral sinus thrombosis. CONCLUSION: Hypertension is the major underlying factor in peri-partum stroke. PE/E syndrome is the major leading cause of stroke in the peri-partum and maternal morbidity/mortality. MRI is very useful in evaluating this entity and, may have a positive impact on the management protocol.

Adult↗
PubMed2002

[Hospital maternal mortality: causes and consistency between clinical and autopsy diagnosis at the Northeastern Medical Center of the IMSS, Mexico].

UNLABELLED: The aim was to study the causes of maternal mortality (MM) and the percent of concordance between the clinical diagnosis and the autopsy findings. METHODOLOGY: The autopsies of maternal death (1980-1999) from the Hospital de Especialidades, Centro Médico del Noreste, IMSS in Monterrey, México, were analyzed. The cases were classified in directly obstetric maternal mortality (DOM) and indirectly obstetric maternal mortality (IOM), the causes were studied and the percent of concordance between pre- and post-mortem diagnosis was determined. RESULTS: There were 124 deaths. Autopsy was performed in 61 (49.1%) women. In 55 cases the clinical file and the autopsy protocol were available. This was our sample for study. Sixty percent of the cases were DO. Causes of DOM were: specific hypertensive pregnancy disease (SHPD) (51.6%), sepsis (35.5%), hypovolemic shock (9.7%), anesthetic accidents (3%); causes of IOM were: sepsis (41.7%), malignancies (16.7%), hematological diseases (12.5%), cardiopathy and systemic arterial hypertension (12.5%), hepatic disorders (12.5%), and Superior Longitudinal Sinus thrombosis (4%). A 100% clinical-pathological concordance was observed in DOM cases, while only a 41.6% was found in IOM cases. In those cases of sepsis (IOM), the etiologic agents were identified only in 20% before death. CONCLUSIONS: The early detection and treatment of SHPD and the prevention of sepsis should decrease the MM. This study showed some weakness in the Health Services that should be improved.

Adult↗
PubMed2002

[Onset of Behçet's disease as meningoencephalitis and sudden blindness].

CASE REPORT: A 41-year-old man presented a clinical picture characterized by lymphocytic meningoencephalitis, visual loss in both eyes and transverse sinus thrombosis. This picture was treated with prednisone and anticoagulation. Fundus examination showed complete occlusive arteritis, periphlebitis, peripheral ischemia and perfusion macular defects affecting both eyes. The picture was suggestive of Behçet's disease. Azathioprine was added to the treatment without improvement in visual acuity. Four months later oral aphthous ulcers developed, confirming the suspected diagnosis. DISCUSSION: Behçet's disease may appear with the sudden onset of visual loss secondary to massive occlusive retinal vasculitis. The critical state of neuro-Behçet disease may delay the diagnosis. This combination of visual and neurological symptoms is associated with a poor visual prognosis (Arch Soc Esp Oftalmol 2002; 77: 275-278).

Adult↗