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Background HIV-associated neurocognitive disorders (HAND) remain problematic even in the antiretroviral therapy (ART) era. Efavirenz (EFV) is well known for neuropsychiatric side effects and has been associated with worsening neurocognitive performance (NC). We hypothesized that switching from an ART regimen with EFV to one with rilpivirine (RPV) would improve NC. Methods This single-center, open-label, superiority, randomized controlled trial was conducted at Maharaj Nakorn Chiang Mai Hospital June 2018 to March 2019. HIV- infected adults (≥18 years), virologically suppressed on an EFV-containing regimen for at least 12 months, who met Frascati’s criteria of asymptomatic neurocognitive impairment (ANI), were randomized to a switching arm (RPV-containing regimen) vs. a control arm (EFV-containing regimen). The primary objective was to compare the improvement of NC at month 6 (M6) after switching. Secondary outcomes were individual NC domains, tolerability, and safety after switching regimens. Results 20 ANI participants (10 per group) completed the study. 11 participants showed NC improvement at M6: 6 in the switching arm and 5 in the control arm. Global neuro deficit scores (GDS), figure learning, and figure memory at M6 significantly improved in both arms (P < 0.05). Verbal memory and speed of information processing improved over time in the switching arm only (0.6 (0.1–1.0); P = 0.02, and 0.7 (0.1–1.2); P = 0.03, respectively). However, no significant differences between the two groups were observed. Male gender, age < 50 and initiation of ART within 5 years after diagnosis all trended toward an association with improved NC. No tolerability issues or clinical significant adverse events were reported. Conclusion Despite some positive trends, switching from an EFV-containing regimen did not significantly improve NC. This finding was consistent with previous reports. A larger study with longer follow-up may be warranted. Disclosures All authors: No reported disclosures.

Facial neuropathy is most commonly seen as an idiopathic unilateral palsy known as Bell’s palsy. Generally, acute onset of typical lower motor neuron facial weakness that is not associated with other atypical or suspicious features, remains unilateral, and recovers completely requires no further workup. A recurrent or bilateral peripheral facial palsy makes an idiopathic cause less likely and prompts a more in-depth workup. The appropriate work-up of unilateral or bilateral facial palsy guided by the presence or absence of associated clinical findings is discussed. The major differentials for bilateral facial paresis include brainstem (especially pontine and prepontine) tumors, Lyme disease (especially in endemic areas), basal menigitides, Guillain-Barre syndrome, and sarcoidosis.

Peripheral Nerve and Muscle Disease uses a case-based approach to cover common and important topics in the diagnosis and treatment of neuromuscular disorders. Neuromuscular cases are always challenging. This is because even with nerve conduction studies and electromyography diagnostic certainty can still be difficult. Even with recent advances in serologic and genetic testing diagnostic certainty may be elusive. Each chapter provides an overview of the approach to the problem in question followed by a discussion of the diagnosis, key points to remember, and selected references for further reading. For this edition, new cases include Lambert-Eaton syndrome, botulism, facioscapulohumeral muscular dystrophy, and several types of neuropathy. Peripheral Nerve and Muscle Disease is an engaging collection of thought-provoking cases that clinicians can use when they encounter difficult patients on the ward or in the clinic. The volume is also a self-assessment tool that tests the reader’s ability to answer the question “What do I do now?”

A number of nutritional causes of peripheral neuropathy have been identified. Patients who have gastrointestinal disease or who have undergone bariatric surgery are at risk, although frequently the specific cause is not ascertained. Vitamin B12 and thiamine deficiencies are the most common causes in bariatric surgery patients. The classic clinical picture of Vitamin B12 deficiency is a myelopathy with a concomitant peripheral neuropathy. Thiamine deficiency is the classic triad of mental status changes, opthalmoplegia, and ataxia. Copper deficiency is often unrecognized. Its presentation can be similar to Vitamin B12 deficiency. It is important that bariatric surgery patients receive continual follow up of their nutritional status and adherence to vitamin supplementation. Alcohol may cause a peripheral neuropathy through nutritional deficiency or perhaps direct toxic effects. Other deficiency states are discussed.

