Technical considerations and long-term results of endovascular venous stenting to control venous hypertension from meningiomas invading intracranial venous sinuses.

OBJECTIVE: Meningiomas invading the intracranial venous sinuses may cause intracranial venous hypertension, papilledema, and visual compromise. Sinus resection and graft reconstructions, however, add significant complexity to tumor surgery, with the potential for increased morbidity. In this study, the authors explored whether venous sinus stenting might provide an alternative means of controlling venous hypertension that would be sustainable over the long term. METHODS: The authors performed a retrospective review of all 16 patients with intracranial meningiomas who underwent stenting at their institution for venous sinus compromise. At presentation, all had headache and 9 had papilledema. Thirteen patients had 1 meningioma and 3 had 2 or more. Three patients had had previous tumor resection and radiotherapy. One patient had been treated with a lumboperitoneal shunt and radiotherapy. The median length of clinical follow-up was 8 years (range 4 months-18 years). RESULTS: Venous sinus narrowing was often not confined to the site of meningioma, and bilateral transverse sinus narrowing, reminiscent of that seen in idiopathic intracranial hypertension, was present in 7 patients with sagittal sinus meningiomas. Eleven patients had stents placed solely across sinus narrowing caused by meningioma. Five patients had additional stents placed at other sites of venous narrowing at the same time: in one of these patients, a stent was placed across a defect in the sagittal sinus caused by previous surgery, and in the 4 other patients, stents were placed across nontumor narrowings of the transverse sinuses. In 1 patient, the jugular vein was also stented. Nine patients developed symptomatic in-stent restenosis at the meningioma site. Eight had further stenting procedures with variable success in restoring the in-stent lumen. The remaining patient, with a late partial relapse, is being reinvestigated. Papilledema resolved in all patients after stenting. Six patients experienced prolonged and very substantial relief of all symptoms. Five patients had persistent headache despite restoration of the sinus lumen. Five had persistent symptoms associated with resistant in-stent stenosis. There were no significant complications from any of the diagnostic or therapeutic procedures. CONCLUSIONS: In patients who are symptomatic with meningiomas obstructing the venous sinuses, successful stenting of the affected segment can give a good outcome, especially in terms of relieving papilledema. However, further procedures are often necessary to maintain stent patency, other areas of venous compromise frequently coexist, and some patients remain symptomatic despite apparently successful treatment of the index lesion. Long-term surveillance is a requirement.

Case report: Post-traumatic Tourette syndrome resolving with treatment of jugular venous narrowing; reconciling organic brain dysfunction following whiplash trauma with the absence of direct brain injury.

We describe a man aged 33 years who developed multiple symptoms, personality change, and a severe tic disorder following a road traffic accident, which were undiminished for 3 years until jugular venous narrowing between the styloid process of the skull and the transverse process of the C1 vertebra was treated by surgical decompression. Immediately following surgery, his abnormal movements almost completely resolved, with no regression in 5 years of follow-up. Vigorously debated at the time was whether or not his condition represented a functional disorder. Unrecognized throughout his illness, however, was a complaint of intermittent, profuse discharge of clear fluid from his nose that began on the day of the accident and continued up to the time of surgery, after which it was substantially reduced. This outcome reinforces the idea that jugular venous narrowing can cause or perpetuate a cerebrospinal fluid leak. It suggests that the interaction between these two pathological defects may have a profound effect on brain function in the absence of any demonstrable brain lesion. It invites a reevaluation of normal head and neck venous anatomy. It should strike a cautionary note in the diagnosis of functional illness. It invites exploration of a remediable structural cause for Tourette syndrome.

Life changing response to successive surgical interventions on cranial venous outflow: A case report on chronic fatigue syndrome.

Recognition of similarities between chronic fatigue syndrome and idiopathic intracranial hypertension (IIH) has raised suggestions that they might be connected, with chronic fatigue syndrome representing a mild version of IIH, sharing many of its symptoms, but without the signature features of elevated intracranial pressure that characterize the complete syndrome. A further development of this idea factors in the effects of a cerebrospinal fluid leak, a known complication of IIH, to explain cases where symptoms seem out of proportion to the apparent physiological disturbance. Cranial venous outflow obstruction has been proposed as the pathological substrate. We describe a patient with multiple symptoms, including headache and disabling fatigue, in which this model guided investigation and treatment. Specifically, CT and catheter venography identified focal narrowings of both jugular and the left brachiocephalic veins. Treatment of brachiocephalic obstruction was not feasible. However, in separate surgical procedures, relief of jugular venous obstruction produced incremental and significant clinical improvements which have proven durable over the length of follow-up. We suggest that investigating chronic fatigue syndrome under this model might not only bring benefit to individual patients but also will provide new insights into IIH and its relationship with spontaneous intracranial hypotension.

