Showing results for delta air lines ±1 855 722191 colorado springs arson booking customer service 855 722191 1 855 service 855 855 722191 855 722191
Impaired Thermoregulation
[…] temperature. Clinically, poikilothermia can be manifested by hypothermia (core temperature less than 35°C/95°F) or hyperthermia (core temperature > 37.8°C /100°F). Etiology Imp aired thermoregulation is a known complication seen in persons with spinal cord injury (SCI), particularly those with level of injury […]
Chemodenervation and Neurolysis
[…] effect through nonselective denaturing of proteins upon application to a nerve.1 This in turn interferes with nerve conduction and imp airs innervation to muscles. Phenol for spasticity is typically used at concentrations between 5% and 7%, and ethyl alcohol in […]
Rehabilitation Management of Hematologic Malignancies and Bone Marrow Transplant (Adults and Pediatrics)
[…] replication of cancerous cells leads to increased viscosity of the blood, increased susceptibility to infections, and cytopenias of other cells lines. Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time) Patients may experience a number of symptoms including fatigue, fever, pruritis, night sweats, weight loss, bone pain, swollen lymph nodes, and bleeding. The initial symptoms often require a work-up including blood work and a bone marrow biopsy that eventually leads to the diagnosis. Abnormalities in cell blood counts are common and can affect white blood cells, red blood cells, and/or platelet levels. Patients are initially treated with three stages of chemotherapy. The first is induction chemotherapy which aims to acutely decrease abnormally high blood counts. The second, or consolidation phase, refers to chemotherapy used to achieve remission of the hematologic cancer. The maintenance phase is chemotherapy given after remission to keep the cancer from returning. Many patients will be offered hematopoietic stem cell transplants (SCT). SCT has the potential to cure some malignant disorders but may be associated with life-long morbidity. Donors for the stem cells can be autologous (from the patient) or allogeneic (from someone else). Allogeneic stem cells can be from a matched unrelated donor or a matched related donor (usually a sibling). Stem cells can be harvested peripherally or from umbilical cords. A conditioning regimen consisting of chemotherapy, monoclonal antibody or total body irradiation are utilized to prepare the bone marrow prior to stem cell infusion. Genetically engineered chimeric antigen receptor T-cell (CAR-T) have been used for refractory hematologic malignancy patients. Specific secondary or associated conditions and complications Patients can present with a number of symptoms or complications that should be addressed by physiatry. Chemotherapy Induced Peripheral Neuropathy (CIPN) is associated with the use of chemotherapy agents. Platinum agents (e.g., cisplatin), taxanes (e.g., pacilitaxel), vincristine, and bortezomib in particular are associated with CIPN. The CIPN can result in pain, weakness, and numbness, all of which can affect function. Patients with a preexisting neuropathy may be more at risk for developing symptoms. Steroid Myopathy is commonly associated with prolonged use of steroids. Steroids are often used in SCT patients to prevent or limit graft versus host disease (GVHD) and are often part of chemotherapy protocols. Patients present with predominantly Type IIB muscle fiber atrophy and proximal greater than distal weakness. This may result in difficulty with sit-to-stand transfers and ascending/descending stairs. Deconditioning/Asthenia is due to the inflammatory and hormonal effects of the cancer and its treatment, medical complications, prolonged immobility, and inadequate nutrition. Fatigue is commonly associated with this.1,2,3,4 Gastrointestinal and oral mucosal GVHD may further reduce oral intake and exacerbate malnutrition. Cognitive dysfunction can be the result of a number of causes related to the malignancy and its treatment, or due to the presence of CNS lesions or leptomeningeal disease. Other causes include cerebrovascular accidents, both hemorrhagic from thrombocytopenia and ischemic from hypercoagulability.5 Graft Versus Host Disease (GVHD) is a common occurrence after a hematopoietic stem cell transplant. Symptoms vary on the site of the GVHD. Acute GVHD typically occurs during the first 100 days after transplantation and may affect the dermal and gastrointestinal symptoms. Gastrointestinal GVHD can cause significant abdominal pain, nausea, diarrhea, and weakness. Chronic GVHD may be potentially most devastating in terms of function and quality of life. Chronic GVHD can affect many systems including the