Multifocal motor neuropathy (MMN) may be mistaken for common entrapment neuropathies, although absence of significant sensory findings is a helpful clue to the diagnosis. Multifocal motor neuropathy may also mimic motor neuron disease. Electrophysiological evidence of conduction block at a nerve site not typically prone to compression is consistent with MMN. A positive anti-GM1 antibody also supports the diagnosis. First-line treatment of MMN is intravenous immunoglobulin (IVIG), and the majority of patients have rapid improvement of their weakness. The clinical features, differential diagnosis, investigations, and treatment options are described in this chapter.

An infant presenting with acute weakness is an alarming problem that requires urgent diagnosis and action. Acute inflammatory demyelinating polyradiculoneuropathy is always a consideration, but botulism is important to remember. We discuss the key clinical signs that distinguish botulism from other causes of acute weakness, including pupillary and gastrointestinal dysfunction. We review the various forms of botulism and the known epidemiology. Botulism-related neuromuscular junction pathophysiology is reviewed. We discuss treatment and stress that supportive modalities and BIG-IV should be instituted early. We discuss prognosis and reinforce that despite the condition's seriousness, prognosis is good with prompt recognition and treatment.

The sudden onset of leg weakness and pain may be mistaken for an acute lumbosacral radiculopathy due to a herniated disc. However, in diabetics it is also essential to consider the entity of Bruns-Farland syndrome, or diabetic lumbosacral plexopathy (DLSP). DLSP is uncommon compared with lumbosacral radiculopathy, so delays in diagnosis are the rule rather than the exception. In this chapter we present the clinical characteristics of DLSP and the key features that can help with making a prompt diagnosis. The use of nerve conduction studies and EMG for confirmation of DLSP is reviewed. Treatment options and controversies are discussed.

A patient presents with a sensorimotor neuropathy and believes he has been poisoned. The approach to the differential diagnosis of arsenic toxicity is presented. Comparisons with mimics of this entity are made, and clinical clues to its early detection are provided. There are typical skin and nail changes with can occur with arsenic poisoning. Arsenic poisoning can appear similar to Guillain-Barre syndrome with gastrointestinal symptoms and later an ascending paralysis. Urine arsenic levels are more reliable than blood levels. Hair and nail samples are very useful in confirming the diagnosis. Electrodiagnostic testing confirmed an axonal polyneuropathy. Treatment of arsenic poisoning is discussed. The recent lead contamination in Flint Michigan points out that heavy metal poisoning still occurs despite public health awareness.

Guillain-Barré syndrome may present in several ways, although predominant proximal weakness is a common feature of the disease to recognize. The differential diagnosis may be extensive and can include infection, vasculitis, toxin exposure, and malignancy. A lumbar puncture must be done with minimal delay to evaluate for cerebrospinal fluid (CSF) albuminocytological dissociation, however results may be normal early in the course of the disease. EMG/NCS are helpful to support the diagnosis, and early treatment with intravenous immunoglobulin (IVIG) is essential. This chapter discusses the clinical features and diagnostic considerations of this important condition.

Distal, symmetric polyneuropathy can occur in a number of conditions including in association with HIV infection. The most common neurologic complication of HIV infection is a painful, distal sensory polyneuropathy (HIV-DSP). Other neuromuscular manifestations of HIV are myriad especially in the setting of fluctuating immunocompetence and the potential impact of neurotoxic medications. In the patient described in the case, peroneal neuropathy as the cause for foot drop is suggested by ankle dorsiflexion and eversion weakness with preservation of inversion and knee flexion strength. Mononeuropathy in the setting of HIV should prompt consideration of vasculitic, lymphomatous, and infectious causes, some of which can manifest with systemic findings. A rare neuromuscular complication of HIV-infected patients with sicca symptoms suggestive of Sjogren’s syndrome is diffuse infiltrative lymphocytosis syndrome. This syndrome can manifest as an acute or subacute painful sensory polyneuropathy but also as a mononeuropathy multiplex. This is discussed in the chapter.

Neurogenic thoracic outlet syndrome (NTOS) is an uncommon cause of chronic arm pain and numbness but should not be missed. It can lead to chronic pain and disability. Symptoms of NTOS are often aggravated by arm exertion and elevation and tend to occur after exercise rather than activity. A thorough diagnostic evaluation is key, which includes provocative tests, imaging, electromyography/nerve conduction study, and diagnostic injections. Electromyography/nerve conduction study (EMG/NCS) are recommended for NTOS as it is important to exclude an entrapment neuropathy or cervical radiculopathy that may be misdiagnosed as NTOS. EMG/NCS is usually normal in NTOS, however, in severe presentations, the EMG/NCS pattern is most consistent with a lower trunk plexopathy. Treatment options of NTOS are also described in this chapter.

