Autonomic dysfunction occurs frequently in multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). Patients with either condition may present with autonomic symptoms such as bladder, sexual, cardiovascular, thermoregulatory, and gastrointestinal dysfunction, and fatigue, but autonomic symptoms that affect quality of life are underrecognized in clinical practice. The immunopathogenesis of MS has been considered to be associated with autonomic dysfunction. Applying appropriate treatment strategies for autonomic dysfunction is important to improve the quality of life of patients. Here we review autonomic dysfunction and how this is managed in patients with MS and NMOSD.

Citations

• Sexual function and related predictors in male with multiple sclerosis and neuromyelitis optica spectrum disorder: a case–control study
  Saeed Vaheb, Mohammad Yazdan Panah, Mohammad Mohammadi, Mohammad Amin Sadri, Narges Ebrahimi, Sarina Loghmani, Marjan Beigi, Vahid Shaygannejad, Omid Mirmosayyeb
  The Journal of Sexual Medicine.2025; 22(2): 274.     CrossRef

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic recurrent acquired immune-mediated disease of the peripheral nerves that presents with progressive sensory and motor deficits in all four limbs. Cranial nerve involvement is not as common as in Guillain-Barre syndrome, and central nervous system involvement including optic neuritis has rarely been reported in patients with CIDP. We recently experienced a case with classic CIDP involving bilateral facial and trigeminal nerves, right lower cranial nerves, and the right optic nerve.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy with heterogeneous features. Appropriate treatment will produce a favorable outcome, but a poor treatment response and severe disability have also been reported. The roles of the clinical phenotypes and electrophysiological features of CIDP as well as of autoantibodies against nodal and paranodal proteins have been highlighted previously due to their association with the treatment response and long-term prognosis. This review addresses the diverse factors associated with the prognosis of CIDP.

Citations

• Tendon-Sparing Extraocular Muscle Enlargement Associated With Chronic Inflammatory Demyelinating Polyradiculoneuropathy
  Antonios D. Dimopoulos, Anne Barmettler
  Ophthalmic Plastic & Reconstructive Surgery.2024; 40(2): e38.     CrossRef

Non-Hodgkin’s lymphoma (NHL) may initially present with atypical neurological manifestations, including paraneoplastic neurological syndromes. Herein, we report the case showing an initial manifestation of systemic NHL with paraneoplastic demyelination in the brain that initially mimicked the symptoms of stroke, seizure, and brain tumor. A high index of suspicion and timely diagnostic workup is required to prevent diagnostic delay and commence proper management of the condition. In this situation, a whole-body FDG PET/CT could be useful to screen for occult malignancy.

Electrodiagnostic tests (EDX) is essential for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). EDX could provide information about demyelinating pathology in the peripheral nerves. According to phenotypes, CIDP could be classified several phenotypes, which has different clinical manifestations, EDX could present a different distribution pattern of demyelinating lesions. In addition, EDX could be useful markers for predicting treatment response of prognosis of CIDP.

Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is a variant of chronic acquired demyelinating polyneuropathy. A 65-year-old women presented with upper arm weakness. A nerve conduction study showed conduction blocks over intermediate segments with sparing of distal compound action potentials. Magnetic resonance imaging revealed asymmetric hypertrophy of the brachial plexus on the affected side. These findings represent important electrophysiological and radiological evidence of MADSAM neuropathy. The condition of the patient began to improve after starting intravenous immunoglobulin administration.

It was sometimes difficult to differentiate between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and subacute inflammatory demyelinating polyneuropathy (SIDP). The CNS involvement of these polyneuropathies has rarely reported in the literature. We present the case of a 42-year-old man who developed rapidly developing inflammatory demyelinating polyneuropathy followed by right optic neuritis. This case showed progressive motor weakness and sensory dysfunction with time to nadir at 8 weeks, demyelination in nerve conduction study, no other etiology of neuropathy, no relapse during follow-up of 18 months, good response to steroid and complete recovery which favor SIDP more than A-CIDP. We experienced the case of SIDP associated with optic neuritis.