skin/fascia, oral, ocular, dental, cardiac, and pulmonary systems. Fascial cGVHD, for example, can cause edema, fibrosis, and joint contracture. Appropriate positioning, range of motion exercises, edema management, and splinting may be of some utility. Cytokine Release Syndrome (CRS) can be associated with CAR-T cell treatment and affect a number of organ systems including pulmonary, renal, liver and cardiac effects. Generalized symptoms including fatigue, chills, and fever are common. Neurologic symptoms include confusion, dizziness, decreased coordination and tremors. Immune effector cell-associated neurotoxicity syndrome (ICANS) can occur in 20-70% of CAR-T cell therapy patients about 3-10 days after administration. It can be associated with a number of neurologic symptoms including cognitive changes, hallucinations, aphasia, apraxia and seizures. ICANS can be potentially life threatening and may require ICU and mechanical ventilation. Edema. Patients can suffer from edema due to capillary leak syndrome (after SCT), malnutrition/hypoproteinemia, venous thrombosis, and lymphedema. This can have both pain and functional implications. The use of lymphedema treatment, such as complex decongestive therapy, (whether or not the etiology is truly lymphatic dysfunction) may be of benefit. Intravenous infusion of albumin and diuretics are also used. Monitoring of cardiac and renal status is recommended.6 Avascular necrosis – typically affects major joints including the femoral heads, knees, and shoulders. Chronic use of steroids, older age, female sex, diabetes, tobacco use, lupus, renal transplant, sickle cell anemia, alcoholism, and hematologic malignancy are risk factors.7 A history of radiation treatment, use of anti-estrogen medications and certain cytotoxic chemotherapies have also been implicated.8 Osteoporosis – patients are at increased risk of osteoporosis due to hormonal and inflammatory changes. Up to 50% of post-SCT patients have osteoporosis.9 Several issues need to be taken into account when treating this patient population: Leukopenia and neutropenia can be of concern. Patients may be at high risk for infection and could benefit from reverse isolation and refraining from group therapy sessions. Clinicians also need to also be sensitive to these concerns from a psychosocial perspective. As exemplified during the COVID-19 pandemic, these legitimate anxieties may take a significant emotional toll, as it may impede efforts in community integration and social activities. Anemia and thrombocytopenia can affect energy levels and ability to participate in therapy. Falls in these patients can be particularly severe due to their bleeding risk. Nevertheless, a recent narrative review concluded that exercise is likely safe and feasible with platelet counts >20,000/µL, although these patients should be closely monitored for any signs of bleeding.10 Essentials of Assessment History History should include […]
Pediatric Botulism
[…] N/A Social role and social support system N/A Rehabilitation Management and Treatments Available or current treatment guidelines Prophylactic intubation for airway protection should be done if progressive respiratory insufficiency is noted.25 Respiratory function may not end up being impaired until […]
Manual Treatments
[…] Art. No.: CD010277. DOI: 10.1002/14651858.CD010277.pub3. Accessed 02 October 2022. McIlwaine M, Button B, Nevitt SJ. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2019, Issue 11. Art. No.: CD003147. DOI: 10.1002/14651858.CD003147.pub5. […]
Ataxia Telangiectasia
[…] recurrent sinopulmonary infections, and progeroid symptoms, typically but not always including telangiectasias. Etiology The ATM gene maps to chromosome region 11q22.3 and its protein product is normally expressed in all tissues in the body. Its functions include: Response to deoxyribonucleic acid (DNA) damage and coordination of DNA repair, particularly double strand breaks due to ionizing radiation. Regulation of cell cycle and apoptosis and telomere maintenance. Response to oxidative stress. Mitochondrial homeostasis. Regulation of cellular protein turnover.1 Epidemiology including risk factors and primary prevention In the United States, the incidence of Ataxia-Telangiectasia is 1 out of 88,000 live births with a carrier frequency of 1% and a prevalence of about 1 in 40,000.2,3 Primary prevention via genetic counseling and prenatal diagnosis is possible. Avoiding consanguinity reduces but does not eliminate risks of autosomal recessive diseases. There is a 25% recurrence risk for each child when both parents are carriers.3 Patho-anatomy/physiology Patients with truncating or inactivating mutations (deletions, insertions, nonsense) in the ATM gene have no functioning ATM kinase, which