Primary Diffuse Leptomeningeal Melanomatosis: Case Report and Review of the Literature.

BACKGROUND: Primary diffuse leptomeningeal melanomatosis (PDLM) is an extremely rare pathologic condition that can mimic several other neurologic disease states. METHODS: We report a rare case of PDLM without evidence of a primary focus. In addition, we performed a comprehensive review of the literature to describe all previously reported cases of PDLM. RESULTS: In the reported case, making the diagnosis of PDLM was difficult. A brain and frontal dural biopsy was nondiagnostic. Computed tomography of the chest, abdomen, and pelvis did not show any distinct solitary mass. After a positron emission tomography scan was performed that showed lumbar sacral enhancement, lumbar dorsal rootlet biopsy was initiated, which was diagnostic of PDLM. Our literature review found 32 previously reported cases of PDLM. Sixteen cases (48.5%) had a distinct focus or mass discovered on imaging workup. The reported case was the seventeenth reported case of PDLM without a distinct focus or mass found on imaging workup. CONCLUSIONS: PDLM is an extremely rare disease, and diagnosis is difficult because of nonspecific clinical, radiographic, and laboratory findings. In approximately half of cases, no distinct mass is shown on imaging workup, which may further complicate diagnosis. PDLM should be on the differential diagnosis for cases of diffuse dural enhancement. Neurosurgical intervention is often limited to ventriculoperitoneal shunting for increased intracranial pressure and dural and cranial biopsy to obtain diagnosis. If the initial biopsy is nondiagnostic, hypermetabolic activity as seen on positron emission tomography may be helpful to find an alternative biopsy site.

Frameless neuronavigation based only on 3D digital subtraction angiography using surface-based facial registration.

OBJECT: Cerebrovascular lesions can have complicated abnormal anatomy that is not completely characterized by CT or MR angiography. Although 3D rotational angiography provides superior spatial and temporal resolution, catheter angiograms are not easily registered to the patient, limiting the use of these images as a source for neuronavigation. However, 3D digital subtraction angiography (DSA) contains not only vascular anatomy but also facial surface anatomy data. The authors report a novel technique to register 3D DSA images by using only the surface anatomy contained within the data set without having to fuse the DSA image set to other imaging modalities or use fiducial markers. METHODS: A cadaver model was first created to assess the accuracy of neuronavigation based on 3D DSA images registered by facial surface anatomy. A 3D DSA scan was obtained of a formalin-fixed cadaver head, with acquisitions of mask and contrast runs. The right common carotid artery was injected prior to the contrast run with a 45% contrast solution diluted with water-soluble red liquid latex. One week later, the head was registered to a neuronavigation system loaded with the 3D DSA images acquired earlier using facial surface anatomy. A right pterional craniotomy was performed and 10 different vascular landmarks were identified and measured for accuracy using the neuronavigation system. Neuronavigation based only on 3D DSA was then used to guide an open clipping procedure for a patient who presented with a ruptured distal lenticulostriate aneurysm. RESULTS: The accuracy of the measurements for the cadaver model was 0.71 ± 0.25 mm (mean ± SE), which is superior to the 1.8-5 mm reported for neuronavigation. The 3D DSA-based navigation-assisted surgery for the distal lenticulostriate aneurysm aided in localization, resulting in a small craniotomy and minimal brain dissection. CONCLUSIONS: This is the first example of frameless neuronavigation based on 3D catheter angiography registered by only the surface anatomy data contained within the 3D DSA image set. This is an easily applied technique that is beneficial for accurately locating vascular pathological entities and reducing the dissection burden of vascular lesions.

Amygdalohippocampectomy for epilepsy in a patient with prior ipsilateral deep brain stimulator lead placement.

In light of failed medical therapy for movement disorders, the use of deep brain stimulation (DBS) has increased the last two decades. Many complications may transpire; however, to our knowledge, the literature does not mention the phenomena of brain shift from a second unrelated neurosurgical procedure and its theoretical effect on lead displacement and lead function. We present a patient with a left sided DBS for essential tremor and subsequent left amygdalohippocampectomy for temporal lobe epilepsy with minimal radiographic distortion of the DBS lead and without clinical or functional complications. A 47-year-old woman presented with bitemporal epilepsy secondary to a brain injury acquired in childhood in addition to a comorbid bilateral essential tremor, both refractory to medical intervention. A successful left-sided DBS placement was performed with satisfactory resolution of her essential tremor. The patient subsequently developed deterioration of seizure control, becoming refractory to anti-epileptic medications, requiring surgical intervention. A left-sided selective amygdalohippocampectomy and techniques to minimize brain shift were performed without complications. Postoperative imaging suggested minimal distortion of the DBS lead. This did not correspond with reemergence of her essential tremor, implying that the lead maintained functional utility. Brain shift secondary to a craniotomy may cause DBS lead displacement. This phenomenon should be considered when planning operative approaches and can be limited by selective resections. With the growing propensity for placement of DBS leads and the risk of lead displacement, it is important to consider operative approaches to minimize brain shift.