A paradigm for chronic fatigue syndrome: caught between idiopathic intracranial hypertension and spontaneous intracranial hypotension; caused by cranial venous outflow obstruction.

Background: Clinical similarities between chronic fatigue syndrome and idiopathic intracranial hypertension, supported by measurements of intracranial pressure, invite suggestions that they may be connected, the first representing a mild version of the second. Yet, if this is to be the basis for a structural explanation for chronic fatigue syndrome, it already seems incomplete, failing to explain cases where disability seems disproportionate. Is there some other confounding variable? Purpose: To refine, in this theoretical paper, an earlier model connecting chronic fatigue syndrome with idiopathic intracranial hypertension to allow for a cerebrospinal fluid (CSF) leak. Model: In this model, the primary structural problem is acquired obstruction to cranial venous outflow. This obstruction can take different forms, may be intermittent and subtle, and even be mistaken for normal venous anatomy, yet would be the driving force behind a tendency towards increased intracranial pressure. This chronic elevation of intracranial pressure stresses the dural membrane maintaining the integrity of the subarachnoid space, which can rupture at a weak point, allowing CSF to leak away and intracranial pressure to fall. The clinical manifestation of this disorder is the product of the severity of cranial venous outflow compromise and of the competing forces on intracranial pressure. In some instances, a CSF leak will mitigate the effects of venous compromise, in others it will compound it, producing a disease spectrum ranging through idiopathic intracranial hypertension, chronic fatigue syndrome, fibromyalgia, and spontaneous intracranial hypotension. Conclusion: In chronic fatigue syndrome a normal intracranial pressure does not exclude significant physiological disturbance.

Chronic fatigue syndrome and idiopathic intracranial hypertension: Different manifestations of the same disorder of intracranial pressure?

Though not discussed in the medical literature or considered in clinical practice, there are similarities between chronic fatigue syndrome and idiopathic intracranial hypertension (IIH) which ought to encourage exploration of a link between them. The cardinal symptoms of each - fatigue and headache - are common in the other and their multiple other symptoms are frequently seen in both. The single discriminating factor is raised intracranial pressure, evidenced in IIH usually by the sign of papilloedema, regarded as responsible for the visual symptoms which can lead to blindness. Some patients with IIH, however, do not have papilloedema and these patients may be clinically indistinguishable from patients with chronic fatigue syndrome. Yet IIH is rare, IIH without papilloedema (IIHWOP) seems rarer still, while chronic fatigue syndrome is common. So are the clinical parallels spurious or is there a way to reconcile these conflicting observations? We suggest that it is a quirk of clinical measurement that has created this discrepancy. Specifically, that the criteria put in place to define IIH have led to a failure to appreciate the existence, clinical significance or numerical importance of patients with lower level disturbances of intracranial pressure. We argue that this has led to a grossly implausible distortion of the epidemiology of IIH such that the milder form of the illness (IIHWOP) is seen as less common than the more severe and that this would be resolved by recognising a connection with chronic fatigue syndrome. We hypothesise, therefore, that IIH, IIHWOP, lesser forms of IIH and an undetermined proportion of chronic fatigue cases are all manifestations of the same disorder of intracranial pressure across a spectrum of disease severity, in which this subset of chronic fatigue syndrome would represent the most common and least severe and IIH the least common and most extreme.

Clinical Experience and Results of Microsurgical Resection of Arterioveonous Malformation in the Presence of Space-Occupying Intracerebral Hematoma.

BACKGROUND: Management of ruptured arteriovenous malformations (AVMs) with a mass-producing intracerebral hematoma (ICH) represents a surgical dilemma. OBJECTIVE: To evaluate the clinical outcome and obliteration rates of microsurgical resection of AVM when performed concomitantly with evacuation of an associated space-occupying ICH. METHODS: Data of patients with AVM were collected prospectively. Cases were identified in which an AVM was resected and an associated space-occupying ICH was evacuated at the same time, and divided into "group 1," in which the surgery was performed acutely within 48 h of presentation (secondary to elevated intracranial pressure); and "group 2," in which selected patients were operated upon in the presence of a liquefying ICH in the "subacute" stage. Clinical outcomes were assessed using the modified Rankin Scale, with a score of 0 to 2 considered a good outcome. Obliteration rates were assessed using postoperative angiography. RESULTS: From 2001 to 2015, 131 patients underwent microsurgical resection of an AVM, of which 65 cases were included. In "group 1" (n = 21; Spetzler-Ponce class A = 13, class B = 5, and class C = 3), 11 of 21 (52%) had a good outcome and in 18 of 19 (95%) of those who had a postoperative angiogram the AVMs were completely obliterated. In "group 2" (n = 44; Spetzler-Ponce class A = 33, class B = 9, and class C = 2), 31 of 44 (93%) had a good outcome and 42 of 44 (95%) were obliterated with a single procedure. For supratentorial AVMs, the ICH cavity was utilized to provide an operative trajectory to a deep AVM in 11 cases, and in 26 cases the ICH cavity was deep to the AVM and hence facilitated the deep dissection of the nidus. CONCLUSION: In selected patients the presence of a liquefying ICH cavity may facilitate the resection of AVMs when performed in the subacute stage resulting in a good neurological outcome and high obliteration rate.

