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Arquivos de Neuro-psiquiatria Dec 2022Studies were carried out with the objective of evaluating the quality of life (QoL) of patients affected by chronic inflammatory demyelinating polyradiculopathy (CIDP)....
BACKGROUND
Studies were carried out with the objective of evaluating the quality of life (QoL) of patients affected by chronic inflammatory demyelinating polyradiculopathy (CIDP). However, the cognitive issue is still little addressed.
OBJECTIVES
To assess the QoL and cognitive impairment of patients with CIDP and to analyze whether there is a correlation between these parameters.
METHODS
Seven patients with CIDP and seven paired controls were subjected to: mini-mental state examination (MMSE); Montreal cognitive assessment (MoCA); digit symbol replacement/symbol copy test (DSST); fatigue severity scale (FSS); Beck depressive inventory-I (BDI-I), and a short-form of health survey (SF-36).
RESULTS
The mean age of the participants was 50 years (71.4% male). The MMSE and MoCA had no statistical difference between the groups. Patients showed superior results in the memory domain in the MoCA (5 vs. 2, = 0.013). In the DSST, we observed a tendency for patients to be slower. There was a strong negative correlation between fatigue levels and vitality domain (SF-36). There was no significant correlation between depression levels and QoL, and there was no correlation between depression and the results obtained in the cognitive tests. The patients presented higher levels of depression (15.28 vs. 3.42, < 0.001). A total of 57% had severe fatigue, 28.8% self-reported pain, and 57.1% complained of cramps.
CONCLUSION
There was no cognitive impairment in these patients. However, there was a tendency of slower processing speed. To better evaluate the alterations found, a study with a larger number of individuals would be necessary. Chronic inflammatory demyelinating polyradiculopathy affects the QoL of patients in different ways.
Topics: Humans; Male; Middle Aged; Female; Quality of Life; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Polyradiculopathy; Cognitive Dysfunction; Cognition; Neuropsychological Tests; Fatigue
PubMed: 36580963
DOI: 10.1055/s-0042-1758455 -
Cureus Nov 2022POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare disorder that can mimic chronic inflammatory...
POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome Masquerading as Chronic Inflammatory Polyradiculoneuropathy: A Case Report.
POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare disorder that can mimic chronic inflammatory demyelinating polyradiculopathy (CIDP). In this report, we present a case of a man with a new diagnosis of POEMS syndrome and a clinical picture of CIDP. He had prostate cancer (s/p prostatectomy) with known diffuse bony osteosclerotic lesions and a monoclonal gammopathy of undetermined significance (MGUS). The objective of this report is to highlight the importance of recognizing POEMS as a rare condition, differentiating it from CIDP, and initiating treatment as soon as possible. The diagnosis of POEMS can be delayed due to its extensive variety of clinical manifestations, and the extensive workup needed for the diagnosis.
PubMed: 36523729
DOI: 10.7759/cureus.31481 -
Journal of Clinical Medicine Nov 2022POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy,... (Review)
Review
POEMS is a rare clonal plasma cell disorder characterized by multi-systemic features that include demyelinating peripheral neuropathy, organomegaly, endocrinopathy, presence of monoclonal proteins (M-protein), and skin changes. Even though the pathophysiology is poorly understood, recent studies suggest that both clonal and polyclonal plasmacytosis leading to the production of pro-inflammatory cytokines and angiogenic mediators play the central role. These mediators including vascular endothelial growth factor (VEGF) are the driving forces of the syndrome. The diagnosis of POEMS is not always straight forward and often the diagnosis is delayed. It is based on fulfilling mandatory criteria of polyradiculoneuropathy and monoclonal protein and the presence of one major criterion (Castleman disease, sclerotic bone lesions, or elevated VEGF), and at least one minor criterion. Due to the presence of neuropathy, it can be confused with chronic inflammatory demyelinating polyradiculopathy (CIDP), and if thrombocytosis and splenomegaly are present, it can be confused with myeloproliferative neoplasms. Due to the rarity of the syndrome, clear guidelines for treatment are still lacking. Immediate treatment targeting the underlying plasma cell proliferation results in a dramatic response in most patients. The key is early diagnosis and immediate anti-plasma cell directed therapy for the best clinical outcomes. For patients with disseminated disease as defined by bone marrow involvement or more than three osteosclerotic bone lesions, high-dose chemotherapy with autologous hematopoietic stem cell transplant (ASCT) yields durable responses and is the preferred treatment in eligible patients. For patients with localized bony disease, radiotherapy has proven to be very effective. Lenalidomide and dexamethasone is a proven therapy in patients ineligible for ASCT. In this review article, we tackle the diagnostic approach and discuss the latest treatment modalities of this rare debilitating disease.
