Reversible cerebral vasoconstriction syndrome.

Reversible cerebral vasoconstriction syndrome (RCVS) is a relatively rare, non-progressive angiopathy frequently heralded by severe thunderclap headache. It is characterised by vasoconstriction of cerebral arteries which usually resolves within three months of onset. Transient focal neurological signs may occur, and persistent deficits associated with haemorrhagic comorbidities have been reported in a small percentage of individuals. In this paper we report the case of RH, a 36-year-old woman who presented at a university teaching hospital in Sydney with a clinical history and radiological evidence consistent with RCVS. There were no haemorrhagic events during the course of her illness, and vasoconstriction resolved within a few days, following treatment with verapamil. Neuropsychological evaluation 16 months later revealed significant deficits in autobiographical memory, verbal and non-verbal new learning and active delayed recall, cognitive flexibility, abstraction and (to a lesser extent) immediate attention span and information processing speed. RH's case was unusual because the "trigger" for RCVS (Ear, Nose and Throat surgery) has not been previously reported, and because despite there being no haemorrhagic complications during the course of RCVS and no subsequent radiological abnormalities, she had significant cognitive impairment. To date, persistent neuropsychological deficits have not been recognised as a feature of RCVS.

Cerebral vasospasm in acute porphyria.

BACKGROUND AND PURPOSE: Porphyrias are a group of inherited metabolic disorders resulting from a specific deficiency along the pathway of haem biosynthesis. A clinical classification distinguishes acute from non-acute porphyrias considering the occurrence of life-threatening neurovisceral attacks, presenting with abdominal pain, neuropsychiatric disturbance and neuropathy. Vasospasm is a very rare complication that can occur in all major types of acute porphyria. METHODS: We describe a porphyric crisis with vasospasm in a woman with previously undiagnosed acute porphyria. Furthermore we performed a systematic review by searching the electronic database Pubmed/MEDLINE for additional data in published studies of vasospasm in acute porphyria. RESULTS: Overall, 9 case reports reporting on 11 patients who suffered vasospasm during an exacerbation of acute porphyria were identified. All of the reported patients were women and the mean age was 29.4 years. When brain MRI was performed, T2-hyperintense lesions, consistent with ischaemic changes, were observed in most patients (10/11, 91%). Although the genetic pathogenesis of the disease is well understood, the precise mechanisms to explain neurologic involvement in acute porphyria remain unclear. CONCLUSION: Acute porphyria is an unusual and rare cause of vasospasm. However, considering porphyria in patients with unexplained cerebral vasospasm, especially in women of childbearing age, is crucial given the severity of possible complications and the available treatment options.

Defining vasospasm after subarachnoid hemorrhage: what is the most clinically relevant definition?

BACKGROUND AND PURPOSE: Vasospasm is an important complication of subarachnoid hemorrhage, but is variably defined in the literature. METHODS: We studied 580 patients with subarachnoid hemorrhage and identified those with: (1) symptomatic vasospasm, defined as clinical deterioration deemed secondary to vasospasm after other causes were eliminated; (2) delayed cerebral ischemia (DCI), defined as symptomatic vasospasm, or infarction on CT attributable to vasospasm; (3) angiographic spasm, as seen on digital subtraction angiography; and (4) transcranial Doppler (TCD) spasm, defined as any mean flow velocity >120 cm/sec. Logistic regression analysis was performed to test the association of each definition of vasospasm with various hospital complications, and 3-month quality of life (sickness impact profile), cognitive status (telephone interview of cognitive status), instrumental activities of daily living (Lawton score), and death or severe disability at 3 months (modified Rankin scale score 4-6), after adjustment for covariates. RESULTS: Symptomatic vasospasm occurred in 16%, DCI in 21%, angiographic vasospasm in 31%, and TCD spasm in 45% of patients. DCI was statistically associated with more hospital complications (N=7; all P<0.05) than symptomatic spasm (N=4), angiographic spasm (N=1), or TCD vasospasm (N=1). Angiographic and TCD vasospasm were not related to any aspect of clinical outcome. Both symptomatic vasospasm and DCI were related to reduced instrumental activities of daily living, cognitive impairment, and poor quality of life (all P<0.05). However, only DCI was associated with death or severe disability at 3 months (adjusted OR, 2.2; 95% CI, 1.2-3.9; P=0.007). CONCLUSIONS: DCI is a more clinically meaningful definition than either symptomatic deterioration alone or the presence of arterial spasm by angiography or TCD.

Neurobehavioral testing in subarachnoid hemorrhage: A review of methods and current findings in rodents.

The most important aspect of a preclinical study seeking to develop a novel therapy for neurological diseases is whether the therapy produces any clinically relevant functional recovery. For this purpose, neurobehavioral tests are commonly used to evaluate the neuroprotective efficacy of treatments in a wide array of cerebrovascular diseases and neurotrauma. Their use, however, has been limited in experimental subarachnoid hemorrhage studies. After several randomized, double-blinded, controlled clinical trials repeatedly failed to produce a benefit in functional outcome despite some improvement in angiographic vasospasm, more rigorous methods of neurobehavioral testing became critical to provide a more comprehensive evaluation of the functional efficacy of proposed treatments. While several subarachnoid hemorrhage studies have incorporated an array of neurobehavioral assays, a standardized methodology has not been agreed upon. Here, we review neurobehavioral tests for rodents and their potential application to subarachnoid hemorrhage studies. Developing a standardized neurobehavioral testing regimen in rodent studies of subarachnoid hemorrhage would allow for better comparison of results between laboratories and a better prediction of what interventions would produce functional benefits in humans.