Auditory hallucination in basilar occlusion: I heard it was the basilar.

Acute basilar artery occlusion is a neurological emergency. Unlike anterior circulation stroke presenting with hemiparesis, the symptoms of basilar artery occlusion are challenging to recognise in the emergency setting. Basilar artery occlusion can rarely lead to ischaemia of the auditory pathways, resulting in bizarre, positive auditory hallucinations. Here, we report two cases of basilar artery occlusion presenting with positive auditory phenomena; in both cases the auditory phenomenon resolved upon arterial recanalisation. We discuss the phenomenology of this unusual and distinctive neurological symptom. Acute auditory hallucinosis in the setting of sudden vomiting, dizziness, visual disturbance or other posterior circulation symptoms should prompt emergency imaging of the basilar artery, to avoid a potentially devastating posterior circulation stroke.

Recovery of oculomotor nerve palsy secondary to posterior communicating artery aneurysms.

BACKGROUND: Recent studies suggest more favourable recovery of oculomotor nerve palsy (ONP) caused by posterior communicating artery (PComA) aneurysms with microsurgical clipping compared to endovascular coiling. We describe a consecutive series of patients with ONP from PComA aneurysms treated by microsurgical clipping or endovascular coiling. METHODS: We retrospectively reviewed medical records of all patients from 2005 to 2009 with complete or partial ONP from PComA aneurysms. RESULTS: Twenty patients were identified, three with unruptured aneurysms. Two patients with ruptured aneurysms were unfit for treatment and therefore excluded. Of the 18 patients included (15 female), 9 underwent microsurgical clipping and 9 received endovascular coiling. Patients treated by surgical clipping were significantly younger compared to those treated by endovascular coiling (mean 52.3 vs. 67.9 years; p = 0.039). Five patients had incomplete ONP (3 clipped, 2 coiled) and thirteen had complete ONP. At 6 months, six of nine patients treated with clipping and five of nine patients treated with coiling had complete resolution of their ONP (p = 1.0); the remainder had partial improvement. There was no significant difference in duration of pre-treatment ONP, age, sex or status of aneurysm (ruptured or unruptured) between patients in the two groups or between those with full or partial recovery. However, all 5 patients with incomplete ONP at presentation recovered fully, compared with 6 of 13 patients who presented with complete ONP. CONCLUSIONS: We found no significant difference between clipping and coiling in the recovery of ONP due to PComA aneurysms. Patient who present with incomplete ONP are more likely to have a full recovery of ONP following either treatment modality than those who present with complete ONP.

Stenting venous outflow gives symptomatic improvement in a patient with an inoperable brainstem arteriovenous malformation.

The extent to which arterial steal or venous hypertension contributes to symptoms in patients with high flow brain arteriovenous malformations (AVMs) is not always clear. We describe a patient with an inoperable AVM of the pons, presenting with headache and neurological deficit where improving venous outflow by stenting produced substantial clinical benefit.

Mirror-image temporal arteriovenous malformations presenting with left cervical internal carotid aneurysm.

We report an unusual case of bilateral mirror-image arteriovenous malformations (AVMs) in association with a large internal carotid aneurysm. The patient, a 45-year-old man, was asymptomatic apart from a lump in his neck. Both AVMs were successfully occluded with embolisation and the patient remains clinically well.

Coiling of a residual internal carotid artery aneurysm via an extracranial-intracranial bypass graft following parent vessel occlusion.

We report the case of a ruptured internal carotid artery (ICA) aneurysm that demonstrated significant persistent filling despite management with a common carotid to middle cerebral artery saphenous vein extracranial-intracranial bypass and subsequent permanent ICA balloon occlusion. The residual aneurysm was successfully embolised with detachable coils using a novel endovascular approach via the bypass graft.

The Tuberous Sclerosis 2000 Study: presentation, initial assessments and implications for diagnosis and management.

