Neuromuscular and Other Neurologic Emergencies

10 Neuromuscular and Other Neurologic Emergencies


Danielle Wilhour and Alison L. Walsh


Abstract


Prompt recognition, diagnosis, and treatment of neuromuscular conditions in the critical care setting are crucial as specific therapies and supportive care can prevent mortality and mitigate morbidity. Neuromuscular disorders are diseases that affect the peripheral nervous system from the anterior horn cells, peripheral nerves, and neuromuscular junction to the muscles. Many of these patients present with rapidly advancing or severe weakness which can lead to respiratory failure. Possessing the acumen to quickly and accurately identify neuromuscular emergencies is therefore essential. This chapter will discuss high-yield information on identification and management of various neuromuscular disorders.


Keywords: Guillain-Barré syndrome, acute inflammatory demyelinating polyradiculoneuropathy, myasthenia gravis, botulism, organophosphate toxicity, neuromuscular junction, neuroleptic malignant syndrome, serotonin syndrome


10.1 Guillain-Barré Syndrome (GBS)/Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)


10.1.1 Definition


Acute immune-mediated polyradiculoneuropathy characterized by flaccid ascending weakness with areflexia, and, when severe, neuromuscular respiratory failure.1,2,3,4


Monophasic course with symptom onset to nadir of weakness < 4 weeks.3


Most often caused by demyelination and occasionally caused by axonal type.4


10.1.2 Epidemiology


Worldwide incidence of 1 to 2 per 100,000 per year.3


Median age is 53 years old with a ratio of men to women of 1.78.2,3


Mortality ranges between 3 and 13% with higher mortality in patients who require mechanical ventilation.7


Respiratory failure, pneumonia, autonomic dysautonomia, and cardiac arrest are the most common causes of death in these patients.7


10.1.3 Differential Diagnosis


Bilateral strokes, posterior fossa structural lesion, transverse myelitis, compressive myelopathy, anterior spinal artery syndrome, poliomyelitis, acute infectious myelitis (West Nile virus, coxsackie, echovirus), Lyme disease, botulism, myasthenia gravis (MG), neuromuscular blocking agents, acute viral myositis, acute inflammatory or metabolic myopathies, periodic paralysis, and psychogenic symptoms


10.1.4 Common Clinical Presentation


Two-thirds of cases are preceded by respiratory infection or gastroenteritis days to weeks before (most commonly Campylobacter jejuni, cytomegalovirus [CMV], Epstein-Barr virus [EBV], varicella-zoster virus [VZV], and Mycoplasma pneumoniae).3,4


Initial symptoms: Numbness, paresthesias, weakness, dysautonomia, and pain in limbs.1,3


Cardinal features: Progressive, bilateral, and symmetric ascending weakness of the limbs with hypo/areflexia which progresses over days to weeks.3,4


Dysautonomia occurs in 70% of patients.4 Dysautonomia is characterized by wide fluctuations in blood pressure and respiratory rate, tachyarrhythmias, bradyarrhythmias, urine retention, diaphoresis, and gastric slowing causing ileus.


10.1.5 Diagnosis


See Table 10.1.


10.1.6 GBS Variants


GBS/AIDP: Areflexia, mild sensory changes, distal paresthesias, loss of deep tendon reflexes (DTR), ascending paralysis, respiratory failure, and autonomic dysautonomia


Acute motor axonal neuropathy (AMAN): Acute, flaccid ascending paralysis associated with GM1 and GD1a ganglioside antibodies


Acute motor and sensory axonal neuropathy (AMSAN): Loss of DTR, distal weakness, and sensory symptoms (GM1 and GD1a antibodies)


Miller-Fisher syndrome (MFS): Ophthalmoplegia, ataxia, areflexia (GQ1b and GT1a antibodies)


Table 10.1 Diagnostic criteria for GBS/AIDP2



















Required features


Supportive features


Unlikely GBS


Progressive weakness of > 1 limb, the trunk, bulbar and facial muscles, or ophthalmoplegia


Hyporeflexia or areflexia


Symptom progression over days to 4 weeks


Relative symmetry


Mild sensory symptoms or signs


Cranial nerve involvement


Recovery 2–4 weeks after plateau


Autonomic dysfunction


No fever at the onset


CSF has ↑ protein with WBC < 10/mm3


GBS electrodiagnostic abnormalities


Sensory level


> 50 WBC in CSF


Asymmetry of weakness


Severe and persistent bowel and bladder dysfunction


Abbreviations: AIDP, acute inflammatory demyelinating polyradiculoneuropathy; CSF, cerebrospinal fluid; GBS, Guillain-Barré syndrome; WBC, white blood cell.


Bickerstaff encephalitis: Encephalopathy, ophthalmoplegia, and ataxia with areflexia to hyperreflexia (GQ1b and GT1a antibodies)


10.1.7 Ancillary Testing


Autoimmune and infectious workup (can be presenting sign of human immunodeficiency virus [HIV])


Serum ganglioside antibodies (GM1, GD1a, GQ1b, and GT1a) when considering a GBS variant


Lumbar puncture can rule out infectious diseases or malignancy.3,4


Albuminocytologic dissociation (elevated cerebrospinal fluid [CSF] protein with normal CSF white blood cell [WBC]) seen in 50% of patients’ CSF in the first week and proportion increases with time.3,4


Nerve-conduction studies (NCS) help confirm the presence, pattern, and severity of neuropathy.


