2022 Clinical problem solving pre conference asynchronous learning around Neuro behcet's

Link to report here

Link to conference here

January 4th and 5th 2022

Professor and HOD of Medicine (Narketpally) introducing Rheumatology expert (Prof Thomas Jefferson university Philadelphia) and Medicine PG (Narketpally) for discussion over suspected case of Neuro Behcet's disease in a patient of our hospital. 

January 8th 2022

Shared case was reviewed by Rheumatology expert and her opinion was shared with the Medicine PG stating that the given case may not be a case of Neuro Behcet's since the classical presentation of mucocutaneous lesions and ocular manifestations were absent. 

Questions raised by the Rheumatology expert were as under:

Q1. What differentials can be made on the basis of infarcts seen bilaterally in the capsuloganglionic? 

Q2. How can the differences between the findings in the MRI and CT scan be explained

Q3. While infarcts have been observed why was the treatment of choice dual antiplatelet therapy and statins? 

Q4. The cause for acute infarcts is not apparent so how was CVA drawn as a conclusion? 

Q5. Lesion in which region resulted in diplopia and was the diplopia observed only in one direction? 

Q6. On what basis was the differential of Neurosarcoidosis made? 

Further, valuable literature was shared pertaining to the syndrome which is as under:

1. Behçet syndrome: a contemporary

view by Yazici (paid article) here

2. An uncommon disease included commonly in the differential diagnosis of neurological diseases: Neuro-Behçet’s syndrome by Ugur Uygunoglu (paid article) here

3. Focus on neuro‑Behçet’s disease:A review by Paola Caruso here

Furthermore, The Rheumatology expert acknowledged that the patient improved remarkably on treatment and also questioned if they would have improved spontaneously. It was also suggested that the patient maybe a case of MS or CNS vasculitis. 

January 22nd 2022 morning 

Medicine PG suggested that the reason for diplopia was due to diffuse infarction in the midbrain causing mild paresis of cranial nerves 3,4 and 6 on examination. 

Further, the treatment of choice was justified as follows:

1. Patient had presented to a previous hospital with similar symptoms and statins and dual platelet therapy was given for secondary prevention of CVA

2. Diffuse restriction was seen on MRI which can be either true suggestive of infarct or false suggestive of demyelination and vasculitis

January 22nd 2022 evening 

Medicine PG reverted back to the Rheumatology expert with supporting factors for Neuro Behcet's and reasons for exclusion of the other differentials, these can be summarized as follows:

1. Reason for exclusion of Neuromyelitus optica: AquaPorin 4 antibody test was negative

2. Reason for exclusion of CNS vasculitis: Normal carotid angiography and MRI reports

3. Reason for exclusion of sarcoidosis: HRCT chest done for mediastinal lymphadenopathy was normal. 

4. Discussion on MS vs Neuro Behcet's: Quoting from the article about differences in neural lesions seen in both these cases: 

"Some symptoms are present in both the conditions, but the frequency of their presentation varies in the two forms, that is, optic neuritis, sensory symptoms, cerebellar symptoms such as dysarthria or ataxia, and spinal cord involvement are common in MS and are quite rare in NBD. On the other hand, headache, pseudobulbar speech, and precocious cognitive–behavioral changes are more common in NBD. Brainstem atrophy seen on MRI is very important for establishing the correct diagnosis of NBD, in particular, when its manifestations are precocious and isolated.[49] Lesions of the brainstem that commonly extend to the basal ganglia and diencephalic structures can strongly support the diagnosis of NBD, whereas MS lesions preferentially involve periventricular areas and the corpus callosum. The brainstem lesions in MS are usually very small. There is lack of brainstem and cerebellar atrophy and there is no cerebral loss in MS, which as has already been previously stated, is a typical finding in NBD"

This was quoted directly from the articles shared by the expert previously. 

 

Further, the Medicine PG informed the Rheumatology expert that patient has been responding well to Azathioprine and that due to patient affordability issues and lack of access, HLA testing and presence of Oligoclonal bands in CSF hasn't been done. 

January 23rd 2022

Rheumatology expert reverted back to Medicine PG and was pleased with the reasoning behind the diagnosis. 

Further discussion to be held on 25th January 2022 at the conference.