PubMed: 36498588
DOI: 10.3390/jcm11237011 -
Legal Medicine (Tokyo, Japan) Feb 2023
Topics: Humans; SARS-CoV-2; Phrenic Nerve; Polyradiculopathy; COVID-19; Death, Sudden
PubMed: 36410282
DOI: 10.1016/j.legalmed.2022.102177 -
International Journal of Molecular... Nov 2022The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on a combination of clinical, electrodiagnostic and laboratory features. The... (Review)
Review
The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on a combination of clinical, electrodiagnostic and laboratory features. The different entities of the disease include chronic immune sensory polyradiculopathy (CISP) and autoimmune nodopathies. It is debatable whether CIDP occurring in the course of other conditions, i.e., monoclonal IgG or IgA gammopathy, should be treated as a separate disease entity from idiopathic CIDP. This study aims to evaluate the molecular differences of the nodes of Ranvier and the initial axon segment (AIS) and juxtaparanode region (JXP) as the potential cause of phenotypic variation of CIDP while also seeking new pathomechanisms since JXP is sequestered behind the paranode and autoantibodies may not access the site easily. The authors initially present the structure of the different parts of the neuron and its functional significance, then discuss the problem of whether damage to the juxtaparanodal region, Schwann cells and axons could cause CIDP or if these damages should be separated as separate disease entities. In particular, AIS's importance for modulating neural excitability and carrying out transport along the axon is highlighted. The disclosure of specific pathomechanisms, including novel target antigens, in the heterogeneous CIDP syndrome is important for diagnosing and treating these patients.
Topics: Humans; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Axons; Autoantibodies; Syndrome
PubMed: 36362407
DOI: 10.3390/ijms232113621 -
Cureus Sep 2022We present a case of a 42-year-old female living with poorly controlled diabetes who presented with a nine-month evolution of ataxic gait, reduced motor and sensitive...
We present a case of a 42-year-old female living with poorly controlled diabetes who presented with a nine-month evolution of ataxic gait, reduced motor and sensitive function of lower and upper limbs, and postural anesthesia of fingers, feet, and toes. Deep tendon reflexes were abolished in the lower limbs and markedly diminished in the upper limbs. Cerebrospinal fluid (CSF) analysis showed a high protein level, and both imaging and serologic studies were normal. Although she had a previous electrophysiologic study showing distal symmetric polyneuropathy (DSPN) with an axonal lesion, nerve conduction studies were repeated, and a diagnosis of chronic inflammatory demyelinating polyneuroradiculopathy (CIDP) was made. According to the state of the art, intravenous immunoglobulin (IVIg) was started. The patient's Inflammatory Neuropathy Cause and Treatment (INCAT) score and Medical Research Council (MRC) Sum Score both improved after two cycles. Unfortunately, symptoms quickly recurred, and corticosteroids were introduced to try to delay symptom recurrence, although it worsened diabetes control. Later, IVIg was stopped due to nephrotic syndrome, and immunosuppression was initiated. CIDP is a potentially treatable disease, but the diagnosis must be made as soon as possible to start therapy and reduce sequelae. Neuropathy in patients living with diabetes is common, but patients must be monitored closely to enable a correct diagnosis and adequate treatment.
PubMed: 36304380
DOI: 10.7759/cureus.29390 -
Cureus Sep 2022The Landry-Guillain-Barré Strohl Syndrome (LGBS) or Guillain-Barré syndrome (GBS) is an acute, frequently severe, potentially fatal, and fulminant polyradiculopathy....
The Landry-Guillain-Barré Strohl Syndrome (LGBS) or Guillain-Barré syndrome (GBS) is an acute, frequently severe, potentially fatal, and fulminant polyradiculopathy. It is an autoimmune illness, which usually occurs as a sequela of certain known infections. In this case report, we are discussing the case of a 12-year-old girl who was managed in the ICU for LGBS successfully and recovered promptly. This case highlights the importance of timely administration of intravenous immunoglobulin (IVIG) therapy, which resulted in prompt recovery, reduced duration of ICU stay, and morbidity.
PubMed: 36237757
DOI: 10.7759/cureus.29047 -
Legal Medicine (Tokyo, Japan) Nov 2022
Topics: Humans; Phrenic Nerve; SARS-CoV-2; Polyradiculopathy; COVID-19; Death, Sudden
PubMed: 36182710
DOI: 10.1016/j.legalmed.2022.102153