Materials and methods: We analysed prospectively collected TPE data for patients treated with centrifugation TPE at our non-acute neurology TPE unit in Kuala Lumpur Hospital between May 2015 and June 2018.
Results: A total of 245 TPE procedures were performed in 55 patients for nine neurological indications, predominantly the central nervous system (79%). Twenty four per cent (n=13) had category I and 73% (n=40) had category II indication (American Society for Apheresis (ASFA) 2019). Others (4%) were not in ASFA indications. Neuromyelitis optica spectrum disorders accounted for half (51%) of the total patients. Twenty-three (41.8%) patients experienced adverse events, with hypotensive episodes being the the most common (n=12/55, 21.8%). Five (9.1%) patients had catheter-related blood stream infection, correlating with higher exchange plasma volume (p=0.023). Symptomatic hypocalcaemia was less common (n=5/55, 9.1%) and allergic reaction to human albumin was rare (n=1/55, 1.8%). Four technical errors detected. Three involved centrifugation sets manufacturing defects and one involved error in centrifugation set installation. Seven (2.9%) procedures were terminated: 5 for adverse effects and 2 for technical errors.
Conclusion: Performing TPE among semi-critical patients with neurology manifestations in basic non-acute set-up proved safe, with predictable complications. This set-up reduced the reliance on critical care services for TPE procedures.
PATIENTS AND METHODS: Clinical characteristics and electrophysiological data of sixty-one consecutive patients admitted between 2012 and 2015 were systematically analysed and reclassified according to the new GBS clinical classification. Neurophysiology was evaluated with Hadden et al.'s vs recently proposed Rajabally et al.'s criteria. Functional severity and clinical outcome of various GBS subtypes were ascertained.
RESULTS: All patients initially identified as GBS or related disorders can be sub-classified into having classical GBS (41, 67%), classic Miller-Fisher Syndrome (MFS) (6, 10%), Pharyngeal-cervical-brachial (PCB) (3, 5%), paraparetic GBS (4, 7%), bifacial weakness with paresthesia (3, 5%), acute ophthalmoparesis (AO) (1, 2%) and overlap syndrome (3, 5%): one (2%) with GBS/Bickerstaff brainstem encephalitis overlap and 2 (3%) with GBS/MFS overlap. Greater proportion of axonal classical GBS (67% vs 55%, p=0.372) seen with Rajabally et al.'s criteria and a predominantly axonal form of paraparetic variant (75%) independent of electrodiagnostic criteria were more representative of Asian GBS cohort. Classical GBS patients had lowest admission and discharge Medical Research Council Sum Score (MRCSS), greater functional disability and longest length of in-patient stay. Twenty (20/21, 95%) patients who needed mechanical ventilation had classical GBS. Patients required repeated dose of intravenous immunoglobulin (5/6, 3%) or plasma exchange (4/4, 100%) more frequently had axonal form of classical GBS.
CONCLUSION: Phenotype recognition based on new GBS clinical classification, supported by electrodiagnostic study permits more precise clinical subtypes determination and outcome prognostication.
Methods: Compound muscle action potentials (CMAP) duration of 3 patients with POEMS syndrome and 2 with hyaline vascular type CD without clonal plasma cell dyscrasia were retrospectively analysed.
Results: Median and ulnar nerves distal CMAP duration were prolonged in all patients irrespective of plasma cell dyscrasia or M protein. All lower limbs distal CMAP responses were absent. Greatest distal CMAP duration prolongation was observed in median nerves for POEMS syndrome (17.0 ms, 158% upper limit normal) and in ulnar nerves for CD (9.8 ms, 47% upper limit normal). Distal/proximal CMAP duration ratio of <0.7 were seen in 33% of median and ulnar nerves studied among POEMS syndrome. Among nerves with ratio >0.7, all had distal CMAP duration prolongation (Range 7%-158% of upper limit normal).
Conclusions: Abnormal distal CMAP dispersion is not uncommon in POEMS syndrome and CD without clonal plasma cell dyscrasia or M protein. POEMS syndrome has greater distal CMAP duration in median and ulnar nerves, particularly in median nerve that can reach up to 150% of upper limit normal, compared to <50% in CD.
Significance: Detailed electrophysiological analysis of distal CMAP duration may help in distinguishing POEMS syndrome and CD.
OBJECTIVE: To provide guidance to primary care physicians on an integrated approach to managing PN with neurotropic B vitamins (B1, B6, and B12).
MATERIALS AND METHODS: A multidisciplinary panel of eight experts participated in an iterative quasi-anonymous Delphi survey consisting of two rounds of questions and a virtual meeting. A literature review formed the basis of the survey questions. The first round included multiple select, qualitative, and Likert Scale questions; the subsequent round consisted of 2-point scale (agree or disagree) questions that sought to develop consensus-based statements refined from the first round and recommendations derived from discussions during the virtual expert panel meeting.
RESULTS: Clinical recommendations for the use of neurotropic B vitamins (B1, B6, and B12) have been developed for the prevention of PN progression or to delay onset in patients at high risk of developing PN. Recommendations have also been provided for the assessment of PN etiology and considerations for the use of loading dose (high dose) and maintenance dose (lower dose) of these neurotropic B vitamins (B1, B6, and B12).
CONCLUSION: These clinical recommendations provide an initial step towards formulating comprehensive guidelines for the early and long-term management of PN with neurotropic B vitamins (B1, B6, and B12) and move beyond addressing only neuropathic pain associated with the late stages of PN.