Differentiating ictal panic with low-grade temporal lobe tumors from psychogenic panic attacks.

OBJECTIVE: Indolent low-grade temporal lobe tumors may present with ictal panic that may be difficult to differentiate from psychogenic panic attacks. The current study aims to demonstrate the differences between the two disorders and help physicians generate a diagnostic paradigm. METHOD: This was a retrospective study of 43 patients who underwent a temporal lobectomy between 1981 and 2008 for the treatment of intractable temporal lobe epilepsy secondary to low-grade neoplasms at Rush University Medical Center. A total of 10 patients in this group presented with ictal panic who were previously being treated for psychogenic panic attacks. Medical records were reviewed for age at seizure onset, duration of symptoms, lateralization of the epileptogenic zone, pathological diagnosis, and postsurgical seizure outcome according to the modified Engel classification. RESULTS: Neuropathologic findings of the 10 tumors were pleomorphic xanthoastrocytoma, ganglioglioma, oligodendroglioma, and dysembryoplastic neuroepithelial. The mean age of the patients undergoing surgery was 28 years (range, 15-49). The mean duration of panic symptoms prior to surgery was 9.8 years (range, 3-23). All patients had unprovoked ictal panic. None had symptoms suggestive of a brain tumor, such as signs of increased intracranial pressure or any focal neurologic deficit. In 5 of the patients, other symptoms associated with the ictal panic, including unusual sounds, nausea, automatism, uprising gastric sensation, and déjà vu were identified. Gross total resection of the lesion resulted in improved seizure outcome in all patients undergoing surgery. Patient follow-up was, on average, 7.4 years (range, 2-14) from time of surgery. CONCLUSIONS: Although similar, ictal panic from epilepsy and classic panic attacks are clinically distinguishable entities with different modalities of treatment. A careful history may help differentiate patients with ictal panic from those with psychogenic panic attacks and determine for which patients to obtain neuroimaging studies.

Displacement of a deep brain stimulator lead during placement of an additional ipsilateral lead.

OBJECTIVE: The use of Deep Brain Stimulation (DBS) has been increasing. It follows the premise of neuromodulation in that it is reversible, as compared to previous lesioning procedures. MATERIALS AND METHODS: Complications with DBS are inherently low and range from short-term complications during surgery such as hemorrhage to long-term complications that include lead fractures and infection. Over time, the authors have experienced indications for additional lead placements or change in position of the lead on the ipsilateral side. There is the inherent possibility of direct contact between leads or the microelectrode. This can lead to malpositioning, displacement of a lead placed previously, and malfunctioning. RESULT: We report a case in which a lead placed previously becomes displaced during microelectrode recording on the ipsilateral side. CONCLUSION: This scenario was corrected and had no clinical or functional complication. Placement of an additional ipsilateral DBS lead can be a safe and effective treatment option.

Beneficial actions of the anti-inflammatory dimethyl fumarate in glioblastomas.

