Presentation
GBS commonly presents as an ascending motor paralysis. [neurologyindia.com]
April 10, 2018 ; 90 (15 Supplement) April 23, 2018 First published April 9, 2018, Abstract Objective: To describe an atypical presentation of AMAN Background: Guillain-Barré syndrome (GBS) typically presents as an acute sensory-motor inflammatory demyelinating [n.neurology.org]
Case Presentation: A 60‐year‐old African American man presented to hospital requesting admission for alcohol detoxification. He was a known polysubstance abuser and hepatitis C carrier. [shmabstracts.com]
The clinical presentation of GBS and ATM overlap varies extensively. [cureus.com]
We present a case in which the patient developed subacute motor and predominantly sensory neuropathy following epidural blockade. [f1000research.com]
Entire Body System
- Pallor
[…] anomaly Abnormal tendon morphology Upper limb dysmetria Abnormal sperm morphology Jerky ocular pursuit movements Impaired vibration sensation at ankles Dysmetria Infertility Cirrhosis Motor delay Bilateral sensorineural hearing impairment Optic disc pallor [mendelian.co]
There was no pallor, icterus, lymphadenopathy, thyromegaly, cyanosis, and clubbing, and Jugular venous pressure was not raised. Central nervous system (CNS) examination revealed no deficiency in higher mental and cranial nerve functions. [atmph.org]
- Short Stature
[…] limbs in infancy followed by severe and progressive kyphoscoliosis causing a reversal in proportions during childhood (short trunk and long limbs) and a final short stature in adulthood. [mendelian.co]
She has facial and bulbar involvement, mental retardation, kinky hair [Figure 1], and short stature. Scoliosis, foot deformities, neuropathy, and cerebellar signs were also noted. [annalsofian.org]
Gastrointestinal
- Constipation
Other autonomic symptoms include dryness of the eyes and mouth and gastrointestinal dysmotility, often manifested by alternating constipation and diarrhea or by early satiety from gastroparesis. [clevelandclinicmeded.com]
[…] initial manifestation of lupus Types of polyneuropathy Multiple mononeuropathies: 2° to Vasculitis Distal Symmetric Sensory-Motor 5 Epidemiology Most common type Clinical Sensory Usually predominant Loss: Distal; Symmetric Pain Autonomic: GI reflux; Constipation [neuromuscular.wustl.edu]
The most common side effects associated with tramadol include dizziness, nausea, constipation, somnolence, and orthostatic hypotension. [practicalpainmanagement.com]
Psychiatrical
- Fear
The fear of every parent of a child with GAN is that the day is coming when our child will have to leave us, taken away by this terrible disease, which finally wins despite years of us struggling, fighting a battle we could never win. [eurordis.org]
Neurologic
- Peripheral Neuropathy
Find out more about diagnosing peripheral neuropathy Causes of peripheral neuropathy In the UK diabetes (both type 1 and type 2) is the most common cause of peripheral neuropathy. [nhs.uk]
[…] development of peripheral neuropathy in cancer patients. [practicalpainmanagement.com]
Diabetes and Chemotherapy Neuropathy. Social intersection with peripheral neuropathy - Peripheral Neuropathy. [freightlanegroup.com]
Growing evidence suggests that GAN is a continuum with the peripheral neuropathy Charcot-Marie-Tooth diseases type 2 (CMT2). [institut-myologie.org]
Peripheral neuropathy is a very broad topic that demands classification for purposes of diagnosis, evaluation, and treatment. An initial distinction may be made between focal and generalized peripheral neuropathies. [neuropathyaction.org]
- Encephalopathy
Wernicke's encephalopathy after vertical banded gastroplasty for morbid obesity. BMJ 1996;312:434. [ Links ] 11. Oczkowski WJ, Kertesz, A. Wernicke's encephalopathy after gastroplasty for morbid obesity. Neurology 1985;35:99-101. [ Links ] 12. [scielo.br]
[…] stature Migraine Status epilepticus Ptosis Intellectual disability Optic atrophy Falls Cerebellar atrophy Polyneuropathy Kyphosis Sensory ataxia Sensory impairment Rare Symptoms - Less than 30% cases Cerebral atrophy Hypogonadism Mitochondrial myopathy Encephalopathy [mendelian.co]
