Lymphoma, a great imitator in neurology.

The title "great imitator" refers to conditions which can cause varied manifestations and mimic many diseases. Lymphoma is worthy of this title. We describe three cases of lymphoma in which lymphoma mimicked other diseases causing neurological dysfunction, specifically sarcoidosis, vasculitis and infection respectively. Case 1 was a 66-year-old man with subacute progressive diplopia and gait disturbance and investigations revealing a supratentorial para-falcine soft tissue lesion, mid-thoracic cord enhancement and right axillary mass and an elevated serum ACE. Right axillary mass core biopsy was diagnostic of Burkitt lymphoma. Case 2 was a 50-year-old man with several weeks of constitutional symptoms and development of lower limb weakness and numbness, urinary retention and confusion while in hospital. MRI brain demonstrated multi-territory cerebral infarcts. Intravascular lymphoma was diagnosed on random skin biopsy. Case 3 was a 65-year-old man with several weeks of headache and diplopia on a background of previously treated Burkitt lymphoma. CSF analysis showed a lymphocytic pleocytosis and markedly low glucose with cytologic analysis negative for malignancy. Investigations for an infective cause were negative. FDG-PET demonstrated marked, disseminated spinal and cranial leptomeningeal disease and a multi-focal, intra-dural relapse of Burkitt lymphoma was diagnosed. The varied manifestations in our cases demonstrate the ability for lymphoma to mimic infective, inflammatory, granulomatous (including sarcoidosis) and neoplastic aetiologies. An elevated serum ACE appears insufficiently diagnostic to confirm sarcoidosis and tissue for histological examination should be sought whenever possible. When the diagnosis is uncertain, the possibility of this great imitator should be considered, especially for multi-focal disease.

Central Conditions Mimicking Benign Paroxysmal Positional Vertigo: A Case Series.

BACKGROUND AND PURPOSE: Benign paroxysmal positional vertigo (BPPV) is the most common cause of positional vertigo. The term "benign" is consistent with a peripheral vestibular disorder that does not carry the potentially sinister sequelae of a central nervous system (CNS) cause. However, in 12% to 20% of cases, positional vertigo may be attributed to CNS pathology, including tumors of the cerebellum. CASE DESCRIPTION: Here, we present a series of 3 cases in which positional vertigo and nystagmus were the only presenting features in 2 cases of cerebellar tumor and 1 case of obstructive hydrocephalus. INTERVENTION: All patients underwent surgical intervention for removal of posterior fossa tumors or posterior fossa decompression for obstructive hydrocephalus. Following surgery, all 3 patients underwent a period of vestibular rehabilitation for postoperative motion sensitivity and balance impairment. OUTCOMES: Despite the continuing presence of central positioning nystagmus, all 3 patients recovered well, putatively with the aid of vestibular rehabilitation. DISCUSSION: The presence of central positioning nystagmus may be the sole presenting feature of serious neurological conditions such as posterior fossa tumor. It is recommended that a diagnosis of BPPV can only be made if Dix-Hallpike or supine roll maneuver elicits nystagmus that is consistent with BPPV. Any features of the nystagmus, which are not consistent with BPPV, should raise suspicion of central pathology, and warrant further investigation.Video Abstract available for more insights from the authors (see Video Abstract, Supplemental Digital Content 1, available at: http://links.lww.com/JNPT/A265).

Creation of a Large Adipose Tissue Construct in Humans Using a Tissue-engineering Chamber: A Step Forward in the Clinical Application of Soft Tissue Engineering.

Tissue engineering is currently exploring new and exciting avenues for the repair of soft tissue and organ defects. Adipose tissue engineering using the tissue engineering chamber (TEC) model has yielded promising results in animals; however, to date, there have been no reports on the use of this device in humans. Five female post mastectomy patients ranging from 35 to 49years old were recruited and a pedicled thoracodorsal artery perforator fat flap ranging from 6 to 50ml was harvested, transposed onto the chest wall and covered by an acrylic perforated dome-shaped chamber ranging from 140 to 350cm(3). Magnetic resonance evaluation was performed at three and six months after chamber implantation. Chambers were removed at six months and samples were obtained for histological analysis. In one patient, newly formed tissue to a volume of 210ml was generated inside the chamber. One patient was unable to complete the trial and the other three failed to develop significant enlargement of the original fat flap, which, at the time of chamber explantation, was encased in a thick fibrous capsule. Our study provides evidence that generation of large well-vascularized tissue engineered constructs using the TEC is feasible in humans.