BACKGROUND: Dimethylfumarate (DMF), a drug used in the treatment of psoriasis and multiple sclerosis, has been shown to limit the growth of melanoma cells. The ability of DMF to inhibit the Rel protein has been used to explain the antioncogenic properties of this drug. Studies analyzing the effect of DMF in gliomas are limited. Therefore, we investigated the potential antitumor effects of DMF by assessing its effects on proliferation, cell death, and differentiation in gliomas in several glioma models. METHODS: Mouse glioma Gl261, human glioblastoma A172 and human glioblastoma cells from patients were exposed to DMF at therapeutic concentrations (100 µM) and supratherapeutic concentrations (300 µM) and studies to assess proliferation, cellular lysis, and differentiation undertaken. The 5-bromo-2'-deoxyuridine (BRDU) proliferation assay and lactate dehydrogenase LDH cell lysis assay were used. Immunocytochemistry was used to assess differentiation: CD133 (stem cell marker), Nestin (progenitor marker), Sox2 (progenitor marker), ß-tubulin III (neuronal marker), glial fibrillary acidic protein (astrocytic marker), and myelin basic protein (oligodendrocytic marker). We also assessed cellular expression of nuclear factor kappa B (NF-κB) via immunocytochemistry. RESULTS: Proliferation significantly decreased and tumor cell lysis significantly increased in all tumor cell lines after exposure to DMF. The human glioblastoma cells expressed the Neuronal Stem Cell marker CD133, Progenitor Cell markers, Neuronal and Astrocytic Cell Markers in vitro. When exposed to DMF, a drastic decline in CD133 expression was observed in addition to a decrease in the expression of NF-κB. CONCLUSION: DMF appears to have a promising role in the treatment of malignant brain neoplasms. DMF reduced proliferation rate, generated cell lysis, decreased the expression of NF-κB, and restricted the growth of CD133 cells in gliomas. This suggests that DMF may be considered for further antitumor studies, and provide a new treatment modality for brain tumors.

Gender differences in cerebral aneurysm location.

BACKGROUND AND PURPOSE: A limited number of studies consisting predominantly of ruptured aneurysms have looked at differences in anatomical distribution of aneurysms between male and females. Unlike all other causes of stroke, subarachnoid hemorrhages (SAH) occur more often in women and are thought to be a result of both hormonal influences and variation in wall shear stress. This paper retrospectively looks at a cohort of largely unruptured intracranial aneurysms to determine if there exists a gender discrepancy in the anatomic distribution of cerebral aneurysms. METHODS: A retrospective review of consecutive patients with ruptured and unruptured intradural saccular cerebral aneurysms treated endovascularly was performed. RESULTS: Six hundred eighty-two aneurysms were treated. Seventy-two percentage of the patients were women and 27% of patients presented with SAH. Among women, most aneurysms were located along the ICA (54%) while men the ACA (29%, compared to 15% in women), a discrepancy evident in both unruptured and ruptured groups. Females tended to present later in life (59 vs. 55 years), with multiple aneurysms (11 vs. 6% in men), and with SAH (28 vs. 23% in men) - the majority of these ruptured aneurysms were located at the ICA (42%), while men at the ACA (47%). Additionally, the majority (68%) of ruptured ICA aneurysms were PCOM. CONCLUSION: Understanding the natural history of aneurysms is imperative in treating incidentally found aneurysms. Significant differences exist between the genders in relation to aneurysm location, the most pronounced at the ICA and ACA. Previously described hormonal and hemodynamic theories behind cerebral aneurysm pathogenesis seem like plausible reasons to explain these differences.

Surgical treatment of cerebellar metastases.

BACKGROUND: Cerebral metastases are a common neurosurgical finding. Surgery confers several advantages to other therapies, including immediate symptomatic improvement, diagnosis, and relief from corticosteroid dependence. Here we evaluate patients with cerebellar metastases who underwent surgery and compare their findings to those in the literature, and address the benefit of avoiding ventriculo-peritoneal shunting in patients undergoing surgery. METHODS: We performed a retrospective analysis involving 50 patients with cerebellar metastases who underwent surgical resection. Ventriculo-peritoneal shunts were placed in patients necessitating permanent CSF drainage. We evaluated presentation, diagnosis, complications, and outcome. RESULTS: Our review included 21 males and 29 females, 29 to 82 years of age. Primary tumors included lung (48%), breast (14%), GI (14%), endometrial/ovarian (6%), melanoma (6%), sarcoma (4%), lymphoma (4%), laryngeal (2%), and other (2%). Clinical symptoms at presentation commonly were those secondary to elevated intracranial pressure and were the initial complaint in 34% of patients. Preoperatively, 29 patients were noted to have hydrocephalus. Importantly, 76% of these patients were able to avoid placement of a ventriculo-peritoneal shunt following surgery. Only two complications were noted in our series of 50 patients, including a symptomatic pseudomeningocele and a wound infection. No symptomatic postoperative hematoma developed in any surgical case. CONCLUSION: A review of the literature has shown a high complication rate in patients undergoing surgical resection of cerebellar metastases. We have shown that surgical resection of cerebellar metastases is a safe procedure and is effective in the treatment of hydrocephalus in the majority of patients harboring cerebellar lesions.