Nonmetastatic manifestations of malignant disease

Disturbance of neurological function can occur in association with malignancy without evidence of metastases (0.1% of all cancer patients). Brain, spinal cord, peripheral nerve and muscle may be affected, either separately or in combination.

Small cell carcinoma of the lung, gynaecological malignancy /" Encephalitis and lymphoma are the commonest associated disorders. These syndromes are antibody-mediated, the targeted antigen being a —t ___v Cerebellar part of the nervous system. Specific antibodies, (anti-neuronal), ' .

are responsible for certain syndromes. ®

The non-metastatic manifestations of malignancy are rare.

These are not discreet, e.g. neuropathy and myopathy may coexist -» carcinomatous neuromyopathy; encephalitis and myelopathy -» carcinomatous encephalomyelitis.

ENCEPHALITIS (anti-Hu syndrome)

Associated commonly with small cell lung cancer (SCLC)

usually before this becomes clinically manifest.

Pathology

Cingulate __ gyrus

Myelopathy

Cingulate __ gyrus

Uncus

Neuropathy Myopathy

Neoromuscular junction disturbance Myasthenic syndrome

Uncus

Limbic system

Parahippocampal gyrus

The encephalitic process selectively affects the limbic system - with neuronal loss, astrocytic proliferation and perivascular inflammatory changes.

Clinical features

Disturbance in behaviour precedes the development of complex partial (temporal lobe) seizures and memory impairment. Autonomic dysfunction and sensory neuropathy often co-exist. Progression is rapid.

Investigations. Most cases are anti-Hu antibody positive. MRI is normal. EEG may show temporal lobe abnormalities. CSF reveals a mild lymphocytosis with protein elevation.

CEREBELLAR DEGENERATION (anti-Yo syndrome) associated with breast or ovarian carcinoma.

Subacute cerebellar degeneration

Pathology:

Characterised by Purkinje cell loss with some involvement of the dentate nucleus. Brain stem changes also occur.

Clinical features:

The patient presents with a rapidly developing ataxia.

Brain stem involvement results in nystagmus, opsiclonus and vertigo. The course is usually rapid.

Investigations

MRI shows cortical and vermal cerebellar atrophy. CSF is mildly abnormal and anti-Yo antibodies are present in 50% of suspected cases.

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