Prolonged F-wave latencies, prolonged distal motor latencies, temporal dispersion, conduction block, and slow motor nerve conduction velocities (typically in demyelinating range).1


Note that NCS are usually not performed immediately and can be delayed by up to 2 weeks.


10.1.8 Complications of GBS


Cardiac: Labile blood pressure (70%), hypertension, hypotension, arrhythmias, tachycardia, bradycardia (4%), atrioventricular (AV) blocks, and asystole


Pulmonary: Respiratory failure, pneumonia, aspiration, atelectasis, and mucus plugging


Ventilator support is required by 20 to 30% of patients.5


Gastrointestinal: Gastroparesis and adynamic ileus


Genitourinary: Bladder dysfunction, retention, and incontinence


Endocrine: Syndrome of inappropriate antidiuretic hormone secretion (SIADH) (monitor sodium and fluid status)


Hematologic: Venous thromboembolism and pulmonary embolism


Neuropathic pain (40–50%): Use gabapentin or carbamazepine in acute phase.


10.1.9 Management


Monitor respiratory function closely. Intubation may be required if vital capacity (VC) is < 20 mL/kg and maximum negative inspiratory force (NIF) is < 30 cm H2O (or a reduction of 50% from baseline VC or NIF).1


Treatment with intravenous immunoglobulin (IVIG) or plasma exchange hastens recovery from GBS if initiated within 4 weeks of the onset of symptoms. Combining these two treatments is not beneficial.1,6 See Table 10.2.


Corticosteroid treatment is not effective and therefore not recommended.1,6


10.1.10 Prognosis


At 1 year


84% of patients will be able to walk.


5 to 10% of patients have incomplete recovery.



4 to 5% of patients with GBS die despite intensive care.1,7


Relapses can occur in approximately 10% of patients.


10.2 Myasthenia Gravis


10.2.1 Definition


Autoimmune disorder affecting postsynaptic neuromuscular transmission at the neuromuscular junction (NMJ), producing characteristic variable and fatigable weakness in skeletal muscles.8,9,10,11


Myasthenic crisis is a complication of MG with worsening muscle weakness, resulting in respiratory failure that often requires intubation and mechanical ventilation.11,12,13


10.2.2 Epidemiology


Prevalence is about 20 per 100,000.10


Bimodal distribution with age of onset: Early peak in the 2nd and 3rd decades (female predominance) and late peak in the 6th to 8th decades (male predominance).14


The median time from onset of MG to first myasthenic crisis is 8 to 12 months. However, myasthenic crisis may be the initial presentation of MG in 20% of patients.11


10.2.3 Differential Diagnosis


Lambert-Eaton myasthenic syndrome, GBS, organophosphate toxicity, botulism, congenital myasthenia syndromes, thyroid ophthalmopathy, mitochondrial disorders, myotonic dystrophy, skull-based tumors, and motor neuron disease.


10.2.4 Clinical Presentation of Generalized Myasthenia Gravis


Presents with several days/weeks of worsening ptosis, diplopia, bulbar symptoms, extremity weakness, and/or shortness of breath.9


Physical examination findings: Ptosis, inability to sustain upgaze, normal pupillary reflexes, bulbar weakness, flaccid dysarthria, neck flexor/extensor weakness, proximal > distal limb weakness, tachypnea, intact sensation, and decreased reflexes.10


10.2.5 Diagnosis


Diagnosis is based on history and physical examination and supported by electrophysiologic and serologic studies.


Electrophysiologic studies


Repetitive nerve stimulation: Low rates of repetitive stimulation (2–5 Hz) deplete acetylcholine and cause > 10% decrement in compound muscle action potential (CMAP) amplitude (80% sensitive for generalized MG).9,10


Single fiber electromyography (EMG): Increased jitter or variation in contraction time between pairs of muscle fibers (95% sensitive, nonspecific).9,10


Serologic antibody tests are positive in > 90% of MG patients.


Acetylcholine receptor (AChR) antibodies


Present in 85% of patients with generalized MG9,10


Highly specific for MG (> 99%)


Correlate with thymic hyperplasia and thymoma


Muscle-specific kinase (MuSK) antibodies: Found in 40% of patients with generalized MG who are negative for AChR antibodies.9


Low-density lipoprotein receptor-related protein 4 (LRP4) antibodies: Found in 18% of patients with generalized MG who are negative for AChR and MuSK.9


Ancillary tests: CT of chest with contrast to evaluate for thymoma


Myasthenic Crisis

Precipitating factors: Infection (38%), medication changes (particularly initiation and withdrawal of corticosteroids), aspiration pneumonitis, upper airway obstruction, pregnancy, surgery, and disease progression.11,12


For one-third of the patients, no precipitating factor can be found.


Cholinergic Crisis

Increase in anticholinergic medications that can lead to weakness associated with signs of increased cholinergic activity (miosis, salivation, lacrimation, bradycardia, diarrhea).11


10.2.6 Management of Myasthenic Crisis


Supportive care: Aspiration precautions, temporary nil per os (NPO) with nasogastric tube feedings, frequent monitoring with NIF and VC, and ventilatory support. ABGs are insensitive.


Treatment typically consists of high-dose steroids with either IVIG or plasma exchange. See Table 10.3.


Aug 7, 2022 | Posted by in NEUROSURGERY | Comments Off on Neuromuscular and Other Neurologic Emergencies

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