Assessment of diagnostic criteria for multifocal motor neuropathy in patients included in the Italian database

Background and purposeThis study aimed to assess the diagnostic criteria, ancillary investigations and treatment response using real-life data in multifocal motor neuropathy (MMN) patients.MethodsClinical and laboratory data were collected from 110 patients enrolled in the Italian MMN database through a structured questionnaire. Twenty-six patients were excluded due to the unavailability of nerve conduction studies or the presence of clinical signs and symptoms and electrodiagnostic abnormalities inconsistent with the MMN diagnosis. Analyses were conducted on 73 patients with a confirmed MMN diagnosis and 11 patients who did not meet the diagnostic criteria.ResultsThe European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria were variably applied. AUTHOR:When applying the American Association of Electrodiagnostic Medicine criteria, an additional 17% of patients fulfilled the criteria for probable/definite diagnosis whilst a further 9.5% missed the diagnosis. In 17% of the patients only compound muscle action potential amplitude, but not area, was measured and subsequently recorded in the database by the treating physician. Additional investigations, including anti-GM1 immunoglobulin M antibodies, cerebrospinal fluid analysis, nerve ultrasound and magnetic resonance imaging, supported the diagnosis in 46%-83% of the patients. Anti-GM1 immunoglobulin M antibodies and nerve ultrasound demonstrated the highest sensitivity. Additional tests were frequently performed outside the EFNS/PNS guideline recommendations.ConclusionsThis study provides insights into the real-world diagnostic and management strategies for MMN, highlighting the challenges in applying diagnostic criteria

Impact of 2021 European Academy of Neurology/Peripheral Nerve Society diagnostic criteria on diagnosis and therapy of chronic inflammatory demyelinating polyradiculoneuropathy variants

Background and purpose: there are different criteria for the diagnosis of different variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines provide specific clinical criteria for each CIDP variant even if their therapeutical impact has not been investigated. Methods: we applied the clinical criteria for CIDP variants of the 2021 EAN/PNS guidelines to 369 patients included in the Italian CIDP database who fulfilled the 2021 EAN/PNS electrodiagnostic criteria for CIDP. Results: according to the 2021 EAN/PNS clinical criteria, 245 patients achieved a clinical diagnosis of typical CIDP or CIDP variant (66%). We identified 106 patients with typical CIDP (29%), 62 distal CIDP (17%), 28 multifocal or focal CIDP (7%), four sensory CIDP (1%), 27 sensory-predominant CIDP (7%), 10 motor CIDP (3%), and eight motor-predominant CIDP (2%). Patients with multifocal, distal, and sensory CIDP had milder impairment and symptoms. Patients with multifocal CIDP had less frequently reduced conduction velocity and prolonged F-wave latency and had lower levels of cerebrospinal fluid protein. Patients with distal CIDP more frequently had reduced distal compound muscle action potentials. Patients with motor CIDP did not improve after steroid therapy, whereas those with motor-predominant CIDP did. None of the patients with sensory CIDP responded to steroids, whereas most of those with sensory-predominant CIDP did. Conclusions: the 2021 EAN/PNS criteria for CIDP allow a better characterization of CIDP variants, permitting their distinction from typical CIDP and more appropriate treatment for patients

High-resolution ultrasound may depict pseudomeningocele

Pseudomeningocele is a pathological condition deriving from cerebrospinal fluid leak throughout a fissure of the dura mater. The came out fluid is enclosed in a space delimited by the soft tissues. It can be congenital, iatrogenic or traumatic (mainly in brachial plexus injury) and can be often associated with root avulsion, a severe condition which should be aware as soon as possible. The incidence of traumatic pseudomeningocele of this type is 21–57 % . In brachial plexus injury, magnetic resonance (MR) is the gold standard to assess the damage, allowing the visualization of root avulsion and pseudomeningocele . Even high-frequency ultrasound (US) may be used as first line tool in brachial plexus injury, showing data that, in some cases, require a comprehensive assessment through MR [4]. No cases of pseudomeningocele, visualized by US, have so far been reported. We describe two patients with traumatic brachial plexus injury, in whom US showed findings indicating the presence of fluid, close to a cervical root, compatible with pseudomeningocele

Neuropathic Pain in the Emergency Setting: Diagnosis and Management

Neuropathic pain, traditionally considered a chronic condition, is increasingly encountered in the emergency department (ED), accounting for approximately 20% of patients presenting with pain. Understanding the physiology and key clinical presentations of neuropathic pain is crucial for ED physicians to provide optimal treatment. While diagnosing neuropathic pain can be challenging, emphasis should be placed on obtaining a comprehensive medical history and conducting a thorough clinical examination. Patients often describe neuropathic pain as a burning or shock-like sensation, leading them to seek care in the ED after ineffective relief from common analgesics such as paracetamol and NSAIDs. Collaboration between emergency medicine specialists, neurologists, and pain management experts can contribute to the development of evidence-based guidelines specifically tailored for the emergency department setting. This article provides a concise overview of the common clinical manifestations of neuropathic pain that may prompt patients to seek emergency care

Transfer to inpatient rehabilitation facilities after neurological admission

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Sensitivity and specificity of a commercial ELISA test for anti-MAG antibodies in patients with neuropathy

For the diagnosis of anti-MAG polyneuropathy the commercial ELISA manufacturer currently recommends a cut-off of 1000 Biihlmann Titer Units (BTU). We analyzed sera from 80 anti-MAG neuropathy patients and 383 controls (with other neuropathies or healthy controls) to assess the ELISA sensitivity and specificity at different thresholds. A better combination of sensitivity/specificity was found at a threshold > 1500 BTU than at > 1000 BTU. The best value of specificity was obtained at threshold > 7000 BTU. There was a diagnostic grey area between 1500 and 7000 BTU in which the clinical phenotypes as well as electrophysiological studies need to be carefully assessed particularly to differentiate CIDP and anti-MAG neuropathy