Background
and Objectives: The proximal and distal nerve segments are preferentially involved in acquireddemyelinating polyneuropathies (ADP). This study was undertaken in order to assess the usefulness of motor evokedpotential (MEP) and somatosensory evoked potential (SSEP) in the detection of the proximal nerve lesion in ADP.Methods: MEP, SSEP and conventional NCS were performed in 6 consecutive patients with ADP (3 AIDP, 3 CIDP).MEP was recorded from abductor pollicis brevis and abductor hallucis using magnetic stimulation of the cortex and thecervical/lumbar spinal roots. SSEP were elicited by stimulating the median and posterior tibial nerves. Latency fromcortex and cervical/lumbar roots, central motor conduction time (CMCT), EN1-CN2 interpeak latency were measuredfor comparison.Results: MEP was recorded in 24 limbs (12 upper and 12 lower limbs) and SSEP in 24 limbs (12 median nerve, 12posterior tibial nerve). F-wave latency was prolonged in 25 motor nerves (25/34, 73.5%). Prolonged CML and PMLwere found in 41.7% (10/24) and 45.8% (11/24), respectively. Interside difference (ISD) of CMCT was abnormallyincreased in the upper extremity, 66.7% (4/6 pairs) in case of CML-PML. EN1-CN2 interpeak latency was abnormallyprolonged in one median nerve (1/10) and LN1-P1 interpeak latency was normal in all posterior tibial nerves.Conclusions: MEP and SSEP may provide useful information for the proximal nerve and root lesion in ADP. MEPand SSEP is supplemental examination as well as complementary to conventional NCS.

Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has beenused in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.

We present a case with stepwise weakness and sensory involvement of both hands for more than 2 months. His nerve conduction study findings revealed prolonged terminal latencies, decreased motor and sensory conduction velocities and conduction blocks of both ulnar nerves, more severely on left side. And there were other abnormalities manifested with mononeuropathy multiplex. Increased cerebrospinal fluid protein was found. We diagnosed him as Lewis-Sumner syndrome and tried high dose oral steroid therapy for 2 months. He showed improvement of motor functioning with persistent conduction block.

By definition, the time to reach nadir in Guillain-Barre syndrome (GBS) is within four weeks. This is in contrast to the chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), which progress for at least two months. However, CIDP can take a relapsing and remitting form and could mimic treatment related fluctuations of GBS (GBS-TRFs) especially during the early phase of disease. We report a patient with CIDP who initially presented with a rapidly progressive limb weakness mimicking GBS, but finally showed good recovery after long term corticosteroid therapy.

Background
: The term

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated polyneuropathy. Corticosteroids,intravenous immunoglobulin (IVIG) and plasmapheresis have been reported to be effective treatment. Rarely, CIDP can occurin the patients with HIV infection. The clinical features and electrophysiological findings of CIDP are known to be similarin patients with and without HIV infection. We report a 30-year-old male with HIV infection associated CIDP who improvedafter the administration of intravenous immunoglobulin and long term oral prednisone.

Background
Peripheral neuropathy is the most frequent neurological complication in human immunodeficiency virus (HIV)infection, related with diverse etiologies including inflammation, opportunistic infection and side effects of medications. Thepurpose of the present study was to evaluate characteristics of HIV associated neuropathy according to the stage of HIVinfection. Methods: In reviewing the medical records of HIV patients who underwent electrodiagnostic studies between 1997and 2011, total 11 patients (all males; median age, 47 years; range, 28-71 years) with comorbid neuropathy were enrolled. Stageof HIV infection was categorized according to the Centers for Disease Control and Prevention (CDC) criteria. Classificationof peripheral neuropathy was based on clinical and electrophysiological features. Results: Distal symmetric polyneuropathywas observed in 8 patients (72.7%), inflammatory demyelinating polyneuropathy in 2 patients (18.1%), and polyradiculopathyin 1 patient (9.1%). Median CD4+ T cell count was 123/mm3 (range, 8-540/mm3) and 7 patients (60%) had the mostadvanced HIV disease stage (CDC-C3). There was no neuropathy caused by CMV infection. Conclusions: Distal symmetricpolyneuropathy was the most common type of neuropathy in HIV infection, but various forms of neuropathy such asinflammatory demyelinating polyneuropathy and polyradiculopathy were also present. HIV associated neuropathy is morefrequently associated with advancing immunosuppression, although it can occur in all stages of HIV infection.