Intraventricular CNS lesions: A pictorial essay.

Intraventricular lesions of the central nervous system (CNS) can present a diagnostic challenge due to a range of differential diagnoses and radiological appearances. Both CT and MRI imaging findings, in combination with location and patient's age, can help limit the differentials. This pictorial essay presents the salient radiological features, location and demographics of the more common intraventricular lesions of the brain.

Large anterior temporal Virchow-Robin spaces: unique MR imaging features.

INTRODUCTION: Large Virchow-Robin (VR) spaces may mimic cystic tumor. The anterior temporal subcortical white matter is a recently described preferential location, with only 18 reported cases. Our aim was to identify unique MR features that could increase prospective diagnostic confidence. METHODS: Thirty-nine cases were identified between November 2003 and February 2014. Demographic, clinical data and the initial radiological report were retrospectively reviewed. Two neuroradiologists reviewed all MR imaging; a neuropathologist reviewed histological data. RESULTS: Median age was 58 years (range 24-86 years); the majority (69 %) was female. There were no clinical symptoms that could be directly referable to the lesion. Two thirds were considered to be VR spaces on the initial radiological report. Mean maximal size was 9 mm (range 5-17 mm); majority (79 %) had perilesional T2 or fluid-attenuated inversion recovery (FLAIR) hyperintensity. The following were identified as potential unique MR features: focal cortical distortion by an adjacent branch of the middle cerebral artery (92 %), smaller adjacent VR spaces (26 %), and a contiguous cerebrospinal fluid (CSF) intensity tract (21 %). Surgery was performed in three asymptomatic patients; histopathology confirmed VR spaces. Unique MR features were retrospectively identified in all three patients. CONCLUSION: Large anterior temporal lobe VR spaces commonly demonstrate perilesional T2 or FLAIR signal and can be misdiagnosed as cystic tumor. Potential unique MR features that could increase prospective diagnostic confidence include focal cortical distortion by an adjacent branch of the middle cerebral artery, smaller adjacent VR spaces, and a contiguous CSF intensity tract.

Anterior visual pathway cavernous malformations.

Anterior visual pathway cavernous malformations (CM) are rare diagnoses with poorly-defined natural history and management. A systematic review of all reports of anterior visual pathway CM was performed to identify all English-language articles with histopathologically-proven anterior visual pathway CM published from 1950 to December 2013. Patient demographics, presenting symptoms, CM location, treatment modality and clinical outcome were recorded and analyzed. The case of a 60-year-old woman from our institution with acute-on-chronic visual disturbance secondary to visual pathway CM is presented. Including the current patient, 70 cases of anterior visual pathway CM have been published to our knowledge. The average patient age is 34.8 ± standard deviation of 14.2 years, with a female preponderance (n = 37, 52.9%). The majority of patients had an acute (n = 44; 62.9%; 95% confidence interval [CI] 0.51-0.73) onset of symptoms. In at least 55.6% (n = 40) of patients, the cause of visual disturbance was initially misdiagnosed. The majority (91.4%; n = 64) of patients underwent craniotomy, with complete resection and subtotal resection achieved in 53.1% (n = 34; 95%CI 0.41-0.65) and 17.2% (n = 11; 95%CI 0.10-0.28) of all surgical patients, respectively. Comparing surgically managed patients, complete resection improved visual deficits in 59.0% (n = 20; 95%CI 0.42-0.75), while subtotal resection improved visual deficits in 50.0% (n = 5; 95%CI 0.24-0.76; p = 0.62). CM is an important differential diagnosis for suprasellar lesions presenting with visual disturbance. A high index of suspicion is required in its diagnosis. Expeditious operative management is recommended to improve clinical outcomes.

Magnetic resonance imaging of intramedullary spinal cord lesions: a pictorial review.

Magnetic resonance imaging (MRI) is the modality of choice for the investigation of intramedullary lesions of the spinal cord. A wide variety of conditions may result in similar imaging findings on MRI, and it is essential that the reporting radiologist have a detailed understanding of spinal cord anatomy, the pertinent imaging features of specific intramedullary lesions and the typical clinical presentation of those conditions to aid clinicians to make a prompt diagnosis. This pictorial essay discusses the clinical features and MRI appearance of a number of intramedullary conditions, which can be broadly categorised as congenital, demyelinating, vascular, neoplastic or infectious, and highlights their differentiating features.

