Manifestations of CNS Lymphoma
CNS lymphoma most commonly presents with focal neurological deficits (70% of cases), followed by neuropsychiatric symptoms or personality changes (43%), and signs of increased intracranial pressure (33%). 1
Neurological Manifestations
Focal Neurological Deficits (70% of cases)
- Hemiparesis (weakness on one side of the body) is the most common focal deficit 1, 2
- Ataxia and coordination problems occur frequently, particularly with cerebellar involvement 1, 2
- Sensory deficits develop when thalamus or basal ganglia are affected (16% of cases) 1, 2
- Movement disorders can occur with deep gray matter involvement 2
Neuropsychiatric and Cognitive Symptoms (43% of cases)
- Personality changes and behavioral alterations are prominent early manifestations 1, 2
- Cognitive dysfunction including confusion, memory deficits (both verbal and non-verbal), and executive function impairment 1, 2
- Reduced processing speed and attention problems with decreased concentration 2
- Working memory deficits affecting the ability to hold information temporarily 2
- Lethargy and altered mental status 1
Signs of Increased Intracranial Pressure (33% of cases)
- Headache that is persistent and progressive 1, 2
- Nausea and vomiting related to elevated intracranial pressure 1
- These symptoms can be rapidly alleviated by CSF drainage when due to circulation disturbances 1
Seizures (14% of cases)
- Seizures occur relatively rarely compared to other brain tumors 1, 2
- New-onset seizures in adults should prompt urgent evaluation for CNS lymphoma 2
Location-Specific Manifestations
Supratentorial Lesions
- Frontal lobe and brain hemispheres (38% of cases): personality changes, cognitive decline, motor deficits 1, 2
- Thalamus or basal ganglia (16%): sensory deficits and movement disorders 1, 2
- Corpus callosum (14%): disconnection syndromes and cognitive issues 1, 2
- Periventricular regions (12%): hydrocephalus symptoms 1, 2
Infratentorial and Other Sites
- Cerebellum (9%): ataxia, coordination problems, dizziness 1, 2
- Meninges (16%): headache, neck stiffness, cranial nerve palsies 1, 2
- Spinal cord (1%): radicular signs including weakness, voiding problems, cauda equina syndrome 1
- Cranial and spinal nerves (<1%): focal or irradiating neck and back pain 1
Cranial Nerve Manifestations
- Visual disturbances from cranial nerve II involvement or ocular lymphoma 1, 2
- Diplopia from cranial nerves III, IV, or VI palsies 1, 2
- Hearing loss from cranial nerve VIII involvement, particularly with meningeal disease 1, 2
- Facial nerve palsies can occur with meningeal involvement 2
Ocular Manifestations
- Blurred vision is the most common ocular symptom 1
- Floaters are frequently reported 1
- Ophthalmological evaluation by slit-lamp fundoscopy is necessary in all patients to exclude intraocular involvement 3
Critical Clinical Considerations
Temporal Pattern
- Symptoms develop gradually over weeks to months, often leading to delayed diagnosis 1, 2
- A delay in diagnosis of weeks to months following symptom onset is common 1
Red Flags Requiring Urgent Evaluation
- Progressive cognitive decline without clear etiology 2
- Multiple focal neurological deficits developing over a short time period 2
- New-onset seizures in adults 2
Important Pitfalls to Avoid
- Distinguish LM-related symptoms from those due to parenchymal metastases, extracranial disease, treatment side-effects, or non-cancer comorbidities 1
- Bladder, sexual, and bowel dysfunction are possibly underreported and should be actively explored at presentation 1
- Corticosteroids cause rapid tumor regression, which can confound diagnosis if administered before biopsy 1, 3