Fulminant demyelinating neuropathy mimicking cerebral death

Guillain-Barré syndrome can very rarely present with acute quadripares and cranial nerve involvement resembling a locked-in state. We describe a very unusual case of fulminant neuropathy in a child who was previously exposed to vincristine. The clinical picture resembled brain death; however, electrodiagnostic studies led to the diagnosis of a peripheral neuropathy. Serial electrodiagnostic studies and pathologic findings confirmed demyelination. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20: 1595–1597, 1997     

Ability of novice clinicians to interpret high-resolution optical coherence tomography for ocular surface lesions

Objective

To assess the ability of novice clinicians to use a commercially available high-resolution anterior segment optical coherence tomography (HR-OCT) device to diagnose various lesions of the ocular surface and cornea.

Factors Associated with Occurrence of Radiation-induced Optic Neuropathy at “Safe” Radiation Dosage

Background: Radiation-induced optic neuropathy (RION) is a rare, and often visually devastating, complication of radiation therapy (RT) near the anterior visual pathways. Methods: A retrospective case series of patients who developed RION at a tertiary medical center, followed by a case-control study comparing RION cases with matched controls who received RT. Results: Thirteen patients (18 eyes) with RION were identified. Radiation modalities included external beam photon radiation, whole brain radiation, stereotactic radiosurgery, proton beam, and unknown. Most patients received doses below published “safe” thresholds (<55 Gy; <8–10 Gy for stereotactic radiosurgery). There was no statistically significant difference in prevalence of vasculopathic factors between cases and controls; on subgroup analysis in three patients who received surprisingly low radiation doses, smoking (p=0.05) and hypertension (p=0.02) appeared more prevalent. Conclusion: RION can occur at doses below published “safe” thresholds and with different RT modalities. Smoking and hypertension might be risk factors for RION.     

Safety of Prostate Stereotactic Body Radiation Therapy after Transurethral Resection of Prostate (TURP): A Propensity Score Matched Pair Analysis

PurposeTo determine the genitourinary (GU) toxicity outcomes in prostate cancer patients treated with stereotactic body radiation therapy (SBRT) who have undergone a prior transurethral resection of prostate (TURP) and compare it to a similar non-TURP cohort.Materials and MethodsFifty prostate cancer patients who had undergone a single TURP, had a good baseline urinary function, and had been subsequently treated with SBRT were chosen from a prospectively maintained database. These were propensity score matched to a similar non-TURP cohort treated during the same period. Matching was done for diabetes mellitus and volume of radiation therapy. Acute GU and late GU toxicity were scored using the Radiation Therapy Oncology Group (RTOG) criteria. Stricture and incontinence were scored using Common Terminology for Common Adverse Events version 4.0.ResultsMedian follow-up for the entire cohort was 26 months (non-TURP vs TURP, 30 months vs 22 months, P = .34). The median duration between TURP and start of SBRT was 10 months. There was no significant difference between non-TURP versus TURP cohort in terms of RTOG acute GU toxicities grade ≥2 (8% vs 6%, P = .45), RTOG late GU toxicities grade ≥2 (8% vs 12%, P = .10), stricture rates (4% vs 6%, P = .64), and incontinence rates (0% vs 4%, P = .15). The median duration of time to late toxicity was 16 months vs 10 months (P = .12) in non-TURP and TURP cohort, respectively.ConclusionsAlthough modestly increased as compared with non-TURP patients, GU toxicities remains low with SBRT in post-TURP patients. SBRT can be safely performed in carefully selected post-TURP prostate cancer patients.     

Metabolic crosstalk between fatty pancreas and fatty liver: effects on local inflammation and insulin secretion

Aims/hypothesis

Obesity-linked ectopic fat accumulation is associated with the development of type 2 diabetes. Whether pancreatic and liver steatosis impairs insulin secretion is controversial. We examined the crosstalk of human pancreatic fat cells with islets and the role of diabetogenic factors, i.e. palmitate and fetuin-A, a hepatokine released from fatty liver.

Methods

Human pancreatic resections were immunohistochemically stained for insulin, glucagon, somatostatin and the macrophage/monocyte marker CD68. Pancreatic adipocytes were identified by Oil Red O and adiponectin staining. Primary pancreatic pre-adipocytes and differentiated adipocytes were co-cultured with human islets isolated from organ donors and the metabolic crosstalk between fatty liver and fatty pancreas was mimicked by the addition of palmitate and fetuin-A. Insulin secretion was evaluated by ELISA and RIA. Cytokine expression and secretion were assessed by RT-PCR and multiplex assay, respectively. Subcellular distribution of proteins was examined by confocal microscopy and protein phosphorylation by western blotting.

