functional-interrogation-adult-hypothalamic-neurogenesis-with-focal

Functional Interrogation of Adult Hypothalamic Neurogenesis with Focal Radiological Inhibition

The functional characterization of adult-born neurons remains a significant challenge. Approaches to inhibit adult neurogenesis via invasive viral ...

Johns Hopkins University School of Medicine

Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis of unknown etiology that affects multiple organs. We report 6 cases of ECD with neurological involvement and neuroradiological abnormalities on brain MRI. A literature review revealed 60 other cases of ECD with neurological involvement. We therefore analyzed 66 ECD patients with neurological involvement. Cerebellar and pyramidal syndromes were the most frequent clinical manifestations (41% and 45% of cases), but seizures, headaches, neuropsychiatric or cognitive troubles, sensory disturbances, cranial nerve paralysis or asymptomatic lesions were also reported. Neurological manifestations were always associated with other organ involvement, especially of bones (at least 86%) and diabetes insipidus (47%). Neurological involvement was responsible for severe functional handicaps in almost all patients and was responsible for the death of 6 of the 66 patients (9%). Neuroradiological findings could be separated into three patterns: the infiltrative pattern (44%), with widespread lesions, nodules or intracerebral masses, the meningeal pattern (37%), with either thickening of the dura mater or meningioma-like tumors, and the composite pattern (19%), with both infiltrative and meningeal lesions.

Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature.

Imatinib mesylate for platelet-derived growth factor receptor-beta-positive Erdheim-Chester histiocytosis.

To assess whether blockade of tumor necrosis factor-alpha (TNF-alpha) influences surgical wound healing in a normal mouse experimental model.

Effect of blockade of tumor necrosis factor-alpha with etanercept on surgical wound healing in SWISS-OF1 mice.

Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis, with noncodified therapeutic management and high mortality. No treatment has yet been shown to improve survival in these patients. We conducted a multicenter prospective observational cohort study to assess whether extraskeletal manifestations and interferon-α treatment would influence survival in a large cohort of ECD patients. To achieve this goal, we thoroughly analyzed the clinical presentation of 53 patients with biopsy-proven ECD, and we performed a survival analysis using Cox proportional hazard model. Fifty-three patients (39 men and 14 women) with biopsy-proven ECD were followed up between November 1981 and November 2010. Forty-six patients (87%) received interferon-α and/or PEGylated interferon-α. Multivariate survival analysis using Cox proportional hazard model revealed that central nervous system involvement was an independent predictor of death (hazard ratio = 2.51; 95% confidence interval, 1.28-5.52; P = .006) in our cohort. Conversely, treatment with interferon-α was identified as an independent predictor of survival (hazard ratio = 0.32; 95% confidence interval, 0.14-0.70; P = .006). Although definitive confirmation would require a randomized controlled trial, these results suggest that interferon-α improves survival in ECD patients. This may be seen as a significant advance, as it is the first time a treatment is shown to improve survival in this multisystemic disease with high mortality.

CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients.

Acute sarcoidosis as parotid fever in rheumatoid arthritis under anti-tumor necrosis factor-alpha therapy.

Patients with syringomyelia may have diverse etiology and experience a variety of symptoms. This report describes two cases of syringomyelia in patients with different profiles, presentations and pathomechanisms.

Syringomyelia: a complication of an underlying pathology.

AA (secondary) amyloidosis is one of the most severe and uncommon complications of several rheumatic disorders and chronic infections such as tuberculosis (TB). Successful treatment depends on the control of the underlying inflammatory process, what can lead to an improvement or a regression in organ dysfunction. If the disorder persists, it has been reported in some cases of AA amyloidosis secondary to rheumatic diseases, that the use of biologic therapy is so far the only opportunity to reduce the development of AA amyloidosis and to reverse established deposits. We report herein a case of a latent TB infection complicated by a life-threatening AA amyloidosis presented as nephrotic syndrome. After an adequate antituberculostatic treatment, AA amyloidosis remained active and Tocilizumab (TCZ) was started with a dramatic resolution of the proteinuria, stabilization of the amyloid deposits and improvement in general condition.

Successful use of tocilizumab in a patient with nephrotic syndrome due to a rapidly progressing AA amyloidosis secondary to latent tuberculosis.

Cellulosimicrobium cellulans has been reported as a rare cause of human pathogenesis. Infections mainly occur in immunocompromised patients and very often are associated with a foreign body. We report the first case of septic arthritis caused by C. cellulans in an immunocompetent patient. Our patient suffered a penetrating palm tree thorn injury to his left knee 8 weeks before admission. Although no foreign objects were found, they were suspected because previous reports suggest a frequent association with this microorganism, and open debridament was performed. Removal of foreign bodies related to this organism must be considered a high-priority treatment in these patients to achieve a complete recovery.