2019 15 Role of Pregabalin in Treatment of Polyneuropathy in Multiple Myeloma Patients: A Retrospective Study. 38 Maschio M...Marchesi F 31361668 2019 16 Observation of novel COX20 mutations related to autosomal recessive axonal neuropathy and static encephalopathy [malacards.org]
Related to infiltration by granulomatous process CNS Variable presentations Frequency: 7% to 11% Pachymeningitis: Hypertrophic Headache: Chronic Paraplegia Cranial nerve paresis CSF: Pleocytosis Pituitary lesions Cerebral vasculitis Generalized: Seizure; Encephalopathy [neuromuscular.wustl.edu]
The etiology may be known (e.g., arsenical, diabetic, ischemic, or traumatic neuropathy) or unknown. encephalopathy and myelopathy are corresponding terms relating to involvement of the brain and spinal cord. [medical-dictionary.thefreedictionary.com]
- Confusion
[…] muscle weakness Mutism Generalized hypotonia Cytochrome C oxidase-negative muscle fibers EMG: myopathic abnormalities Bilateral ptosis Language impairment Respiratory insufficiency Paraparesis Axonal degeneration Abnormality of mitochondrial metabolism Confusion [mendelian.co]
Confusion occurs when healthcare professionals unfamiliar with neuropathic pain assume that all anticonvulsants or antidepressants may be efficacious in the management of neuropathic pain. [practicalpainmanagement.com]
- Neurologic Manifestation
Many case reports have documented the association of hepatitis E infection with a variety of neurological manifestations including encephalitis and different types of neuropathies ( 9, 10 ). [ncbi.nlm.nih.gov]
- Insomnia
[…] pelvis Dumbbell-shaped metaphyses Long coccyx Abnormal metaphyseal vascular invasion Relatively short spine Snail-like ilia Flared humeral metaphysis Myalgia Muscle cramps Hypoplastic cervical vertebrae Exercise intolerance Bipolar affective disorder Insomnia [mendelian.co]
Workup
2018/61/3/339/236609 Nerve biopsy has been widely used to investigate patients with peripheral neuropathy and is often the final step in the diagnostic workup of neuropathy of unknown origin. [ijpmonline.org]
A detailed workup for acute transverse myelitis was initiated while he was being managed with intravenous corticosteroids. [cureus.com]
Treatment
Secondary objectives of this study are 1) to assess motor and sensory disease symptoms pre- and post-treatment, 2) to examine neuropathology in peripheral nerve biopsies in response to treatment, 3) to examine cerebrospinal fluid (CSF) and to conduct [clinicaltrials.gov]
Selection criteria: We sought all randomised or quasi-randomised (alternate or other systematic treatment allocation) trials that examined the effects of any drug therapy in people with CIAP at least one year after the onset of treatment. [cochrane.org]
Disease in symmetric neuropathy related to capillary pathology Axonal loss Prognosis No treatment: 5 yr survival 7% Treatment induces remission in 90% Some residual deficit common Treatment Prednisone and Cyclophosphamide Improvement common: Especially [neuromuscular.wustl.edu]
[…] containing phenytoin and a (co-) analgesic for the treatment of chronic pain. [peertechz.com]
Treatment Treatment is symptomatic. Children with GAN and their families usually work with a medical team that includes a pediatric neurologist, orthopedic surgeon, physiotherapist, psychologist, and speech and occupational therapists. [childneurologyfoundation.org]
Prognosis
Furthermore, antiganglioside antibodies (anti‐GM) tend to portend a poorer prognosis. Its absence in our patient may be supportive of his good prognosis. Conclusions: Among the different subtypes of GBS the AMAN variety presents in 5%–10% of cases. [shmabstracts.com]
prognosis A primary axonal form of Guillain–Barré syndrome was first described by Feasby and colleagues 1 in 1986. [jnnp.bmj.com]
The prognosis and main prognostic indicators of Guillain-Barré syndrome. A multicentre prospective study of 297 patients. The Italian Guillain-Barré Group. [frontiersin.org]
