Clinical heterogeneity of low flow spinal arteriovenous fistulas; a case series.

BACKGROUND: Spinal AVF (SAVF), a potentially treatable cause of myelopathy, remains a challenging diagnosis. Its rarity and non-specific imaging findings often result in misdiagnosis despite a high index of clinical suspicion. The classically described high T2 signal in the spinal cord or prominent vascular flow voids in the intradural space were not infrequently missed on initial imaging, only to be picked up at follow-up imaging after progression of symptoms. Additionally, small sized fistulas(< 1 mm) and SAVF involving less frequent locations like the craniocervical junction in a patient presenting with paraplegia further complicates the diagnosis. On rare occasions, acute atypical presentation following a surgery adds to the conundrum. Definite diagnosis with spinal angiography, the gold-standard modality requires the expertise of highly skilled interventionists which may otherwise lead to false negative findings. We describe four SAVF patients with unconventional presentations, highlighting less described clinical findings. CASE PRESENTATION: First was a 50-year-old man presented with spastic paraparesis and was found to have an AVF at the cervical region arising from the vertebral artery. Second, a 45-year-old man with acute paraplegia post-operatively, initially treated for a transverse myelitis before lumbar region AVF was detected. Thirdly, a 27-year-old man presented with subacute lower thoracic myelopathy and deteriorated after corticosteroid treatment. The last patient, who initially appeared to have conus medullaris/cauda equina syndrome had a SAVF at the mid thoracic level. Presentation varied with some exhibiting acute deterioration mimicking other spinal cord pathology such as inflammatory disorders. All patients eventually underwent endovascular treatment with successful embolization of SDAVF. None of them exhibited further neurological deterioration after embolization. CONCLUSION: Successful treatment of SAVF is possible provided the diagnosis is made early, allowing timely intervention. Certain clues may aid the diagnosis. Firstly, arteriovenous fistula can be located distant to the clinical localization of myelopathy resulting in the unexpected longitudinally extensive spinal cord signal change. This clinical-radiological discrepancy can be a useful clue in diagnosing SAVF. Secondly, an acute myelopathic presentation immediately post-surgery may be related to SAVF. Other SAVF feature of note includes progressive myelopathy mimicking immune-mediated myelitis among young adults below 30 years of age refractory to immune therapy.

Prevalence, correlates and impact of pain and cramps in anti-MAG neuropathy: a multicentre European study.

BACKGROUND AND PURPOSE: The frequency of pain and cramps is uncertain in anti-myelin associated glycoprotein antibody (anti-MAG) neuropathy. Whether these symptoms may affect function/quality of life is unknown. METHODS: A cross-sectional study of the prevalence, correlates and impact of pain, pain subtypes and cramps, their severity, frequency and anatomical distribution was performed for 55 clinically stable patients with anti-MAG neuropathy. RESULTS: Pain of any type was reported by 80% of subjects. The most common subtype was paraesthesiae and dysaesthesiae (70%). Cramps were reported by >60% of patients, with lower limb cramps in all and upper limb cramps in about 20%. Cramps affected daily activities in >30% of these subjects, sleep in 60%, ability to exercise in >30%. Total pain score correlated with several Short Form 36 health-related quality of life (SF-36 HR-QoL) measures (P < 0.05), with Inflammatory Rasch-built Overall Disability Scale (I-RODS) (P = 0.006) and 10-m timed walk (P = 0.019). An independent association was ascertained with I-RODS (P = 0.002). Different pain subtypes showed multiple associations with SF-36 HR-QoL measures and/or functional scales. Upper limb cramps had multiple SF-36 HR-QoL functional correlates, with an independent association with the Overall Neuropathy Limitation Score (ONLS) (P = 0.004). Cramp severity correlated with ONLS (P = 0.04) and I-RODS (P = 0.028) and inversely with level of physiotherapy input (P = 0.009). Cramp frequency was associated with tremor score (P = 0.004) and multiple SF-36 HR-QoL subsections. CONCLUSIONS: Neuropathic pain and cramps may affect function and quality of life in anti-MAG neuropathy. Optimizing treatments of these symptoms, including by adequate levels of physiotherapy, may be beneficial in affected patients and requires further research.

Steroids for chronic inflammatory demyelinating polyradiculoneuropathy: evidence base and clinical practice.