appears to be involved in a surveillance mechanism that, in the presence of DNA damage, will stall progression of the cell cycle. This delay allows the cell to repair the damage. Without ATM kinase, cells can build up somatic mutations, leading to malignant transformation. The defect in nuclear DNA repair in AT also explains the sensitivity of cells to ionizing radiation.4 Mitochondrial DNA dysfunction may also contribute to AT and affects cerebellar, basal gangliar, and peripheral nerve function, leading to clinical features such as ataxia, neurodegeneration, and premature aging.5,6 Missense mutations tend to lead to a milder disease with later, even adult onset and more solid tumor than hematologic malignancy risk. The severity and progression rate vary even within affected families.5 Both the central and peripheral nervous systems are involved in AT. The thymus usually is hypoplastic, which is consistent with the associated immune deficiency.4 Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time) Patients with classic A-T have been shown to have decreased birthweight with a median percentile of 27% but have been noted to grow at an average rate until ~2 years.7 The first symptom is usually ataxic gait, seen soon after independent walking is achieved. In rare cases, dystonia may be the presenting symptom. From 0 to 5 years, recurrent sinopulmonary infections in those with immune deficiencies are common. Cerebellar atrophy on MRI may not be seen at this stage. Telangiectasia may be minimal or absent, but can occur around 3-4 years of age, especially in the bulbar conjunctiva. 8 By 5 to 15 years, increased falling and overt dysmetria are usually present, along with oropharyngeal dysphagia and dysarthria. Oculomotor apraxia (irregular saccades with extraocular movement testing) is usually present by 10 years of age. Walker and wheelchair use, supplemental tube feedings, and academic modifications start to become necessary. At this age the decreased height and weight become more apparent, especially in females. For females’ median height goes below the 3rd percentile around 11.5 years of age and for median weight around 10.5 years of age. For males’ median height goes below the 3rd percentile around 16.5 years of age and for median weight around 14.5 years of age.7 With the recurrent sinopulmonary infections it is recommended to start annual lung function testing at 6 years if age. 9 During the adolescent years, ataxia worsens and is accompanied by extrapyramidal symptoms including choreoathetosis, dystonia, tremor, reduced facial expression, bradykinesia, hyperkinesis, and sometimes spasticity. Acquired strabismus may be seen and aspiration is increasingly common. Cognitive impairment is frequently present at the end of the first decade of life.10,11 It should be noted that cognitive impairment is usually milder but does progressively worsen.8 More recent research shows patients can have cerebellar cognitive affective syndrome (CCAS) or Schmahmann’s Syndrome, which shows deficits in executive functioning, language, and overall intellectual disability that progressively worsens over time.12 During this stage Lymphoblastic leukemia and lymphoma are the most common malignancies. Beyond 15 years of age and into the third decade, there are also progressive central nervous system vascular abnormalities, and the risks of solid tumors increase. Insulin-resistant pre-diabetes or diabetes may occur. Pulmonary restrictive disease with interstitial fibrosis may develop in addition to recurrent infections and bronchiectasis. ILD can be seen in ~25 % of individuals with chronic respiratory symptoms or pulmonary disease as the reason for death.13 Variant AT has a milder course than classic AT, perhaps due to its residual ATM protein and kinase activity. Due to the more mild presentation the diagnosis of the variant form is much more difficult and in one study the delay to diagnosis was >10 years in 68% of patients, although 81% had symptoms by 10 years of age.14 The impact on growth is also not seen in the variant form as it is in the classic form.7 Although more mild, a significant portion of patients can have their function impacted with one study showing >70% of these patients using a wheelchair 20 years after their diagnosis. The variant form can be classified into various forms based on the neurological deficits with the main feature depending on the presence and predominance of extrapyramidal features. Extrapyramidal presentation, especially exclusive extrapyramidal symptoms, correlated with mild disease severity.The type of mutation is important in the variant form with missense mutations having less severe neurological disease, but increased risk of malignancy.More