Paraneoplastic Neurological Syndromes



Fig. 27.1
Paraneoplastic limbic encephalitis. Coronal Flair images. (a, b) demonstrate first acute phase with swelling and hyperintensity in right insular and mesial temporal lobes; after 3 months, (c) atrophy is clearly observed. In (d, e) a second acute phase with swelling and hyperintensity is seen in the left temporal mesial and partially in the insular lobes. After 3 months, (f) bilateral marked hippocampal atrophy is present





EEG

Non-specific slow disorganized or epileptic activity.


Electromyography (EMG)

Non-length dependent pattern (SSN); decremental response to repetitive nerve stimulation (myasthenia) and post-tetanic potentiation of CMAP (LEMS), peripheral hyperexcitability with fibrillation, and fasciculations (neuromyotonia).


Antibody Screening

The antibodies are associated with clinical syndromes and tumors according to a number of common patterns (see Tables 27.1 and 27.2).


Table 27.1
Onconeural antibodies: association with tumors and PNS




















































Antibody
Most commonly associated tumors
Syndrome
Antibody positivity without a tumor (%)
Frequency in tumor without PNS (%)

Anti-Hu
SCLC
Encephalomyelitis

Limbic encephalitis

PCD

Brainstem encephalitis

Intestinal pseudo-obstruction
2
16

Anti-CV2
SCLC, thymoma
Encephalomyelitis

Chorea

PCD

Limbic encephalitis
4
9

Anti-amphiphysin
Breast, SCLC
SPS

Myelopathy

PEM
5
1

Anti-Ri
Breast, SCLC
Brainstem encephalitis

Opsoclonus-Myoclonus
3
4

Anti-Yo
Ovary, breast
PCD
2
1

Anti-Ma2
Testicle
Limbic encephalitis

Brainstem encephalitis
4
0



Table 27.2
Antibodies against surface antigens: most common association with tumors and syndromes




































Antibody
Syndromes
Most commonly associated tumors
Frequency of paraneoplastic cases

Anti-LGI1
Limbic encephalitis
SCLC, thymoma
Rare

Anti-Caspr2
Limbic encephalitis, neuromyotonia, Morvan’s syndrome
Thymoma
40 %

Anti-NMDAR
Encephalitis
Ovarian teratoma
6 % in children; 50 % in adults

Anti-AMPAR
Limbic encephalitis
SCLC, breast, thymoma
70 %

Anti-GABAR

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Nov 10, 2016 | Posted by in NEUROLOGY | Comments Off on Paraneoplastic Neurological Syndromes

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