Chemotherapy-induced peripheral neuropathy is a common complication of cancer therapy. The diagnosis is generally straightforward, based on the temporal association of symptoms with the offending agent. However, it can sometimes take on a different form of severe, fulminant neuropathy and must be differentiated from other neurologic conditions that occur in the setting of cancer. Particular care must be taken in the patient with clinical indicators of longstanding, hereditary neuropathy as these can confer a distinctive susceptibility to neurotoxicity. The diagnosis relies on an index of suspicion and the exclusion of mimics of toxic neuropathy. The most important and common agents responsible for toxic neuropathy and their range of effects on nerve are discussed.

Sciatic neuropathy presents with buttock pain worsened by sitting on the affected side and associated with ankle and knee extension weakness. Electrodiagnostic evaluation will help to distinguish it from peroneal or tibial mononeuropathies, lumbosacral plexopathy, or lumbosacral radiculopathy. It can be difficult to distinguish from a peroneal mononeuropathy due to the preferential involvement of the peroneal division of the sciatic nerve. EMG study of the short head of the biceps femoris allows for distinction between these entities. Long-term outcome and prognosis studies are sparse although Preservation of distal lower-extremity strength may be a significant predictor earlier and/or better clinical recovery.

Lambert-Eaton myasthenic syndrome (LEMS) can be a difficult condition to diagnose. In this chapter, the clinical picture and characteristics are discussed. The pathophysiology is also reviewed. Features to distinguish LEMS from more common neuromuscular junction conditions such as myasthenia gravis are reviewed. We review features that distinguish between idiopathic and paraneoplastic forms, and we discuss the importance of tumor surveillance. Antibody testing and the correct electrodiagnostic strategy are presented. Treatment of LEMS is outlined.Lambert-Eaton syndrome (LES) is a difficult condition to diagnose. The unique clinical picture and its characteristics are discussed. This is especially true in the patient without a diagnois of cancer. Antibody testing and the correct electrodiagnostic strategy are presented. Treatment of LES is outlined.

Severe shoulder pain in the absence of a clear orthopedic cause may be due to acute brachial plexitis. Numbness and tingling in association with weakness and muscle atrophy that cannot be accounted for by a single nerve or nerve root distribution suggests the diagnosis. Additional clues suggesting brachial plexitis include intensity of shoulder pain and antecedent events such as illness, vaccination, injury, unusual physical activity or surgery. The approach to diagnosis of plexitis/plexopathy and appropriate evaluation for etiology are discussed. Management of this condition is conservative, relating to pain control and judicious use of mobilization and strengthening with physical therapy. Prognosis is generally good with recovery of strength occurring in weeks to months.

Hereditary neuropathies are common. They are often diagnosed when another medical issue begins, for example, orthopedic foot problems or chemotherapy that causes a persistent severe neuropathy. Our patient developed a peroneal neuropathy after a long international airline flight. Initially it was thought to be related to lumbosacral radiculopathy. Careful history, neurological examination, and electrodiagnostic testing was helpful in pointing to a peroneal neuropathy rather than a lumbosacral radiculopathy. Family history in evaluating patients with peripheral neuropathy is essential. The actual examination and testing of relatives may be necessary. Genetic testing for the confirmation of the diagnosis of hereditary neuropathy is outlined. Clinical features of the most common hereditary peripheral are listed. It is important to recognize that a patient has a hereditary neuropathy before therapy with a chemotherapeutic agent is initiated.

Treatment issues are discussed in the setting of a very challenging patient with amyotrophic lateral sclerosis with frontotemporal dementia (FTD), including management of neurological symptoms and cognitive dysfunction. Features of FTD are discussed. It is now appreciated that FTD is more common than previously thought. FTD can occur before the onset of motor symptoms. An ALS multidisciplinary clinic is the best approach for the care of ALS patients. Ethical issues are analyzed in this case as well. The need for the discussion of end of life issues is important for the patient family and caregivers. The careful consideration of tracheostomy and ventilator use is important in light of pulmonary function. Pulmonary function testing and sleep studies are helpful in managing respiratory issues. Palliative care consultation can be very beneficial.