common malignancies in females include premenopausal breast cancer and hematological malignancies. In MRI, cerebellar atrophy and vermian atrophy can also be commonly seen.14 Specific secondary or associated conditions and complications Combined immunodeficiency with thymic dysplasia, decreased immunoglobulins, and reduction of T-lymphocytes occurs in 70%. Sinopulmonary infections occur in more than 80% of patients.4 Recurrent sinopulmonary infections and bronchiectasis alone can lead to chronic pulmonary compromise. Even in the absence of infection, patients can develop interstitial lung disease.15 Diabetes mellitus type 2 due to insulin resistance tends to develop in the third decade. Somatic growth is slowed and limited with adult heights below the third percentile, and pubertal growth spurt and fertility may not occur due to hypogonadism. In general individuals with classic A-T are also infertile.16 Hypopituitarism and/or hypothyroidism are not usually part of this condition but have been seen.8 Lifetime risk of cancer is 38%. Cancer risks include acute lymphocytic leukemia, lymphoma, and solid tumors, with extreme sensitivity to chemotherapy and radiation therapies. In children, 85% of neoplasms are lymphomas and acute leukemias. In adults, solid tumors are more frequent. The average lifespan is approximately 25 years although the average lifespan is increasing, with patients succumbing to progressive pulmonary disease secondary to repeated infection or cancer.6 Some individuals with later onset of disease and slower progression survive into their 50s.3 Cutaneous Granulomas9 Progressive daily fatigue16 Vitamin D Deficiency8 Scoliosis, pes cavus and equinus foot abnormalities8 Telangiectasia with 80-90% in conjunctiva, although this does not affect vision17 Radiation sensitivity Fatty liver disease and elevated liver enzymes18 Autoimmune and chronic inflammatory diseases such as ITP and vitiligo Essentials of Assessment History Birth and early developmental history (usually normal) Frequent falls, clumsiness, gait abnormalities Frequently dropping things Inability to sit or stand still Uncontrolled eye or limb movements Frequent infections Family history will usually be negative; ask sensitively about consanguinity, other members with neurologic disorders that could be AT mimics, and cancer occurrence particularly in young adults. Poor appetite7 Physical examination General: Small size, low weight. HEENT: Possible microcephaly, conjunctival telangiectasias can be seen at 3-4 years, small tonsils.8 Chest: abnormal breath sounds, congestion, crackles8 Neurologic: Initially intact sensation and negative Romberg but may later develop proprioceptive and vibratory loss. Look for truncal ataxia followed by dysmetria, dystonia especially in the hands and fingers, bradykinesia, facial and proximal hypotonia, choreoathetosis and myoclonic jerks on intention. Decreased or absent muscle stretch reflexes may be noted after age 7-8. Babinskis may eventually become upgoing, or mute. Ophthalmologic exam findings include oculomotor apraxia, slow hypometric saccades, head tilt, turn, or thrust, forced blinking, and absent optokinetic nystagmus. Strabismus may develop. Seborrheic blepharitis may be noted later. Orthopedic effects include equinovarus positioning with tight heel cords and postural kyphosis. Scoliosis is uncommon. Gait may show initial contact with whole foot, stomping, and leaning forward in running. Look for skin and hair progeria changes, such as gray hair and atrophic and inelastic skin, plus cutaneous telangiectasias, and sometimes hirsutism in women. Children with AT frequently have café-au-lait macules and cutaneous granulomas.8 Functional assessment Findings Seen in: First Decade Second Decade Mobility Ambulates with walker; ankle-foot orthoses: solid or articulated; frequent falls Wheelchair use more often; may need power chair depending on progression. Self-care Uses utensils; progressive oral and pharyngeal dysphagia; assistance for dressing/toileting May lose self-feeding and swallowing abilities; gastrostomy placement may be needed; drooling dependent for most activities of daily living Communication “Speaks softly in sentences with slow monotone, ‘scanning’ dysarthria and hypomimia (limited facial expression); fatigues with writing” Can communicate verbally but with severe fatigue and poor respiratory control; may lose abilities to read and write Social interaction Friendly, shy, responsive, appreciative, undemanding Good sense of humor; may be limited by severe fatigue Cognition Slow information processing; learns well auditorily Measured IQ may decline; short-term memory loss may develop19 Laboratory studies Consider this diagnosis in cases of progressive ataxia or “ataxic cerebral palsy” that worsen or develop additional neurologic findings. The European Society of Immunology Clinical Criteria for probable diagnosis20 Ataxia + at least 2 of the following 1. Oculocutaneous telangiectasia 2. Elevated AFP (10-fold upper limit of normal, fetoprotein >10ng/ml is seen in 95% of A-T individuals)21 3. Lymphocyte A-T karyotype 4. Cerebellum hypoplasia Elevated serum alpha-fetoprotein can […]