Results

In human pancreatic parenchyma, highly differentiated adipocytes were detected in the proximity of islets with normal architecture and hormone distribution. Infiltration of adipocytes was associated with an increased number of CD68-positive cells within islets. In isolated primary pancreatic pre-adipocytes and differentiated adipocytes, palmitate and fetuin-A induced IL6, CXCL8 and CCL2 mRNA expression. Cytokine production was toll-like receptor 4 (TLR4)-dependent and further accentuated in pre-adipocytes when co-cultured with islets. In islets, IL6 and CXCL8 mRNA levels were also increased by fetuin-A and palmitate. Only in macrophages within the isolated islets, palmitate and fetuin-A stimulated the production of the cytotoxic cytokine IL-1β. Palmitate, but not fetuin-A, exerted pro-apoptotic effects in islet cells. Instead, fetuin-A impaired glucose-induced insulin secretion in a TLR4-independent, but c-Jun N-terminal kinase- and Ca²⁺-dependent, manner.

Conclusions/interpretation

These results provide the first evidence that fetuin-A-mediated metabolic crosstalk of fatty liver with islets may contribute to obesity-linked glucose blindness of beta cells, while fatty pancreas may exacerbate local inflammation.

     

Epithelioid angiomyolipoma of kidney with atypical nuclear features and intranuclear inclusions on cytology

Described herein are the cytological findings of epithelioid angiomyolipoma (EAML) of the kidney with atypical nuclear features mistaken for renal cell carcinoma (RCC) in a 61‐year‐old male patient. Aspirates from this large renal mass were cellular and showed epithelioid cell clusters with focally crowded nuclei showing moderate anisonucleosis, small nucleoli, and prominent eosinophilic intranuclear inclusions. Failure to recognize the scanty adipose tissue component and preponderance of epithelioid cells with nuclear pleomorphism lead to a diagnosis of RCC on cytology. On histology, the tumor was essentially composed of epithelioid and spindle cells that showed the typical immunoprofile of an angiomyolipoma and only occasional foci of typical AML were seen. The hilar lymph node was involved in contiguity. However, in view of lack of obvious features of malignancy, the tumor was labeled as EAML with atypical features. Immunocytochemistry on the destained cytology aspirates revealed strong smooth muscle actin staining of all cells. To conclude, EAML can mimic a RCC. In such instances, lack of arborizing vasculature, absence of cytoplasmic fatty vacoulation, crowded nuclei with intranuclear inclusions, and lack of prominent nucleoli along with typical immunophenotype of EAML may assist in the cytology diagnosis. Diagn. Cytopathol. 2011;39:278–282. © 2010 Wiley‐Liss, Inc.     

Brain MRI Lesion Load at 1.5T and 3T versus Clinical Status in Multiple Sclerosis

ABSTRACT

BACKGROUND/PURPOSE

To assess correlation between brain lesions and clinical status with 1.5T and 3T magnetic resonance imaging (MRI).

METHODS

Brain MRI fluid‐attenuated inversion‐recovery (FLAIR) sequences were performed in 32 multiple sclerosis (MS) patients. Expanded Disability Status Scale (EDSS) score (mean ± standard deviation) was 2 ± 2.0 (range 0‐8), disease duration 9.3 ± 8.0 (range .8‐29) years.

RESULTS

FLAIR lesion volume (FLLV) at 3T was higher than at 1.5T (P= .01). Correlation between 1.5T FLLV and EDSS score was poor, while 3T FLLV correlated moderately and significantly (r_s= .39, P= .03). When controlling for age and depression, correlations between FLLV and cognitive measures were significant at 1.5T for the Judgment of Line Orientation test (JLO) (r_s=−.44, P= .05), the Symbol Digit Modalities Test (SDMT) (r_s=−.49, P= .02), and the California Verbal Learning Test Delayed Free Recall (CVLT DR) (r_s=−.44, P= .04). Correlations at 3T were also significant for these tests, but of greater magnitude: JLO (r_s=−.70, P= .0005), SDMT (r_s=−.73, P= .0001), CVLT DR (r_s=−.061, P= .003). Additional significant correlations obtained only at 3T included the 2 second‐paced auditory serial addition test (r_s=−.55, P= .01), the Brief Visuospatial Memory Test‐Delayed Free Recall (r_s=−.56, P= .007), and the California Verbal Learning Test Total Recall (r_s=−.42, P= .05).

CONCLUSION

MRI at 3T may boost sensitivity and improve validity in MS brain lesion assessment.