Complex neurological symptoms in bilateral thalamic stroke due to Percheron artery occlusion.

The artery of Percheron is a rare anatomical variant where a single thalamic perforating artery arises from the proximal posterior cerebral artery (P1 segment) between the basilar artery and the posterior communicating artery and supplies the rostral mesencephalon and both paramedian territories of the thalami. Almost one-third of human brains present this variant. Occlusion of the artery of Percheron mostly results in a bilateral medial thalamic infarction, which usually manifests with altered consciousness (including coma), vertical gaze paresis, and cognitive disturbance. The presentation is similar to the "top of the basilar syndrome", and early recognition should be prompted. We describe the case of a young female with this vessel variant who experienced a bilateral thalamic stroke. Magnetic resonance angiography demonstrated bilateral thalamic infarcts and a truncated artery of Percheron. Occlusion of the vessel was presumably due to embolism from a patent foramen ovale. Thrombolysis was performed, with incomplete symptom remission, cognitive impairment, and persistence of speech disorders. Early recognition and treatment of posterior circulation strokes is mandatory, and further investigation for underlying stroke etiologies is needed.

Dural arteriovenous fistula-induced thalamic dementia: report of 4 cases.

Nonhemorrhagic neurological deficits are underrecognized symptoms of intracranial dural arteriovenous fistulas (dAVFs) having cortical venous drainage. These symptoms are the consequence of cortical venous hypertension and portend a clinical course with increased risk of neurological morbidity and mortality. One rarely documented and easily misinterpreted type of nonhemorrhagic neurological deficit is progressive dementia, which can result from venous hypertension in the cortex or in bilateral thalami. The latter, which is due to dAVF drainage into the deep venous system, is the less common of these 2 dementia syndromes. Herein, the authors report 4 cases of dAVF with venous drainage into the vein of Galen causing bithalamic edema and rapidly progressive dementia. Two patients were treated successfully with endovascular embolization, and the other 2 patients were treated successfully with endovascular embolization followed by surgery. The radiographic abnormalities and presenting symptoms rapidly resolved after dAVF obliteration in all 4 cases. Detailed descriptions of these 4 cases are presented along with a critical review of 15 previously reported cases. In our analysis of these 19 published cases, the following were emphasized: 1) the clinical and radiographic differences between dAVF-induced thalamic versus cortical dementia syndromes; 2) the differential diagnosis and necessary radiographic workup for patients presenting with a rapidly progressive thalamic dementia syndrome; 3) the frequency at which delays in diagnosis occurred and potentially dangerous and avoidable diagnostic procedures were used; and 4) the rapidity and completeness of symptom resolution following dAVF treatment.

Dural arteriovenous fistula (D-AVF): a rare cause of symmetrical bithalamic changes with free diffusivity changes.

We report a rare case of an encephalopathic presentation of a posterior fossa dural arteriovenous fistula with concomitant florid symmetrical bithalamic free diffusivity changes on MRI in a previously healthy individual. We describe the structural and functional imaging findings and the role of catheter angiography in diagnosis, prognostication and the timing and strategy in the management of this challenging vascular entity.

[Bilateral thalamic ischaemia secondary to a tentorial dural fistula]. / Isquemia talamica bilateral secundaria a fistula dural tentorial.

TITLE: Isquemia talamica bilateral secundaria a fistula dural tentorial.

Redefining thalamic vascularization vicariously through gerald percheron: a historical vignette.

Gerard Percheron, M.D., a practicing neurologist and prolific researcher at the Institute Nationale de la Sante et de la Recherche Medicale (INSERM), made significant and valuable contributions to medicine, in particular, to the vascular anatomy of the basal ganglia. His particular interest in the thalamus eventually led to the identification of an anatomic variation in its vascular supply. This newly identified artery was subsequently named the artery of Percheron (AOP). Given the estimated prevalence of the AOP in up to one third of the population and its significant proportion of all thalamic infarcts, it is necessary for physicians to be aware of this anatomic vascular variant and its clinical consequences. Although occlusion of the AOP may present similar to other arterial thalamic occlusions, it can be identified through susceptibility-weighted imaging and ruled out with conventional or magnetic resonance angiography. Occlusion of the AOP typically causes a simultaneous and symmetric infarction. Treatment efficacy is time-dependent and necessitates thrombolytics and anticoagulative medications. Here, we trace a course from the artery's initial description in 1973 to its current implications in cerebrovascular stroke, and offer a synopsis of the proposed treatment.

Cognitive decline and hypersomnolence: thalamic manifestations of a tentorial dural arteriovenous fistula (dAVF).