The Mediterranean diet improves hepatic steatosis and insulin sensitivity in individuals with non-alcoholic fatty liver disease.

BACKGROUND & AIMS: Non-alcoholic fatty liver disease (NAFLD) affects up to 30% of the population and signifies increased risk of liver fibrosis and cirrhosis, type 2 diabetes, and cardiovascular disease. Therapies are limited. Weight loss is of benefit but is difficult to maintain. We aimed at examining the effect of the Mediterranean diet (MD), a diet high in monounsaturated fatty acids, on steatosis and insulin sensitivity, using gold standard techniques. METHODS: Twelve non-diabetic subjects (6 Females/6 Males) with biopsy-proven NAFLD were recruited for a randomised, cross-over 6-week dietary intervention study. All subjects undertook both the MD and a control diet, a low fat-high carbohydrate diet (LF/HCD), in random order with a 6-week wash-out period in- between. Insulin sensitivity was determined with a 3-h hyperinsulinemic-euglycemic clamp study and hepatic steatosis was assessed with localized magnetic resonance (1)H spectroscopy ((1)H-MRS). RESULTS: At baseline, subjects were abdominally obese with elevated fasting concentrations of glucose, insulin, triglycerides, ALT, and GGT. Insulin sensitivity at baseline was low (M=2.7 ± 1.0 mg/kg/min(-1)). Mean weight loss was not different between the two diets (p=0.22). There was a significant relative reduction in hepatic steatosis after the MD compared with the LF/HCD: 39 ± 4% versus 7 ± 3%, as measured by (1)H-MRS (p=0.012). Insulin sensitivity improved with the MD, whereas after the LF/HCD there was no change (p=0.03 between diets). CONCLUSIONS: Even without weight loss, MD reduces liver steatosis and improves insulin sensitivity in an insulin-resistant population with NAFLD, compared to current dietary advice. This diet should be further investigated in subjects with NAFLD.

Primary central nervous system lymphoma in immunocompetent patients: a retrospective review of MRI features.

INTRODUCTION: To define the features of primary central nervous system lymphoma (PCNSL) on MRI in immunocompetent patients. METHODS: A retrospective review of the authors' institutional database was performed to identify histologically proven cases of PCNSL. Images were retrieved and reviewed with respect to location, lesion number, size, signal intensity, enhancement characteristics, oedema and necrosis. RESULTS: Thirty-one cases of histologically proven PCNSL had available imaging. One patient was excluded due to immunosuppression. Of the 30 remaining cases, the average age was 65.5 years, and males and females were equally represented. A total of 68 lesions (average of 2.5 per patient) were identified. With diffusion-weighted imaging, all but two had restricted diffusion (40.3% mild and 55.6% marked) and all but one had enhancement (51.5% homogeneous, 42.6% heterogeneous and ring 4.4%). Most lesions were isointense to grey matter (75.8% on T2-weighted image (WI) and 82.5% on T1-WI). Oedema was mild in 43.4% and marked in 55.2%. Necrosis was seen in only five lesions (7.4%). On a per patient basis, 50% had bilateral lesions and 96.7% had lesions contacting a cerebrospinal fluid (CSF) surface. 16.7% of patients had posterior fossa involvement and 30% had lesions in the basal ganglia or thalami. CONCLUSION: The vast majority of cases of PCNSL in immunocompetent patients have lesions contacting a CSF surface, enhancement and restricted diffusion with no necrosis. These features should alert radiologists to the diagnosis of PCNSL.

Tissue-engineered breast reconstruction: bridging the gap toward large-volume tissue engineering in humans.

