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Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumor-like lesion with unknown pathogenesis. It is likely under-reported due to diagnostic challenges including the nonspecific radiographic features, lack of diagnostic markers, and often asymptomatic nature of the lesions.
We performed detailed examination of 11 CAPNON specimens diagnosed by histopathology, with the help of electron microscopy and immunohistochemistry.
Electron microscopy revealed the presence of fibrillary materials consistent with neurofilaments. In addition to some entrapped axons at the periphery of CAPNONs, we discovered that all specimens stained positive for neurofilament-light (NF-L) within the granular amorphous cores, but not neurofilament-phosphorylated (NF-p). CAPNONs also showed variable infiltration of CD8+ T-cells and a decreased ratio of CD4/CD8+ T-cells, suggesting an immune-mediated process in the pathogenesis of CAPNON.
NF-L and CD4/CD8 immunostains may serve as diagnostic markers for CAPNON and shed light on its pathogenesis.
Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare tumefactive lesion. CAPNONs can mimic calcified meningiomas at the skull base.
Here, we report two cases of CAPNON and present a systematic review of the literature on skull base CAPNONs, to compare CAPNONs with calcified meningiomas.
Case 1: A 57-year-old man presented with right-sided lower cranial neuropathies and gait ataxia. He underwent a subtotal resection of a right cerebellopontine angle lesion, with significant improvement of his gait ataxia. However, his cranial neuropathies persisted. Pathological examination of the lesion was diagnostic of CAPNON, with the entrapped nerve fibers identified at the periphery of the lesion, correlating with the patient’s cranial neuropathy. Case 2: A 70-year-old man presented with progressive headache, gait difficulty, and cognitive impairment. He underwent a frontotemporal craniotomy for a near-total resection of his right basal frontal CAPNON. He remained neurologically stable 7 years after the initial resection without evidence of disease recurrence. We analyzed 24 reported CAPNONs at the skull base in our systematic review of the literature. Cranial neuropathies were present in 11 (45.8%) patients. Outcomes regarding cranial neuropathies were documented in six patients: two had sacrifice of the nerve function with surgical approaches and four had persistent cranial neuropathies.
While CAPNON can radiologically and grossly mimic calcified meningiomas, they are two distinctly different pathologies. CAPNONs located at the skull base are commonly associated with cranial neuropathies, which may be difficult to reverse despite surgical intervention.
Decompressive hemicraniectomy (DH) has been shown to reduce mortality in patients with malignant middle cerebral artery (MCA) territory infarction. However, many patients survive with moderate-to-severe disability and controversy exists as to whether this should be considered good outcome. To answer this question, we assessed the quality of life (QoL) of patients after DH for malignant MCA territory infarction in our milieu.
The outcome of all patients undergoing DH for malignant MCAterritory infarction between 2001 and 2009 was assessed using retrospective chart analysis and telephone follow-up in survivors. Functional outcome was determined using Glasgow outcome scale, modifed Rankin scale (mRS), and Barthel index (BI). The stroke impact scale was used to assess QoL.
There were 14 patients, 6 men and 8 women, with a mean age of 44 years (range 27-57). All patients had reduced level of consciousness preoperatively. Five had dominant-hemisphere stroke. Median time to surgery was 45 hours (range 1- 96). Two patients died and one was lost to follow-up. Of 11 survivors, 7 (63.6%) had a favorable functional outcome (mRS<4). No patient was in persistent vegetative state. Despite impaired QoL, particularly in physical domains, the majority of interviewed patients and caregivers (7 of 8), including those with dominant-hemisphere stroke, were satisfied after a median follow-up of 18 months (range 6-43).
Most patients report satisfactory QoL despite significant disability even in the face of moderate-to-severe disability and dominant-hemsiphere stroke. Dominant-hemisphere malignant MCA territory infarction should not be considered a contraindication to DH.
To present a new semiological description of unruptured middle cerebral artery (MCA) aneurysms.
We present a series of three MCA aneurysms presenting with progressive or paroxystic somatosensory symptoms in combination with visceral, motor, language or autonomic symptoms.