Vestibular Dysfunction (after brain injury)
[…] head back and forth. A normal result is more than a one-line change in younger individuals or more than two lines in older individuals. Functional assessment A thorough evaluation of the patient’s functional status takes into account not only the […]
Iliotibial Band Syndrome
[…] ultrasound study of 20 runners aged 18-40 years by Jelsing, supporting this theory 12. An alternative concept initiated by F airclough involves compression, by the ITB, of a highly vascular fat pad just deep to the ITB, creating inflammation and […]
Biomechanic of Gait and Treatment of Abnormal Gait Patterns
[…] which results in the forward progression of the center of gravity. It is generally established by 4-8 years of age. 1 Toddler’s gait has increased trunk movement, wide base of support, arms in high guard position, high foot lift during […]
Immune Mediated Neuropathies
[…] process, and others are an isolated peripheral nerve disorder. The major groupings include Acute immune-mediated neuropathy (Guillain-Barré Syndromes and variants) 1 Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) Acute motor and sensory axonal neuropathy (AMSAN) Acute motor axonal neuropathy (AMAN) Miller-Fisher syndrome (MFS) Sensory ataxic GBS Other acute variants such as acute pandysautonomic neuropathy Chronic immune-mediated polyneuropathy1,2 Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and variants Pure motor form or pure sensory form of CIDP Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), known as Lewis-Summer Syndrome Distal acquired demyelinating symmetrical neuropathy (DADSN) Chronic inflammatory axonal neuropathy Demyelinating neuropathies with monoclonal gammopathy of undetermined significance (MGUS) other than IgM Multifocal motor neuropathy (MMN) with/without conduction block Demyelinating neuropathy associated with monoclonal IgM MGUS with and without anti-MAG (Myelin Associated Glycoprotein) neuropathies Chronic ataxic neuropathy with ophthalmoplegia, monoclonal protein, cold agglutinins and disialosyl antibodies (CANOMAD) Chronic ataxic neuropathy with disialosyl antibodies (CANDA) Other immune-mediated neuropathies related to systemic immune disorders, and rarer types such as paraneoplastic neuropathies associated with anti-Hu antibodies and vasculitic neuropathies, neuropathy associated with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin abnormalities) syndrome Epidemiology including risk factors The annual incidence of immune-mediated neuropathies (IN) varies among specific types with the most common being GBS and subtype AIDP as well as CIDP, MMN, and POEMS syndrome. GBS The annual incidence is reported to be 0.81-2.3 per 100,000, the most common cause of acute, flaccid paralysis worldwide. Men are more often affected than women.3 The incidence increases in older age. AIDP is the major occurring subtype of GBS in Europe and North America (60-80%) while axonal forms such as AMAN and AMSAN are found more commonly in China and Japan (50% of cases). GBS is commonly precipitated by an infection with incidence varying depending on geographic locations and infectious outbreaks. Campylobacter jejuni (most common identifiable antecedent infection), Cytomegalovirus (CMV), Epstein-Barr virus (EBV) predominate as infectious pathogens. Other organisms implicated are influenza A, Mycoplasma pneumoniae, Haemophilus influenzae, Hepatitis A, B and C.2,4 Connection between C.jejuni and GBS is strong, particularly the AMAN variant (67%-92%). Zika virus is the recently identified antecedent infection with several-fold increased incidence reported in several countries as of 2016.4 The swine flu vaccine was felt to be a potential risk factor in 1976; however, in 2009 the risk in China was lower in those vaccinated than in those who were not.5 Coronavirus disease-2019 was reported to be associated with GBS by post-infectious immune-mediated mechanism.6 Other risk factors include autoimmune disorders, malignancy and surgery. Immune checkpoint inhibitors as cancer treatment are associated with GBS (about 0.1%) likely by a T-cell-mediated mechanism.7 Chronic acquired demyelinating neuropathies The overall prevalence of around 6 cases per 100,000 individuals, the most common type being CIDP with estimated prevalence rate of 