AIMS: The Tuberous Sclerosis 2000 Study is the first comprehensive longitudinal study of tuberous sclerosis (TS) and aims to identify factors that determine prognosis. Mode of presentation and findings at initial assessments are reported here. METHODS: Children aged 0-16 years newly diagnosed with TS in the UK were evaluated. RESULTS: 125 children with TS were studied. 114 (91%) met clinical criteria for a definite diagnosis and the remaining 11 (9%) had pathogenic TSC1 or TSC2 mutations. In families with a definite clinical diagnosis, the detection rate for pathogenic mutations was 89%. 21 cases (17%) were identified prenatally, usually with abnormalities found at routine antenatal ultrasound examination. 30 cases (24%) presented before developing seizures and in 10 of these without a definite diagnosis at onset of seizures, genetic testing could have confirmed TS. 77 cases (62%) presented with seizures. Median age at recruitment assessment was 2.7 years (range: 4 weeks-18 years). Dermatological features of TS were present in 81%. The detection rate of TS abnormalities was 20/107 (19%) for renal ultrasound including three cases with polycystic kidney disease, 51/88 (58%) for echocardiography, 29/35 (83%) for cranial CT and 95/104 (91%) for cranial MRI. 91% of cases had epilepsy and 65% had intellectual disability (IQ<70). CONCLUSIONS: Genetic testing can be valuable in confirming the diagnosis. Increasing numbers of cases present prenatally or in early infancy, before onset of seizures, raising important questions about whether these children should have EEG monitoring and concerning the criteria for starting anticonvulsant therapy.

Severe brain edema caused by a meningioma obstructing cerebral venous outflow and treated with venous sinus stenting. Case report.

Peritumoral edema is a common feature of cerebral meningiomas, but venous outflow obstruction is not normally considered as a cause even when a tumor involves the venous sinuses. The authors describe a patient with a ventricu-loperitoneal shunt in situ, who had undergone several debulking operations and conventional radiotherapy and in whom had developed progressive life-threatening edema from a tentorial meningioma. Radiological studies showed occlusion of the straight sinus and stenosis of the sagittal sinus at the confluence of sinuses. The sagittal sinus was stented, and thereafter the patient made an almost complete symptomatic recovery and returned to work. This case shows that progressive edema in patients with meningioma is not necessarily caused by radiotherapy or tumor enlargement into the brain and that in venous outflow obstruction, addressing the obstructive lesion directly can improve the palliation afforded by a shunt.

Stent placement for management of a small parasagittal meningioma. Technical note.

The patient in this report had a parasagittal meningioma with an intrasinus extension that presented with features of benign intracranial hypertension and no focal neurological deficit or seizure. The meningioma was managed with a combination of endovascular stent placement and radiotherapy. The authors describe the investigation and technical aspects of stent placement for the stenosed sinus. Good symptomatic relief in the patient was achieved.

Intracranial aneurysms treated with endovascular coils: detection of recurrences using unenhanced and contrast-enhanced transcranial color-coded duplex sonography.

BACKGROUND AND PURPOSE: Because neck recurrence after endovascular treatment of intracranial aneurysms (IAs) is not uncommon, surveillance to assess long-term stability of occlusion is clearly important. This study evaluated unenhanced and contrast-enhanced transcranial color-coded duplex sonography (TCCS) in detecting refilling of IAs treated with detachable coils. METHODS: Patients with coiled IAs were imaged before and after contrast enhancement. The results were compared with those of a surveillance digital subtraction angiogram (DSA). The operator was blinded to the results of the DSA. Aneurysms were classed as either occluded or with residual flow and quantified as minor, moderate, or extensive. There were 208 studies performed in 4 neurosurgical centers. Of those, 141 studies received ultrasonic contrast enhancement with Levovist, and 68 had an additional enhanced study with SonoVue. RESULTS: We excluded 44 studies. Of the 164 unenhanced studies, TCCS correctly identified 52 of 67 cases defined as completely occluded by DSA (sensitivity 78%; specificity 77%), 13 of 50 aneurysms with minor refilling (sensitivity 26%; specificity 88%), 15 of 27 aneurysms with moderate refilling (sensitivity 56%; specificity 95%), and 9 of 20 aneurysms with extensive refilling (sensitivity 45%; specificity 100%). TCCS correctly identified an additional 10 aneurysms with minor refilling after Levovist enhancement and 3 with SonoVue. Both SonoVue and Levovist enhancement identified an additional 1 aneurysm with moderate refilling and 3 with extensive refilling. CONCLUSIONS: TCCS could be used to selectively monitor IAs, which would reduce the requirement for long-term invasive monitoring. The detection of neck refilling is improved with contrast enhancement.

Monozygotic twins with tuberous sclerosis discordant for the severity of developmental deficits.

A pair of monozygotic male twins with tuberous sclerosis (TS) were followed between 18 months and 3 years of age. Twin A with 25 large cortical tubers and hence extensive brain involvement was moderately mentally retarded and met criteria for autism. The other twin had more (n = 31) but smaller tubers. He was not mentally retarded and did not meet criteria for autism. This study provides evidence that nongenetic factors such as extent of brain abnormality and not just number of cortical tubers are important in determining phenotypic variability in TS. The findings also raise questions about the mechanisms giving rise to autism in TS.