A surgical approach was proposed for two aneurysms, and both patients experienced complete resolution of their symptoms. The third aneurysm was successfully excluded by endovascular coiling but the symptoms persisted.
To our knowledge this is the first description of unruptured aneurysms presenting with insular-related symptoms.
Patients harboring an unruptured cerebral aneurysm may present with ischemic events. The goal of this study is to assess the clinical and radiological characteristics and the outcome following treatment of these patients.
The study population included 463 patients with unruptured cerebral aneurysms treated between January 2000 and November 2006. Patients with aneurysms manifesting with ischemic events were included. Outcome was assessed 12 months following aneurysm treatment using the modified Rankin scale.
Eleven patients were included in this series. An acute ischemic lesion in the symptomatic territory was demonstrated in six patients. The aneurysms were located on the internal carotid artery (n=4), middle cerebral artery (n=4), superior cerebellar artery (n=2) and basilar artery (n=1). They measured 10mm or less (n=7); 11-20mm (n=2); more than 21mm (n=2). Five aneurysms were partially thrombosed on imaging. Five patients were referred for coiling. Of these, one patient had an unsuccessful coiling attempt, one had a residual neck, and three presented an aneurysm recurrence. Six patients were treated surgically. Symptomatic thromboembolism occurred after surgery in three patients. Complete aneurysm exclusion was documented in five of six operated patients. Nine of the ten treated patients had a favorable outcome.
Even though aneurysms presenting with ischemic events are often small and located on the anterior circulation, in this series the risk of thromboembolic events following aneurysm treatment is noteworthy. This information is relevant given the possible benign natural history in terms of stroke and risk of bleeding for some of these aneurysms.
The timing of aneurysmal surgery for patients presenting within the period at risk for vasospasm (VS) is controversial. The goal of this study is to review our experience of surgically treated patients in the presence of angiographic VS.
Materials and Methods:
From 1990-2004, 894 consecutive patients presented with an aneurysmal subarachnoid hemorrhage (SAH) and were treated with a policy of early surgery. We retrospectively analyzed the patients that had pre-operative angiographic VS. In this study, symptomatic VS was diagnosed when a decreased level of consciousness and/or focal deficit occurred after SAH in the presence of angiographic VS without confounding factors. Functional outcome was assessed three months after SAH using the Glasgow Outcome Scale.
Of the 40 patients studied, 62.5% were in good clinical grade Hunt & Hess (H&H 1-2) on admission; 25%, intermediate grade (H&H 3); 12.5%, poor grade (H&H 4-5). Surgery was performed 24 hours or less after initial angiography in 87.5% of patients and less than 48 hours in 97.5%. Pre-operative symptomatic VS was diagnosed in 25%. Postoperatively, angiographic VS was documented in 87.2%. Of the 30% of patients that presented post-operative symptomatic VS, 66.7% also demonstrated pre-operative symptomatic VS. The functional outcome was favorable in 92.5% of the studied patients. Two deaths occurred in patients presenting pre-operative early radiological and symptomatic VS.
Aneurysmal surgery, especially between 3-12 days following SAH, in the presence of asymptomatic pre-operative angiographic VS can be associated with a good outcome. Early surgery is not contra-indicated and might enable optimal treatment of VS.
Pulmonary edema (PE) can occur in the early or late period following subarachnoid hemorrhage (SAH). The incidence of each type of PE is unknown and the association with ventricular dysfunction, both systolic and diastolic, has not been described.
Retrospective chart review of 178 consecutive patients with SAH surgically treated over a three-year period. Patients with pulmonary edema diagnosed by a radiologist were included. Early onset SAH was defined as occurring within 12 hours. Cardiac function at the time of the PE was analyzed using hemodynamic and echocardiographic criteria of systolic and diastolic dysfunction. Pulmonary edema was observed in 42 patients (28.8%) and was more often delayed (89.4%). Evidence of cardiac involvement during PE varied between 40 to 100%.
Results and conclusions:
Pulmonary edema occurs in 28.8% of patients after SAH, and is most commonly delayed. Cardiac dysfunction, both systolic and diastolic, is commonly observed during SAH and could contribute to the genesis of PE after SAH.
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