1.0 to 8.9 cases per 100,000.8 MMN is a rare motor asymmetric neuropathy affecting no more than 1-2 individuals per 100,000 affecting males more than females by 3 times.8 Most chronic demyelinating neuropathies affect males more commonly and typically occur in the middle to old age.8 Etiology and pathogenesis Immune-mediated neuropathies most commonly occur when immunologic tolerance to key antigenic sites on myelin, axon, nodes of Ranvier or ganglionic neurons is lost. Demyelinating neuropathies (occurring at myeline) are the most common type. Either isolated demyelination or a combination of axonal injury and demyelination. Current evidence supports the notion that IN is mediated by antibodies directed against myelin antigens, along with autoreactive T cells and macrophages that invade the myelin sheath, axonal membranes or the nodes of Ranvier. In certain disorders the triggering factors have been identified and progress made in the understanding of immunopathologic process and in many others the exact mechanisms remain unclear.9 GBS10 AIDP – the main target appears to be the myelin AMAN- where primary pathology is in the axon, either due to massive acute demyelination and inflammation or due to a primary attack on axons and nodes of Ranvier mediated by macrophages and antibodies. AMSAN- which is like AMAN but with concurrent involvement of sensory axons. Miller-Fisher syndrome- the presence of IgG antibodies against GQ1b ganglioside. Sensory ataxic GBS- probably occurs due to the involvement of dorsal roots and ganglionic neurons. Some of these patients have also IgG antibodies to GQ1b or GQ1B ganglioside. Acute pandysautonomic neuropathy- where the target antigen is probably in the sympathetic ganglionic neurons. CIDP The pathogenesis is poorly understood. Different patterns of immune responses or different antigen targets underlie the different subtypes. The demyelination in CIDP is multifocal, like the one seen in GBS, affecting roots, plexuses, and proximal nerve trunks, accounting for variable distribution of symptoms. Inflammatory cells such as T cells and macrophages infiltrate nerves throughout the perineurium and endoneurim with disruption of blood-nerve barriers and macrophages splitting and destroying the myelin. Different T cell responses partly explain the clinical heterogeneity of CIDP. Humoral immunity processes such as antibodies and complements play a role in CIDP.11 The role of myelin proteins such as P0, myelin P2 protein, PMP22, and connexin as autoantigens in CIDP has not been confirmed. Nodal proteins such as neurofascin 186 (NF 186) and gliomedin, and paranodal proteins (NF 155 and contactin 1 associated with paranodal dissection) are identified and associated with the specific pathology. Antibodies to glycolipids LM1 (ganglioside, frequently with ataxia), GM1, or GD1b were subsequently detected in some patients with CIDP. Several studies suggest that genetically determined factors contribute to the development of CIDP.10 MMN IgM antibodies against GM1 were found in 43-85% of patients with MMN. Anti-GM1 antibodies (similar to anti-GM1 IgG Ab in AMAN) bind at the node of Ranvier and activate complement disrupting sodium channel clustering. Higher complement activating capacity is associated with severe axon loss and muscle weakness. Pathological studies of nerves revealed loss of myelinated axons as well as axonal degeneration and regeneration. One biopsy study suggested that axonal loss degeneration and loss predominate vs other studies demonstrated demyelination and onion bulbs.10 Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time) New onset/Acute GBS is a self-limited acute neuropathy characterized by symmetric flaccid paralysis, areflexia and sensory deficits that start 1-3 weeks after infection. Symptoms develop in hours to weeks and reach a nadir within 4 weeks, but often less than 2 weeks. 10% of GBS showed treatment-related fluctuation, typically within 4-8 weeks from onset. Between 4-15% of patients die, and up to 20% are disabled after a year despite modern treatment with worsening prognosis with advanced age. In children, the clinical course is more rapid with likely complete recovery.2,10 Subacute Subacute neuropathies (i.e., SIDP or subacute inflammatory demyelinating polyradiculoneuropathy) are defined as progressing for 4-8 weeks.3 Chronic/Stable CIDP is characterized by the occurrence of chronic progressive, relapsing or monophasic symmetrical weakness in both proximal and distal muscles, impaired sensation and paresthesia due to distal fiber sensory loss and absent or diminished reflexes which progress over 2 months.8,10 CIDP has an unpredictable course, although the majority of patients have severe/disabling motor, sensory, or