Evidence-based therapies for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consist of corticosteroids, intravenous immunglobulins (IVIg), and plasma exchange. Steroids represent the oldest treatment used historically. In countries where readily available and affordable, IVIg tends to be favored as first-line treatment. The reason for this preference, despite substantially higher costs, is the perception that IVIg is more efficacious and safer than corticosteroids. However, the unselected use of IVIg as a first-line treatment option in all cases of CIDP raises issues of cost-effectiveness in the long-term. Furthermore, serious although rare, particularly thromboembolic side effects may result from their use. Recent data from randomized trials suggest pulsed corticosteroids to have a higher potential in achieving therapy-free remission or longer remission-free periods compared with IVIg, as well as relatively low rates of serious side effects when given as pulsed intravenous infusions during short periods of time. These specific advantages suggest that pulsed steroids could in many cases be used, as the first, rather than second choice of treatment when initiating immunomodulation in CIDP, primarily in hopes of achieving a remission after the short-term use. This article reviews the evidence base for the use of corticosteroids in its various forms in CIDP and factors that may influence clinicians' choice between IVIg and pulsed steroid treatment. The issue of efficacy, relapse rate and time, and side effect profile are analyzed, and some aspects from the authors' experience are discussed in relation to the possibility of using the steroid option as first-line therapy in a large proportion of patients with CIDP.

Hyponatraemia in Guillain-Barré syndrome revisited.

OBJECTIVES: The objective of this study was to determine the relevance of hyponatraemia in the prognosis of Guillain-Barré syndrome (GBS). MATERIALS AND METHODS: We retrospectively analysed records of 48 consecutive patients with GBS and performed a systematic literature review on frequency/correlates of hyponatraemia in GBS. RESULTS: Hyponatraemia <133 mmol/l was detected in 18/48 of our patients with GBS (37.5%). In 10/18 (55.5%), hyponatraemia occurred post-immunoglobulin therapy. Hyponatraemia correlated with age >50 years (P = 0.011), concurrent malignancy (P = 0.039), diuretic use (P < 0.001), preceding diarrhoea (P = 0.042) and Medical Research Council (MRC) sum score at discharge (MRCSSD) (P = 0.026). Only concurrent malignancy (P < 0.001) and diuretic use (P < 0.001) were independently associated with hyponatraemia. MRCSSD also correlated with MRC sum score on admission (MRCSSA) (P < 0.001), length of hospital stay (P < 0.001), summated compound muscle action potential (P = 0.034) and lowest forced vital capacity (P = 0.001). Only MRCSSA (P = 0.004) and length of hospital stay (P < 0.001) independently predicted MRCSSD. Combining our findings with previous literature indicates comparable frequencies of hyponatraemia in GBS in four of five studies and association with mortality in three of four studies, with an independent link in one. Independent association of hyponatraemia with muscle strength is not demonstrated. CONCLUSION: Hyponatraemia appears of comparable frequency in GBS to that in other diseased cohorts suggesting it is common but non-specific. Hyponatraemia has otherwise been shown to be an independent predictor of death in other disorders and available data indicate the same is also likely in GBS, although this may vary in patient subgroups. Hyponatraemia is, however, not an independent prognostic indicator of neuromuscular weakness severity in GBS.

Disease impact and patient insight--a study on a local population of asthmatics.

The cornerstone of asthma management is achieving adequate symptom control and patient education. We studied in our local population of asthmatic patients how well their symptoms were controlled with currently prescribed treatment and their insight into the disease and its management. Over a 6-month period, 93 asthmatics recruited from two local government health clinics and a state hospital were interviewed using a standard questionnaire. Patients were classified into 4 groups based on the treatment they were on according to Global Initiative for Asthma (GINA) treatment guidelines. The number of patients in Step 1 (rescue medication alone), Step 2 (1 controller medication), Step 3 (2 controller medications) and Step 4 (at least 3 controller medications) were 8, 39, 34 and 12, respectively. Except for day symptoms in Step 1 group, fewer than 50% achieved minimum day or night symptoms and no restriction of daily activities. Questions on patient insight were only available for 50 patients. Weather change (74%), air pollution (66%) and physical stress (46%) were the three highest ranked common asthma triggers. More than half correctly recognized the important symptoms of a serious asthma attack but fewer than 15% were familiar with the peak flow meter and its use or with the asthma self-management plan. Most patients perceived that their treatment had helped reduce disease severity and exacerbations. We conclude that symptom control and some aspect of patient education are still lacking in our local asthmatics.