Non-invasive assessment of vasospasm following aneurysmal SAH using C-arm FDCT parenchymal blood volume measurement in the neuro-interventional suite: Technical feasibility.

INTRODUCTION: Cerebral vasospasm is the leading cause of morbidity and mortality in patients with aneurysmal subarachnoid haemorrhage (SAH) surviving the initial ictus. Commonly used techniques for vasospasm assessment are digital subtraction angiography and transcranial Doppler sonography. These techniques can reliably identify only the major vessel spasm and fail to estimate its haemodynamic significance. To overcome these issues and to enable comprehensive non-invasive assessment of vasospasm inside the interventional suite, a novel protocol involving measurement of parenchymal blood volume (PBV) using C-arm flat detector computed tomography (FDCT) was implemented. MATERIALS AND METHODS: Patients from the neuro-intensive treatment unit (ITU) with suspected vasospasm following aneurysmal SAH were scanned with a biplane C-arm angiography system using an intravenous contrast injection protocol. The PBV maps were generated using prototype software. Contemporaneous clinically indicated MR scan including the diffusion- and perfusion-weighted sequences was performed. C-arm PBV maps were compared against the MR perfusion maps. RESULTS: Distribution of haemodynamic impairment on C-arm PBV maps closely matched the pattern of abnormality on MR perfusion maps. On visual comparison between the two techniques, the extent of abnormality indicated PBV to be both cerebral blood flow and cerebral blood volume weighted. CONCLUSION: C-arm FDCT PBV measurements allow an objective assessment of the severity and localisation of cerebral hypoperfusion resulting from vasospasm. The technique has proved feasible and useful in very sick patients after aneurysmal SAH. The promise shown in this early study indicates that it deserves further evaluation both for post-SAH vasospasm and in other relevant clinical settings.

The neuro-behavioral profile in rats after subarachnoid hemorrhage.

Despite significant advancements in the understanding of the pathophysiological mechanisms of subarachnoid hemorrhage (SAH), little is known about the emotional consequences. The primary goal of this study was to describe the locomotor and behavioral patterns in rats following both a single-injection and double-injection model of SAH. In 48 rats, SAH was induced by injecting 0.3 ml of autologous arterial blood into the cisterna magnum (single-hemorrhagic model). In 24 of these rats, post-SAH vasospasm was induced by a repeated injection of blood into the cisterna magnum 24h later (double-hemorrhagic model). In 24 additional rats, 0.3 ml of saline was injected into the cisterna magnum (sham group). Neurological performance was assessed at 24, 48 h, 1, 2 and 3 weeks after SAH. Four behavioral tests were performed for 3 weeks after SAH for the duration of 6 consequent days, in the following order: open field test, sucrose preference test, elevated plus maze test and forced swimming test. Following both, a single and double-hemorrhagic models of SAH, rats were found to have significant behavioral abnormalities on the open field test, sucrose preference test, elevated plus maze test, and forced swimming test. A more prominent disability was found in rats that underwent the double-hemorrhagic model of SAH than rats that underwent the single-hemorrhagic model. Both a single and double injection model of rats SAH are associated with significant behavioral disturbances including locomotor abnormalities, depressive behavior and increased anxiety, even as early as 3 weeks after SAH.

Cerebral vasospasm and health-related quality of life after subarachnoid hemorrhage.

OBJECTIVE: To examine the influence of cerebral vasospasm on health-related quality of life after subarachnoid hemorrhage. An additional objective was to determine how the timing of nimodipine therapy can influence health-related quality of life. METHODS: Patients treated between 1998 and 2008 for nontraumatic subarachnoid hemorrhages were sent a standardized questionnaire for the purposes of documenting their health-related quality of life. Initially the patients were divided into two groups: those with and those without cerebral vasospasm after hemorrhage (radiologically confirmed). They were then differentiated according to four types of treatment options for vasospasm: 1) nimodipine since admission (N = 179); 2) nimodipine since diagnosis of vasospasm (N = 14); 3) no nimodipine/no vasospasm (N = 34); and 4) no nimodipine despite vasospasm (N = 5). Significance was established as P ≤ 0.05. RESULTS: Evaluable questionnaires were returned by 236 patients (68% women, mean age 56.35 ± 12.68 years; 32% men, mean age 54.57 ± 12.20 years). Health-related quality of life generally appeared to be impaired. Yet with the exception of the subscale (1 of 8) of physical role (P = 0.019), there were no differences between patients with and without vasospasm. Variations in the different treatment options revealed significant effects in terms of the component summaries and subscales: physical role and pain, general health, vitality, social functioning, emotional role, mental health, and mental component summary (P ≤ 0.04). CONCLUSIONS: Cerebral vasospasm had little influence on health-related quality of life in our patient population. Health-related quality of life cannot be used as the only argument in favor of treating cerebral vasospasm with nimodipine.

Acute vasospasm following transcallosal resection of a xanthogranulomatous colloid cyst of the 3rd ventricle.

We present the first case of a 57 year old man who developed severe, acute vasospasm following transcallosal resection of an unusual, xanthogranulomatous colloid cyst. The 16 year history of growth of this cyst may have resulted in its unusual pathology, and the subsequent vasospastic reaction to its excision. We discuss the potential pathological relationship between the inflammatory nature of the cyst, chemical meningitis and vasospasm, and what this implies about vasospasm in general. The severe, life-threatening vasospasm affected all four major vessels and required aggressive management by endovascular injection of nimodipine and angioplasty, with good recovery. The case illustrates a previously undescribed sequel of surgery for this condition, demonstrates an effective treatment and offers possible insights into the pathogenesis of vasospasm.