Paraproteinemic demyelinating peripheral neuropathies require specific diagnostic and management approaches. The clinical features as well as the type of monoclonal protein and possible associated antibodies are all important considerations in evaluation and management of these complex presentations. It is important to recognize anti-myelin associated glycoproteins positive peripheral neuropathy, which is associated with IgM gammopathy, and typically presents with a sensory ataxia. Hematologic disease may mimic chronic inflammatory demyelinating polyneuropathy. This chapter also describes the role of hematological evaluation for patients with peripheral neuropathy and a monoclonal gammopathy.

Facioscapulohumeral muscular dystrophy (FSHD) is the third most common muscular dystrophy, following Duchenne muscular dystrophy and myotonic dystrophy. The clinical secerity is extremely variable, with symptom onset anywhere from infancy to middle adulthood. The cardinal clinical features of facioscapulohumeral muscular dystrophy include facial weakness and scapular winging. Other important examination findings including scalloping of the trapezius, “Popeye” forearms, horizontal axillary folds, and a positive Beevor’s sign. It can rarely present as a pattern of weakness mimicking limb-girdle muscular dystrophy with approximately 20% of patients eventually requiring a wheelchair for ambulation. Creatine kinase is normal or mildly elevated. Genetic testing for the D4Z4 repeat contraction on chromosome 4q35 detects 95% of cases but may not reflect severity of the disease.

Neuromuscular disorders are important causes of newly acquired weakness in the intensive care unit. Although evaluation usually begins with physical examination findings, these can be compromised in the intensive care unit environment. Therefore, electrodiagnostic study becomes even more important as a tool in localizing weakness to nerve, muscle or neuromuscular junction. Critical illness neuropathy and myopathy occurs in the setting of sepsis and multiple organ failure where sepsis is accompanied by the systemic inflammatory response syndrome. Additional, intensive care unit-specific risk factors exist, predominantly relating to administration of high-dose steroids, nondepolarizing neuromuscular blocking agents, and sedating agents such as propofol. There is no specific treatment except for optimizing medical management of the underlying disorder, including prevention and management of sepsis, systemic inflammatory response syndrome, and organ damage, as well as avoidance of exacerbating medications.

Small-fiber neuropathy typically presents with burning pain or with widespread brief stabbing pains, by atypical presentations including asymmetric sensory symptoms are common. Nerve conduction studies are usually normal, as this disorder test only interrogates large fiber function; in small-fiber neuropathy the pathology is restricted to smaller unmyelinated fibers. Autonomic neuropathy can accompany the painful peripheral neuropathy but can be difficult to recognize since the symptoms can be protean. In this chapter, clinical characteristics of small-fiber and autonomic neuropathy are discussed. Various diagnostic modalities are described, including the benefits and pitfalls of available options. The most common conditions causing small-fiber and autonomic neuropathy are reviewed. The controversy surrounding impaired glucose tolerance as an etiological factor is dicusssed. We discuss the available medications and outline a rational approach to treatment.

Myotonic dystrophy type 1 affects multiple organ systems and is associated with cardiac, endocrine, and ophthalmological disorders. Executive dysfunction can lead to missed appointments and an apathetic attitude, causing patients to underestimate symptoms. The findings of both clinical and electrical myotonia should suggest myotonia congenita, DM1, or DM2. Myotonic dystrophy demonstrates anticipation, where the disease has an earlier onset and more severe course with subsequent generations due to increasing trinucleotide repeats. Therefore, the diagnosis can be heralded in an adult by his/her affected child. Management of the disorder is supportive, addressing issues relating to loss of strength, cardiac conduction defects, endocrinopathies (regular monitoring with glycosylated hemoglobin and thyroid-stimulating hormone). Diagnosis and management of this disorder is discussed.

The distinctive, but somewhat odd, development of intermittent “attacks” of weakness/stiffness define this disorder. Interview of the patient to determine the impact of diet (including timing, content and relationship of attack to fasting), activity, emotional stress, alcohol intake, cold exposure, time of day and physiologic perturbations (eg – electrolytes, infection, dehydration, etc) help to confirm and categorize the periodic paralysis. Periodic paralyses are most often associated with a genetic channelopathy. However, they can also occur as a result of a secondary causes including thyroid disturbance or distal renal tubular acidosis. The clinical picture, laboratory investigation, and treatment of this condition are presented.