BACKGROUND: Use of autologous tissue is ideal in breast reconstruction; however, insufficient donor tissue and surgical and donor-site morbidity all limit its use. Tissue engineering could provide replacement tissue, but only if vascularization of large tissue volumes is achievable. The authors sought to upscale their small-animal adipose tissue-engineering models to produce large volumes of tissue in a large animal (i.e., pig). METHODS: Bilateral large-volume (78.5 ml) chambers were inserted subcutaneously in the groin enclosing a fat flap (5 ml) based on the superficial circumflex iliac pedicle for 6 (n = 4), 12 (n = 1), and 22 weeks (n = 2). Right chambers included a poly(L-lactide-co-glycolide) sponge. Other pedicle configurations, including a vascular pedicle alone (n = 2) or in combination with muscle (n = 2) or a free fat graft (n = 2), were investigated in preliminary studies. Serial assessment of tissue growth and vascularization by magnetic resonance imaging was undertaken during growth and correlated with quantitative histomorphometry at chamber removal. RESULTS: All chambers filled with new tissue by 6 weeks, vascularized by the arteriovenous pedicle. In the fat flap chambers, the initial 5 ml of fat expanded to 25.9 ± 2.4, 39.4 ± 3.9, and 56.5 ml (by magnetic resonance imaging) at 6, 12, and 22 weeks, respectively. Adipose tissue volume was maintained up to 22 weeks after chamber removal (n = 2), including one where the specimen was transferred on its pedicle to an adjacent submammary pocket. CONCLUSION: The first clinically relevant volumes of tissue for in situ and remote breast reconstruction have been formed with implications for scaling of existing tissue-engineering models into human trials.

Long-term stability of adipose tissue generated from a vascularized pedicled fat flap inside a chamber.

BACKGROUND: Numerous studies demonstrate the generation and short-term survival of adipose tissue; however, long-term persistence remains elusive. This study evaluates long-term survival and transferability of de novo adipose constructs based on a ligated vascular pedicle and tissue engineering chamber combination. METHODS: Defined adipose tissue flaps were implanted into rats in either intact or perforated domed chambers. In half of the groups, the chambers were removed after 10 weeks and the constructs transferred on their vascular pedicle to a new site, where they were observed for a further 10 weeks. In the remaining groups, the tissue construct was observed for 20 weeks inside the chamber. Tissue volume was assessed using magnetic resonance imaging and histologic measures, and constructs were assessed for stability and necrosis. Sections were assessed histologically and for proliferation using Ki-67. RESULTS: At 20 weeks, volume analysis revealed an increase in adipose volume from 0.04 ± 0.001 ml at the time of insertion into the chambers to 0.27 ± 0.004 ml in the closed and 0.44 ± 0.014 ml in the perforated chambers. There was an additional increase of approximately 10 to 15 percent in tissue volume in flaps that remained in chambers for 20 weeks, whereas the volume of the transferred tissue not in chambers remained unaltered. Histomorphometric assessment of the tissues documented no signs of hypertrophy, fat necrosis, or atypical changes of the newly generated tissue. CONCLUSION: This study presents a promising new method of generating significant amounts of mature, vascularized, stable, and transferable adipose tissue for permanent autologous soft-tissue replacement.

Brainstem distortion from postoperative cerebellar herniation through a dural and bony defect.

We report a patient with a cerebellar encephalocele following excision of a cerebellar metastasis. This is a life-threatening condition that may be prevented with adequate dural and bony closure.

Unusual case of skull metastasis from hepatocellular carcinoma.

Hepatocellular carcinoma with a skull metastasis is a rare clinical entity especially in Western countries. The authors of the present article report a case of solitary skull metastasis from hepatocellular carcinoma in a 75-year-old lady who had no prior history of the primary disease or liver dysfunction. The clinicopathological and radiological features are reviewed and discussed.

Subfrontal schwannoma: a case report and literature review.

Subfrontal schwannomas are rare and usually misdiagnosed preoperatively. We present the third reported case of a schwannoma arising from the olfactory nerve. The neuroradiological and pathological features of the case, as well as the origin of the tumour are discussed.

Meningoencephalomyelitis with vasculitis due to varicella zoster virus: a case report and review of the literature.

Varicella zoster virus (VZV) encephalitis is associated with large or small vessel vasculopathy. We report the case of a 67-year-old woman with a history of non-Hodgkin's lymphoma and cancers of the breast and colon, who presented with a zosteriform rash and Brown-Sequard syndrome. Despite 10 days therapy with intravenous acyclovir, meningoencephalitis developed and the patient died 15 days after onset of neurological symptoms. Autopsy showed meningoencephalomyelitis with necrotising vasculitis of leptomeningeal vessels, which is a rare complication of VZV, and we review the literature of the nine similar published cases. Polymerase chain reaction of cerebrospinal fluid for VZV was negative 6 days after onset of neurological symptoms, but became positive by day 10. Only one multinucleated giant cell with intranuclear Cowdry type A inclusions was seen within an endothelial cell in a leptomeningeal vessel involved by vasculitis.