Dr. Ahmed gave us a great noon conference lecture last week on IBD. If you missed it or just want a refresher, you can hear her talk on the resident website or view her slides below.
(Note to email subscribers, the slides are only visible from the original post. To view, click on the link to go to the full website)
- Excluding headaches, neurological complications of Behçet syndrome (neuro-Behçet syndrome) occur in less than 12% of cases, often a few years after the onset of the other systemic features.
Clinical Manifestations and Diagnosis
- In parenchymal disease, meningoencephalitis occurs, with a mixed inflammatory cell infiltrate leading to necrosis and apoptotic neuronal loss. Inflammatory infiltration, rather than fibrinoid necrosis, is seen around small vessels.
- The brainstem and mid-brain are the most commonly affected areas, but spinal cord lesions and cerebral involvement may also occur and, occasionally, neuro-Behçet syndrome presents as a pseudotumour cerebri.
- Brainstem involvement usually presents subacutely, with headache, cranial neuropathies or cerebellar or corticospinal tract dysfunction.
- Sensorineural hearing loss can occur, resulting in sudden deafness, balance disturbances and dizziness.
- The characteristic MRI lesion in parenchymal neuro-Behçet syndrome is a unilateral upper brainstem lesion extending into the thalamus and basal ganglia.
- Analysis of cerebrospinal fluid shows a neutrophilic (in early disease) or lymphocytic (in late disease) pleocytosis, but usually no oligoclonal bands.
- Neurovascular disease accounts for approximately 20% of cases of neuro-Behçet syndrome and symptoms include dural sinus thrombosis, intracranial aneurysm and extracranial aneurysm and/or dissection.
- The clinical findings are usually limited to those of intracranial hypertension (that is, headache, vomiting, altered levels of consciousness and papilloedema).
- Intracranial hypertension may be present without MRI abnormalities and should be managed in the same way as idiopathic benign intracranial hypertension.
- Aneurysms of the cerebral, vertebral and carotid arteries can also occur.
- Headaches are often under-treated. An effort should be made to classify the type of headache and, in the case of migraine, agents such as pizotifen and βblockers should be offered.
- Parenchymal disease:
- Initial Management: treated with high-dose steroids in the first instance, along with initiation of a DMARD, usually azathioprine.
- Methotrexate, mycophenolate, cyclophosphamide, tacrolimus and IFN-α are probably effective, although evidence is lacking.
- Cyclosporin is potentially neurotoxic and should not be used for patients with a history of CNS disease.
- The alternative therapeutic option for severe and aggressive disease is the early use of a biologic agent (i.e. anti-TNF therapy, like infliximab)
- Neurovascular disease:
- Cerebral vascular thrombosis and aneurysms also require aggressive immunosuppression.
- Anti-coagulation for venous thrombosis needs to be assessed on a case-by-case basis.
Ambrose, N. L. & Haskard, D. O. Nat. Rev. Rheumatol. 9, 79–89 (2013); published online 25 September 2012; doi:10.1038/nrrheum.2012.156
Continuing the recent trend of posting entries related to GI comes an article from The Washington Post about getting paid for donating your stool to be used for fecal transplants. The article focuses on the organization, OpenBiome, which collects these stool samples, processes, and delivers them all over the country. But they don’t just take any sample. It’s a rigorous screening process as one of the co-founders jokes it’s easier to get into M.I.T than to have your stool accepted. They want stool samples from very healthy individuals. Recent research has shown remarkable results in treating C. diff infections with donor feces. Click on the table below to read more about this!