BACKGROUND: Intracranial dural arteriovenous fistulas (dAVFs) often present with pulsatile tinnitus, orbital congestion, and headache. Occasionally, they present with focal neurologic deficits, a dementia-like syndrome, hemorrhage, or ischemic infarction. METHODS: This study is based on the case of a 71-year-old gentleman who presented with 6 months of progressive forgetfulness, inattention, and hypersomnolence. Four weeks prior to presentation, he developed symptoms of left-sided pain, numbness, and worsening weakness. Neurologic examination demonstrated hypersomnolence, a score of 30/38 on the Kokmen Short Test of Mental Status, and left hemiparesis. MRI brain revealed bilateral thalamic T2 hyperintensities with associated enhancement. MR venogram (MRV) showed a vascular malformation in the posterior fossa and occlusion of the straight sinus. Conventional cerebral angiogram confirmed a tentorial dAVF. The dAVF was definitively treated with transarterial embolization, followed by clip ligation of the arterialized draining vein. Twelve weeks later, there was clinical resolution of left hemiparesis and improvement in cognitive status. MRI revealed complete resolution of the thalamic hyperintensities. MRV demonstrated recanalization of the straight sinus. RESULTS: Intracranial dAVFs are uncommon but potentially life-threatening acquired vascular malformations. The initiating factor is venous hypertension, causing retrograde flow, venous congestion, ischemia, and sometimes infarction. The spectrum of clinical manifestations in dAVFs reflects the degree of venous congestion present. If retrograde venous flow is surgically obliterated, then venous hypertension may be reversible. Bilateral thalamic venous congestion can present as a thalamic dementia. CONCLUSION: We conclude that intracranial dAVFs with thalamic venous congestion should be considered in the diagnostic differential for patients who present with subacute cognitive decline and T2 hyperintense thalamic signal change.

Carotid endarterectomy in patients with foetal-type posterior circle of Willis: is there an indication for local anaesthesia?

Foetal-type posterior circle of Willis is a common anatomical variation with a variable degree of vessel asymmetry. In patients with this abnormality, carotid endarterectomy (CEA) may create cerebral hypo-perfusion intraoperatively, and this may be underestimated under general anaesthesia. There is currently no evidence that anatomical variations in the circle of Willis represent an independent risk factor for stroke. Moreover, there is a paucity of data on treating patients with such anatomical variations and co-existing ICA stenosis. We present a case of CEA under local anaesthesia (LA) in a 52-year-old female patient with symptomatic stenosis of the right ICA and coexistent foetal-type posterior circle of Willis. There were no post-operative complications and she was discharged free from symptoms. She was seen again 3 months later and was free from complications. This case higlights that LA should be strongly considered to enable better intra-operative neurological monitoring in the event of foetal-type posterior circle of Willis.

Ondine's curse with accompanying trigeminal and glossopharyngeal neuralgia secondary to medullary telangiectasia.

BACKGROUND: Central hypoventilation syndrome ("Ondine's Curse") is an infrequent disorder that can lead to serious acute or chronic health consequences. This syndrome, especially in adults, is rare, and even less frequent in the absence of clear pathogenic lesions on MRI. In addition, we are not aware of any previously reported cases with associated cranial nerve neuralgias. METHODS: We describe a patient with baseline trigeminal and glossopharyngeal neuralgia, admitted with episodes of severe hypoventilatory failure of central origin, consistent with "Ondine's Curse". After evaluation, she was found to have a medullary capillary telangiectasia, thought to be the causative lesion, and which could explain her complete neurologic and hypoventilatory syndrome. The patient was treated with placement of a diaphragmatic pacing system, which has been effective thus far. RESULTS: This case illustrates the need for investigation of centrally mediated apnea, especially when co-occurring cranial nerve neuralgia is present and cardiopulmonary evaluation is negative. It provides an example of capillary telangiectasia as the causative lesion, one that to our knowledge has not been reported before. CONCLUSIONS: Placement of a diaphragmatic pacing system was warranted and became lifesaving as the patient was deemed to be severely incapacitated by chronic ventilatory insufficiency.

Endovascular angioplasty and stent placement in venous hypertension related to dural arteriovenous fistulas and venous sinus thrombosis.

Venous hypertension in lateral sinuses resulting from dural arteriovenous fistula (DAVF) and venous sinus thrombosis (VST) can manifest with severe neurological deficits, such as infarction or intracerebral hemorrhage. It has been proved that venous hypertension plays a significant role in the evolution and progression of DAVF and VST. The definite treatment in complicated conditions such as multiple DAVFs or multiple sinus occlusions is still unknown. Traditional transarterial embolization, transvenous embolization or radiosurgery alone has limited effects on these conditions. We reported one case with venous hypertension presenting with severe neurological symptoms. The case had quick clinical recovery after correction of venous hypertension by endovascular angioplasty and stent placement in occluded lateral sinuses. Accordingly, we propose this method can be an ideal treatment option either in single or staged therapy of venous hypertension related to DAVFs and VST.