sensorimotor deficits after several years. In a recent population-based study, the nadir overall neuropathy limitation scale score was 5, and 58 % of patients were unable to walk independently at some points of their illness.12,13 MMN presents with asymmetric weakness often related to the distribution of individual nerves. Arms are usually affected earlier and more severely than legs, with more than 80% of patients initially affected in forearm or hand muscles along with a patchy reduction of reflexes or sometimes even brisk reflexes (often confused with motor-neuron disease).2,10 Essentials of Assessment History and Physical Examination Acute (GBS) Progressive bilateral weakness in GBS with difficulty in climbing stairs getting out of chair is most notable 1-2 days after paresthesias.2 Cranial nerves including facial, bulbar, and ocular motor nerves are commonly involved in GBS (especially in MFS). MFS may present with ophthalmoplegia, ataxia, and areflexia.2 Respiratory muscles are commonly affected, and 25% of patients may need artificial ventilation. Autonomic dysfunction such as cardiac arrhythmias, arterial hypertension or hypotension, abnormal sweating, GI dysmotility may occur in two-thirds of patients Bowel or bladder complaints are rare. Autonomic complaints are common in GBS and variants. Chronic14 Weakness is proximal and distal in typical CIDP. Fasciculations and cramps common in MMN (approximately 50%). The typical presentation of anti-MAG neuropathy is that of distal, predominantly sensory large fiber ataxic neuropathy, like DADSN. Some patients may also exhibit a neurogenic tremor in the arms.10 Muscle stretch reflexes are diminished in GBS and CIDP by definition, except for the AMAN variant, which can have increased reflexes in the recovery phase. Differential diagnosis GBS: transverse myelitis, myasthenia gravis, botulism, carcinomatous or lymphomatous meningitis, toxin-related neuropathy, vasculitis-related neuropathy. CIDP IgA, IgM, or IgG monoclonal gammopathies Neuropathy related to human immunodeficiency virus (HIV), hepatitis C, Sjogren’s disease, lymphoma, ulcerative colitis and Crohn disease, melanoma and diabetes. Secondary immune-mediated neuropathies are associated with vasculitic diseases such as polyarteritis nodosa (PAN), Wegener granulomatosis, Churg-Strauss syndrome (CSS), microscopic polyangiitis, temporal arteritis, drug-induced vasculitis, nonsystemic vasculitis neuropathy), connective tissue disease, rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren syndrome, systemic sclerosis, cryoglobulinemia), sarcoidosis, or malignancies.2 Electrodiagnosis Electrodiagnostics (EDX) is an important clinical tool for distinguishing demyelinating and axonal types.10 Electrodiagnostic criteria for demyelination Partial motor conduction block and temporal dispersion reduced motor conduction velocity (60% of the normal mean),10 prolonged motor latencies, and F wave latencies2 Conduction block10 Typical EDX findings in GBS Test results can be normal early in acute disease. Prolonged F wave or loss of F wave and H reflex may be the only finding if the patient is tested early.4 In AIDP, electrodiagnostic performed within 2-15 days after onset may shows motor conduction blocks (CB) in approximately 60% of patients. Conduction block resolves with the appearance of CMAPs with slow initial components and increased duration on stimulation proximal to the site of block, consistent with the remyelinating process.3 The other abnormalities are prolonged motor distal latencies and decreased amplitudes.15 Serial NCS lead to reclassification of GBS subtypes in as many as 40% of patients, especially from AIDP to an axonal form. Thus, the classification may differ among studies that employ single or repeated NCS.3 AMAN: decreased distal CMAPs, prolonged distal motor latency (DML), CB, and conduction slowing in forearm segments in the first week.10 CIDP16 American Academy of Neurology Ad Hoc Subcommittee Criteria in 1991 (requiring 3 of 4 following criteria) Reduced conduction velocities (CVs; e.g. 150% of ULN if the amplitude is reduced for distal latencies and F-waves) CB/temporal dispersion (TD); e.g. CB is >50% reduction of proximal/distal amplitude and abnormal temporal dispersion is >130% increase of p/d duration) European Federation of Neurological Societies/Peripheral Nerve Society Guideline in 2010 Definite: at least one of the following: At least 50% prolongation of DML above ULN in two nerves At least 30% reduction of motor CV below LLN in two nerves At least 20% prolongation of F-wave latency above ULN in two nerves (>50% if amplitude of distal negative peak CMAP 30% duration increase between the proximal and distal negative peak CMAP) in […]