A regional consensus recommendation on brain atrophy as an outcome measure in multiple sclerosis.

BACKGROUND: Multiple sclerosis (MS) is a chronic autoimmune disease characterized by inflammatory and neurodegenerative processes leading to irreversible neurological impairment. Brain atrophy occurs early in the course of the disease at a rate greater than the general population. Brain volume loss (BVL) is associated with disability progression and cognitive impairment in patients with MS; hence its value as a potential target in monitoring and treating MS is discussed. METHODS: A group of MS neurologists and neuro-radiologists reviewed the current literature on brain atrophy and discussed the challenges in assessing and implementing brain atrophy measurements in clinical practice. The panel used a voting system to reach a consensus and the votes were counted for the proposed set of questions for cognitive and brain atrophy assessments. RESULTS: The panel of experts was able to identify recent studies, which demonstrated the correlation between BVL and future worsening of disability and cognition. The current evidence revealed that reduction of BVL could be achieved with different disease-modifying therapies (DMTs). BVL provided a better treatment and monitoring strategy when it is combined to the composite measures of "no evidence of disease activity" (NEDA). The panel recommended a set of cognitive assessment tools and MRI methods and software applications that may help in capturing and measuring the underlying MS pathology with high degree of specificity. CONCLUSION: BVL was considered to be a useful measurement to longitudinally assess disease progression and cognitive function in patients with MS. Brain atrophy measurement was recommended to be incorporated into the concept of NEDA. Consequently, a consensus recommendation was reached in anticipation for implementation of the use of cognitive assessment and brain atrophy measurements on a regional level.

Corticosteroids can help distinguish between Guillain-Barré syndrome and first attack of chronic inflammatory demyelinating neuropathy: an illustrative case report.

OBJECTIVE: To report a case of acquired demyelinating neuropathy that failed to improve upon treatment with intravenous immunoglobulins and plasmapheresis but responded dramatically to corticosteroids, illustrating the occasional difficulty in distinguishing Guillain-Barré syndrome (GBS) from a first attack of chronic inflammatory demyelinating polyneuropathy (CIDP). CLINICAL PRESENTATION AND INTERVENTION: A 25-year-old previously healthy man was admitted with a 5-day history of ascending areflexic paralysis of all 4 limbs and diagnosed with GBS. On admission, he was administered intravenous immunoglobulins at a dosage of 400 mg/kg/day for 5 days yet continued to worsen. He became quadriparetic by the second week. As there had been no improvement, he was plasmapheresed with 7 sessions of plasma exchange, 50 ml/kg of plasma at each session, including appropriate replacement fluid. After failing to improve within 8 weeks, he was started on intravenous methylprednisolone and a dramatic improvement was observed by the 5th day. He continued to get better on oral prednisolone, was ambulatory with support 4 weeks later and could walk without support on follow-up. CONCLUSION: This case illustrates that there is a subset of patients initially diagnosed with GBS who do not respond to immunoglobulins or plasmapheresis but do specifically well on steroids. Hence treatment with prednisolone should not be delayed in selective cases of GBS as it may actually be a first episode of CIDP.

Cerebral Venous Thrombosis in a Patient with Iron Deficiency Anemia and Thrombocytopenia: A Case Report.

AIM: To report a potential association of thrombosis, thrombocytopenia with iron deficiency anaemia. CASE REPORT: A 43-year-old female experienced an episode of a headache, with bilateral papilledema by neurological examination, magnetic resonance venography (MRV) brain showed cerebrovenous thrombosis (CVT), iron deficiency anaemia and thrombocytopenia by blood investigations, that was treated with iron supplementations and anticoagulation. CONCLUSION: In this patient, cerebrovenous thrombosis (CVT) was discovered in a patient with thrombocytopenia and iron deficiency anaemia and treated with iron supplements and anticoagulation, we concluded that thrombocytopenia is not a protective factor against thrombosis especially with iron deficiency anaemia.

Intravenous immunoglobulin in the treatment of drug rash eosinophilia and systemic symptoms caused by phenytoin.

A 32 year old Asian female on 300 mg per day of phenytoin following meningioma excision developed a fever with a diffuse maculopapular rash, lymphadenopathy and splenomegaly after12 days. A diagnosis of DRESS (Drug Rash Eosinophilia and Systemic Symptoms) syndrome was made. Patient was started on prednisolone at a dose of 1 mg/kg but since there was further deterioration in her condition, intravenous immunoglobulin was started. Clinical and blood parameters began to improve by the next day with liver functions returning to normal by the third week. DRESS syndrome is a drug hypersensitivity syndrome which can be fatal and therefore needs to be recognized early for the appropriate treatment to be started. The use of Intravenous immunoglobulins is anecdotal and the dramatic improvement noted in this case indicates that it is another treatment choice. The case and a brief review of the literature are discussed.