Fatigable weakness is the hallmark of myasthenia gravis (MG). It may present with false localizing signs leading to an itinal incorrect diagnosis of a brainstem stroke. MRI scanning of the brain with specific sequences can rule out the diagnosis of stroke. Differential diagnosis of MG may also include also motor neuron disease. Electromyography is very helpful in confirming the diagnosis of motor neuron disease. The two major diseases of the neuromuscular junction are MG and Lambert-Eaton syndrome (LEMS). A table presents the differing characteristics of each. LEMS can be associated with malignancy and MG with thyoma. Laboratory examinations have greatly assisted in differentiating these two conditions. There is specific antibody testing for each condition. Repetitive stimulation and single fiber electromyography also improve diagnostic acumen.

Paraneoplastic peripheral neuropathies are rare but important to consider in the evaluation of subacute peripheral neuropathy. The clinical and electrophysiological pattern as well as antibody evaluation is essential order to identify a specific paraneoplastic neuropathy. A positive paraneoplastic antibody in the cerebral spinal fluid is not required to make the diagnosis, but is helpful to consider if the serum antibodies are negative. This chapter emphasizes the importance of differential diagnosis and work up. Treatment options are described. Immunotherapy is also an important consideration.

Chronic inflammatory demyelinating polyneuropathy (CIPD) typically presents with both proximal and distal weakness, areflexia, and distal sensory findings. Two-thirds of patients have a progressive course over many months to years, however one-third of patients have a relapsing course with partial or complete recovery. It is important to be aware of several systemic disorders which may be associated with CIDP. Immunoglobulin M antibody–producing neuropathies have a monoclonal protein that is usually detected with serum protein electrophoresis, which may mimic CIDP. This chapter emphasizes the importance of differential diagnosis and workup. Treatment options are also described.

A metabolic myopathy may be an overlooked etiology of recurrent exercised-induced weakness. This chapter discusses the clinical features and diagnostic considerations for adult patients with suspected metabolic myopathy. Adult patients with carbohydrate or fatty acid disorders typically have a history since childhood of exercise-induced myalgias, cramps, and fatigue. Episodes of myoglobinuria tend to present later, usually in the second decade. For any unexplained myopathy, especially if there is concern for a metabolic myopathy, there should also be consideration of a mitochondrial disease. Many mitochondrial disorders present with multiple neurological problems, such as deafness, seizures, encephalopathy, ptosis, or ophthalmoplegia. This chapter will discuss the complexities of genetic testing and muscle biopsy for the evaluation of a possible metabolic myopathy.

Adult-onset spinal muscular atrophy (SMA type IV) presents in a manner similar to a myopathy. Normal CK and a neurogenic pattern of denervated changes on electrodiagnostic study suggest the actual diagnosis. The diagnosis of SMA is confirmed by the presence of a homozygous deletion or mutation of the SMN1 gene in most patients. Both the presence of SMN1 deletion/mutation and the effectiveness of rescue from a second copy of the gene, SMN2, determines the age at onset and severity of SMA. Differential includes other genetic conditions affecting motor neurons, postpolio syndrome, monomelic amyotrophy, and motor neuropathies/poly-radiculopathies. The management of SMA is predominantly supportive, but this can significantly impact quality of life.

Syncope in a patient with orthostatic hypotension (OH) may indicate autonomic dysfunction. The definition of OH is presented. Clinical features of parasympathetic and sympathetic function are discussed. The differential of acute autonomic dysfunction includes a number of conditions. An autoimmune etiology may occur autoimmune autonomic ganglionopathy. Serologic testing can assist in this diagnosis. If autoimmune immune modulating therapies may be indicated. Autonomic neuropathy may be a paraneoplastuc syndrome. Autonomic testing can also help with documenting autonomic neuropathy as well as the whether the defects are predominately parasympathetic or sympathetic. Amyloid should be considered as should diabetes but both have a more chronic course. An appropriate tissue biopsy with Congo Red staining can help to confirm the diagnosis of amyloid.

Statin myopathy can occur at anytime during use. This chapter discusses an approach to diagnosis, and emphasizes management considerations, including awareness of statin metabolism by the cytochrome P-450 system. A statin must be discontinued in any patient with evidence of myopathy or myalgias. A muscle biopsy should be done in cases that do not improve clinically or by creatine kinase level. It is important to note that there are cases of apparent statin myopathy which transform into a chronic autoimmune inflammatory myopathy. Antibodies to hydroxymethylglutaryl coenzyme A (HMG-CoA) reductase have recently been identified in the majority of patients with autoimmune statin myopathy and this test may give further weight to treat with immunotherapy.