Septic arthritis due to Cellulosimicrobium cellulans.

The term "carcinoma erysipeloides" (CE) designates an uncommon form of cutaneous metastasis. CE is most often associated with carcinoma of the breast. However, there have been reports of CE from carcinoma of the uterus, prostate, lung, ovary, stomach, tonsils, thyroid, pancreas, rectum, parotid glands and melanoma. To our knowledge, CE of laryngeal origin has not been previously reported. We describe a patient diagnosed with human immunodeficiency virus and hepatitis C virus coinfection who developed a supraglottic laryngeal squamous cell carcinoma and erythematous cutaneous lesions. A skin biopsy demonstrated invasion of dilated dermal lymphatics by clusters of atypical squamous cells with polymorphic nuclei and extensive infiltration of the dermis by tumor cells. The histology of the metastatic cells was similar to that of the laryngeal carcinoma.

Carcinoma erysipeloides of laryngeal origin.

Back pain and headaches in a patient with rheumatoid arthritis and Marfan's syndrome.

Recurrent parotid pseudogout.

Behçet's disease (BD) is a chronic, complex multisystem vasculitis of unknown cause characterised for its ability to involve blood vessels of all sizes on both the arterial and venous sides of the circulation. It has been suggested that TNF-alpha plays a main role in the pathogenesis of BD. This hypothesis is supported by the efficacy of TNF-blocking antibodies in these patients, which have been shown to be very powerful in the induction of remission and as maintenance treatment on different BD manifestations, including severe vascular involvement. However, little is known about when and how to stop IFX after long-standing complete remission of these patients to avoid relapses. We describe a case of BD without previous vascular involvement that developed myocardial infarction and severe venous thromboses only four months after discontinuation of infliximab (IFX) after more than three years of complete remission. The patient did not respond to corticosteroids and intravenous cyclophosphamide and only recovered completely after reintroducing IFX.

Life-threatening vasculo-Behçet following discontinuation of infliximab after three years of complete remission.

To assess cardiovascular (CV) risk in psoriatic arthritis (PsA) patients without clinically evident CV disease or classic atherosclerosis risk factors according to the SCORE chart following the EULAR recommendations.

Cardiovascular risk assessment according to a national calibrated score risk index in psoriatic arthritis patients without clinically evident cardiovascular disease or classic atherosclerosis risk factors.

Article	Year
Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature. Journal of neurology\| PubMed ID: 17063320	2006
Imatinib mesylate for platelet-derived growth factor receptor-beta-positive Erdheim-Chester histiocytosis. Blood\| PubMed ID: 18502845	2008
Effect of blockade of tumor necrosis factor-alpha with etanercept on surgical wound healing in SWISS-OF1 mice. The Journal of rheumatology\| PubMed ID: 19723900	2009
CNS involvement and treatment with interferon-α are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients. Blood\| PubMed ID: 21239701	2011
Acute sarcoidosis as parotid fever in rheumatoid arthritis under anti-tumor necrosis factor-alpha therapy. Rheumatology (Oxford, England)\| PubMed ID: 21525138	2011
Syringomyelia: a complication of an underlying pathology. Journal of clinical medicine research\| PubMed ID: 21811530	2010
Successful use of tocilizumab in a patient with nephrotic syndrome due to a rapidly progressing AA amyloidosis secondary to latent tuberculosis. Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis\| PubMed ID: 21992511	2011
Septic arthritis due to Cellulosimicrobium cellulans. Journal of clinical microbiology\| PubMed ID: 21998421	2011
Carcinoma erysipeloides of laryngeal origin. The American Journal of dermatopathology\| PubMed ID: 22668578	2012
Back pain and headaches in a patient with rheumatoid arthritis and Marfan's syndrome. Joint, bone, spine : revue du rhumatisme\| PubMed ID: 22749665	2012
Recurrent parotid pseudogout. Rheumatology (Oxford, England)\| PubMed ID: 23033502	2012
Life-threatening vasculo-Behçet following discontinuation of infliximab after three years of complete remission. Clinical and experimental rheumatology\| PubMed ID: 23739315	2013
Cardiovascular risk assessment according to a national calibrated score risk index in psoriatic arthritis patients without clinically evident cardiovascular disease or classic atherosclerosi... Joint, bone, spine : revue du rhumatisme\| PubMed ID: 23932927	2013

Juan Salvatierra

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