Prognosis Although the clinical course initially can be severe in AMAN, recovery can be rapid. However, recovery may be significantly prolonged in patients with extensive axonal degeneration. [orpha.net]
Treatment and prognosis Treatment focuses on symptom control. [radiopaedia.org]
Etiology
Out of seven, six cases were published after 2007. [10] It was found in the literature that the responsible organisms in etiology of concurrent GBS and ATM are mycoplasma, influenza, Bartonella henselae, and mumps. [atmph.org]
We discuss the possible role of serum effects in the etiology of GAN and speculate as to the molecular nature of the gan- defect. [rupress.org]
Etiology In the majority of cases, AMAN occurs following Campylobacter jejuni infection, in particular following infection with strains of C jejuni that cause enteritis. [orpha.net]
Etiology Mutations in both alleles of the giaxonin gene on chromosome 16 cause large accumulations of neurofilaments, axonal distention ("giant" axons), and degeneration of the myelin sheath 7. [radiopaedia.org]
To conclude, that authors have developed a new penetrant and specific test to diagnose GAN among a set of individuals exhibiting CMT2 of unknown etiology to suggest that the prevalence of GAN is probably under-evaluated among peripheral neuropathies. [institut-myologie.org]
Epidemiology
Use at onset of corticosteroid Rx to prevent vascular occlusion Systemic connective tissue disorders & Vasculitis: Specific syndromes 4 Rheumatoid Arthritis Neuropathy: Vasculitic Epidemiology 10% of all RA 50% of RA with systemic vasculitis Longstanding [neuromuscular.wustl.edu]
Summary Epidemiology The overall annual incidence of GBS is estimated at between 1/91,000 and 1/55,000. [orpha.net]
Clinical and epidemiologic features of Guillain-Barré syndrome. J Infect Dis. 1997 Dec. 176 Suppl 2:S92-8. [Medline]. Walgaard C, Lingsma HF, Ruts L, Drenthen J, van Koningsveld R, Garssen MJ, et al. [medscape.com]
Benassi GD'Alessandro RGallassi RMorreale ALugaresi E Neurological examination in subjects over 65 years: an epidemiological survey. Neuroepidemiology. 1990;9:27-38. Google Scholar 36. [jamanetwork.com]
Pathophysiology
It is possible that several different pathophysiological processes exist in AMAN to explain the dichotomous recovery patterns: reversible distal nerve failure at motor terminals or conduction block at nodes of Ranvier in association with rapid recovery [jnnp.bmj.com]
These cases we have reported show how axonal variants of GBS may masquerade as demyelinating pattern in initial nerve conduction studies and how do serial studies help in understanding the underlying pathophysiology. 1. [annalsofian.org]
Because pathophysiology and symptoms are related, polyneuropathies are often classified by area of dysfunction: Myelin Vasa nervorum Axon Polyneuropathies may be acquired or inherited. [merckmanuals.com]
Back to Top Pathophysiology and Natural History Despite the diverse array of medical disorders that cause peripheral neuropathies, peripheral nerves exhibit only a few distinct pathologic reactions to an insult or disease: wallerian degeneration, axonal [clevelandclinicmeded.com]
The NMDA receptors within the spinal cord have been shown to play a significant role in the pathophysiology of chronic neuropathic pain. [practicalpainmanagement.com]
Prevention
Preventive treatment. The Journal of Diabetic Foot Complications 2012; Volume 4, Issue 2, No. [freightlanegroup.com]
Much of this research focuses on finding better ways to prevent, treat, and ultimately cure inherited neurological disorders such as GAN. [childneurologyfoundation.org]
Preventing CIPN Calcium and magnesium infusions have been shown to reduce the incidence of CIPN. [practicalpainmanagement.com]
Generally, the sooner peripheral neuropathy is diagnosed, the better the chance of limiting the damage and preventing further complications. [nhs.uk]
Centers for Disease Control and Prevention. Available at http://www.cdc.gov/zika/hc-providers/diagnostic.html. 2016 Feb 11; Accessed: 2016 Feb 12. Souayah N, Nasar A, Suri MF, Qureshi AI. [medscape.com]