Vertebral and spinal cavernous angiomas associated with familial cerebral cavernous malformation.

BACKGROUND: Cerebral cavernous malformations are vascular malformations that affect the CNS and have been associated with cutaneous, retinal, and hepatic lesions. Until now, vertebral hemangiomas associated with CCM have been described only in one case. The coexistence of intracranial and spinal cavernous angiomas in familial CCM is extremely rare. In addition to previous studies, the occurrence of spinal, vertebral, and cutaneous cavernous angiomas is now described in different members of a large family with CCM. CASE DESCRIPTION: Our study reports a previously described family (IFCAS-07) with 12 members affected by autosomal dominant cavernous angiomas: 11 had CCM either alone or associated with hepatic or retinal angiomas, and one had only hepatic angioma. In all 11 members affected by CCM, the mutation of CCM1 gene was detected. During the follow-up, 8 subjects underwent a spinal MRI: 2 because they were symptomatic (thoracic paresthesias, enuresis, back pain) and 6 as a screening examination. Spinal MRI showed in 5 subjects spinal cavernous angiomas either alone or associated with vertebral hemangiomas. CONCLUSIONS: To our knowledge, this is the largest family reported with different subjects affected by CCM associated with multiple cavernous angiomas throughout (brain and spinal cord) and besides (retina, skin, liver, and vertebral column) the CNS. Comprehensive care of patients with familial CCM includes screening of all the tissues that can be affected and appropriate management by specialists. We emphasize the importance of spinal MRI in the diagnosis of spinal and vertebral cavernous angiomas in all patients affected by familial CCM.

Neuropyschological profile of reversible cognitive impairment in a patient with a dural arteriovenous fistula.

OBJECTIVE AND IMPORTANCE: Patients with dural arteriovenous fistulas (dAVFs) may present with cognitive impairment secondary to venous hypertension or ischemia. CLINICAL PRESENTATION: We present a patient with a dAVF supplied by the posterior meningeal artery who presented with severe encephalopathy and imaging consistent with bilateral thalamic ischemia. RESULTS: Detailed pre-operative neuropsychological testing documented severe cognitive deficits across multiple domains, localizing diffusely in the cerebral cortex, beyond that which would be expected from purely thalamic involvement. Approximately 2 months following a combined endovascular and surgical repair, repeat neuropsychological testing documented a dramatic improvement in cognitive symptoms while MRI abnormalities in the thalami resolved. CONCLUSION: Detailed neuropsychological testing may be useful in patients presenting with dAVFs in order to identify cognitive impairment, which may be out of proportion to imaging findings. Recognition of dAVF-associated cognitive impairment may lead to more aggressive, timely treatment in patients with otherwise lower-risk lesions. This detailed testing can also provide a baseline in order to document cognitive recovery after fistula repair.

Dural arteriovenous fistulas with aggressive course: clinical and angiographic correlations in two patients.

Cranial dural arteriovenous fistulas (DAVFs) usually present with non-aggressive symptoms. We here report two patients who presented a peculiar clinical picture related to DAVFs, with focal neurological signs and haemorrhagic (case 1) or ischaemic lesions (case 2) respectively. The clinical and angiographic findings and putative pathophysiological mechanisms are discussed.

Aneurysm originating from the fenestration of the posterior cerebral artery: case report.

OBJECTIVE AND IMPORTANCE: A rare case of an aneurysm arising at the fenestration of the P2 segment of the posterior cerebral artery is reported. CLINICAL PRESENTATION: A 37-year-old man presented with severe headache and disturbance of consciousness. Computed tomographic scanning showed diffuse subarachnoid hemorrhage. Cerebral angiography revealed an aneurysm at the fenestration of the P2 segment of the right posterior cerebral artery. The aneurysm was located at the middle portion of the lower trunk of the fenestration. An unruptured arteriovenous malformation was incidentally found in the right thalamus. INTERVENTION: A right frontotemporal craniotomy with orbitozygomatic osteotomy was made, and the aneurysm was successfully clipped. One year after the operation, gamma knife surgery was performed for the right thalamic arteriovenous malformation. CONCLUSION: This is the first reported case of an aneurysm originating from the middle portion of a fenestrated posterior cerebral artery.