Muscle cramps are a common complaint. When not associated with weakness, the course is usually benign but can still be frustrating to patients. Diffuse cramping may be indicative of a neuromuscular disorder. Cramps can be associated with a metabolic or endocrine disorder or medications. Normal nerve conduction studies and electromyography are reassuring for a benign cause of cramps. In motor neuron disease cramps are common. There are rare spontaneous activity syndromes associated with cramping. Stiff person syndrome is rare and usually has severe axial rigidity. It can be treated with immune modulating medications. McArdles disease has a prominent symptom of diffuse muscle cramping associated with activity. Unfortunately not always will a specific cause for cramps be discovered. Various treatment modalities are presented.

Vasculitic neuropathy often presents as a mononeuritis multiplex pattern. Ischemic nerve injury can lead to abrupt-onset, painful, and multifocal sensorimotor neuropathy. This chapter emphasizes the diagnostic considerations of vasculitic neuropathy, which includes the significant limitations of serologic markers in non-systemic vasculitic neuropathy. Nerve and muscle biopsy are important investigations to consider to make the diagnosis. Keys to management are also reviewed. It is important to manage systemic vasculitis with a rheumatologist. Nonsystemic vasculitis has a much better prognosis; immunosuppressive treatment is less aggressive, but it is recommended to have a rheumatologist’s input. There is no conclusive evidence on how to treat nonsystemic vasculitis. Mild cases may be treated with steroids alone.

Early in its course, amyotrophic lateral sclerosis (ALS) is mistaken for a number of other neuromuscular problems, including spinal disease, multifocal motor neuropathy, and even carpal tunnel syndrome (CTS) when the weakness is distal and focal. In our patient CTS or cervical spine disease was considered. MRI scan of the appropriate spinal level is important to rule out spinal disease. Nerve conduction studies (NCS) and electromyography (EMG) help to exclude other possibilities and point to the diagnosis of ALS. Later in the clinical course, the clinical picture is pathognomonic with upper and lower motor neuron signs. The differential diagnosis of focal weakness is discussed, as is recognition of the more typical ALS clinical syndrome and familial ALS. NCS and EMG findings in ALS are discussed.

In myasthenia gravis, weakness and respiratory insufficiency can occur quickly. It is important for the treating physician to recognize this and institute treatment rapidly. Increasing weakness of the neck may herald impending respiratory insufficiency. The single breath count is an easy way ti assess ventilatory function. Because of bulbar weakness and increasing secretions usually bi-level positive pressure airway pressure is used with extreme caution. Intubation with effective management if the airway is preferred. Differentiation of myasthenic crisis from cholinergic crisis is explained; although cholinergic crisis is relatively uncommon. Treatment modalities can include intravenous immunoglobulin, plasma exchange, and corticosteroids. Corticosteroids should be used with caution since they may exacerbate myasthenic symptoms. Treatment with a steroid sparing agent is discussed. A table is presented which lists signs and symptoms that can suggest the need for intubation.

Slow progression of proximal weakness in an adult may suggest Becker muscular dystrophy, but limb-girdle muscular dystrophy needs to be considered. This chapter discusses a diagnostic approach that includes the utility of ever-evolving genetic tests. Electromyography is helpful in cases of suspected muscular dystrophy without a family history, if creatine kinase levels are low, or in evaluation of possible female carriers for BMD who are symptomatic. Management considerations are also outlined. A muscular dystrophy clinic with a multidisciplinary approach is helpful for coordination of care. A consult from a clinical geneticist is often very helpful.

Management of Guillain-Barré syndrome involves several factors. Pulmonary function tests are important to monitor. Telemetry and blood pressure must be monitored because of the significant risk of autonomic dysfunction. Intravenous immunoglobulin is the immunotherapy of choice for this disease, as it is less complicated to administer than plasma exchange. Supportive management issues to address include deep vein thrombosis prophylaxis, bowel/bladder care as ileus and urinary retention may be develop secondary to dysautonomia, physical/occupational/speech therapy consults, nutrition, and pain control. Gabapentin can also be helpful for GBS-related pain. This chapter discusses an approach to treatment that includes the role of respiratory and autonomic monitoring as well as immunotherapy considerations.