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Key Features

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  • Can occur as a result of a lesion anywhere along the neuroaxis; the key to diagnosis is localizing the lesion

  • Associated changes in reflexes, sensory changes, abnormal reflexes such as a positive Babinski sign, and bowel and bladder changes can help in localizing the lesion

  • Common causes

    • Mass lesions

    • Infectious; postinfectious

    • Toxins (eg, from a tick or due to botulism)

    • Metabolic

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Clinical Findings

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  • Features assisting diagnosis are age, a history of preceding illness, rapidity of progression, cranial nerve findings, bowel and bladder changes, and sensory findings

  • Increased reflexes and upgoing toes suggests a CNS lesion

  • Fatigability in sucking on a bottle and constipation may be seen in infants with botulism

  • Ascending paresthesia and loss of reflexes before development of overt weakness may be present in patients with Guillain-Barre syndrome (also known as acute inflammatory demyelinating polyneuropathy [AIDP])

  • Ophthalmoplegia, ataxia, and loss of reflexes is classic constellation of symptoms in patients with the Miller Fisher variant of Guillain-Barre syndrome

  • Back pain is suggestive of a spinal cord lesion, such as in transverse myelitis or a spinal cord mass

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Diagnosis

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  • MRI

    • Helpful when a spinal cord or brain lesion is suspected

    • Essential when mass lesion is suspected

  • Once a mass lesion is excluded, cerebrospinal fluid (CSF) studies, including opening pressure, can be obtained

  • Viral cultures (CSF, throat, and stool) and titers aid in diagnosing poliomyelitis

  • A high sedimentation rate may suggest tumor, abscess, or autoimmune disorder

  • EMG and nerve conduction studies (NCSs)

    • Can be helpful in diagnosing Guillain-Barre syndrome

    • Fibrillation potentials and increased compound muscle action potential amplitudes with high-frequency stimulation suggest botulism

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Treatment

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  • Remove ticks

  • Administration of botulism immune globulin administration shortens hospital stay

  • IVIg or plasmapheresis for patients with Guillain-Barre syndrome shortens hospital stay

  • Corticosteroids, IVIg, and plasmapheresis have been used in transverse myelitis

  • Supportive treatment

    • Pulmonary toilet

    • Adequate fluids and nutrition

    • Bladder and bowel care

    • Prevention of pressure ulcers

    • Psychiatric support

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