Leptomeningeal Carcinomatosis Clinical Presentation

Updated: Jun 24, 2016
  • Author: Michael J Schneck, MD, MBA; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
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Presentation

History

Meningeal symptoms are the first manifestations in some patients; however, most patients already have widespread and progressive cancer with few therapeutic options left.

A high index of suspicion is necessary, and involvement of multiple anatomic sites in the CNS should raise the suspicion for LC, although multiple metastases are more likely with that presentation.

The symptoms are protean and can include the following:

  • Headaches (usually associated with nausea, vomiting, lightheadedness)
  • Gait difficulties from weakness or ataxia
  • Memory problems
  • Incontinence
  • Sensory abnormalities

Pain and seizures are the most common presenting complaints.

Next:

Physical

Signs generally exceed patient-reported symptoms.

Involvement of the CNS is divided into the following 3 broad anatomical groups:

  • Cerebral involvement results in headache, lethargy, papilledema, behavior changes, and gait disturbance (the latter can be due to either cerebellar or cauda equina involvement). Major dysfunction, such as hemiparesis and hemisensory loss or visual field defects, is rare and more indicative of parenchymal metastasis.
  • Cranial-nerve involvement presents with impaired vision, diplopia (most common), hearing loss, and sensory deficits, including vertigo. Palsies of cranial nerves III, V, and VI are most common; palsy of nerve VII is less common. Isolated VII nerve palsies may occur however, and may be hard to differentiate from idiopathic facial nerve (Bell's) palsies. [30] Solid tumor-derived LC has a higher affinity for the optic and extraocular nerves, while leukemic meningitis preferentially affects the facial nerve. Involvement of multiple cranial nerves is the rule rather than the exception.
  • Spinal-root involvement is caused by either meningeal irritation, presenting with nuchal rigidity (15%) and neck and back pain (rare), or invasion of the spinal roots. The latter can cause leg weakness, radiculopathy (usually lumbar, mimicking a herniated disk), reflex asymmetry or loss (most common, noted in 70% of patients), sphincter incontinence (less common), positive Babinski reflexes, paresthesias, and numbness. Asymptomatic bladder enlargement can occur from spinal cord compression. Spinal-root symptoms are usually followed by cranial-nerve symptoms. Nuchal rigidity, positive results on the straight-leg raising test, and decreased rectal tone are rare.

Over the course of the disease, cranial-nerve deficits are the most frequent signs, occurring in 94% of patients. Although these are seldom the presenting complaint (30% of patients), mild cranial-nerve abnormalities are usually present on physical examination; the abnormalities typically include diplopia, dysphagia, dysarthria, and hearing loss. However, most patients do not have isolated cranial-nerve deficits; rather, they have a combination of cranial-nerve, cerebral, and spinal signs.

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