paraneoplastic neurological syndromes - academia.cat · paraneoplastic neurological syndromes...
TRANSCRIPT
![Page 1: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/1.jpg)
Paraneoplastic Neurological Syndromes
Neurological syndromes of unknown cause that often
associate with cancer
Immune-mediated?
![Page 2: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/2.jpg)
Onconeural antibodies
•Hu (ANNA1)
•CV2 (CRMP5)
•Amphiphysin
•Yo (PCA1)
•Ri (ANNA2)
•Ma2
• Tr
Lung
Breast, ovary
testis
Hodgkin
![Page 3: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/3.jpg)
Classical syndromes• Encephalomyelitis• Limbic encephalitis• Subacute cerebellar degeneration• Opsoclonus-myoclonus• Subacute sensory neuronopathy• Gastrointestinal pseudoobstruction• Lambert-Eaton myasthenic syndrome• Dermatomyositis
![Page 4: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/4.jpg)
A 61 year-old, non smoker, woman presented with a 2 week history of vertigo, diplopia, and gait instability.
Unable to walk or to stand alone in the last 48 hours
![Page 5: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/5.jpg)
![Page 6: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/6.jpg)
Detection of onconeural (Hu, Yo, Ri, Ta, Tr, CV2, amphiphysin) antibodies: negative
CSF: Normal;
Brain MR: Normal
Breast Cancer
![Page 7: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/7.jpg)
A 75 year-old man presented with a 6-month history of weight loss (10 Kg) and abnormal movements
No previous medical problems
![Page 8: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/8.jpg)
- May ’05: Admitted to Hospital Clinic
Normal general examination
Cognitive evaluation: Normal
Neuroexam: Normal except video
Blood analysis: Normal
Cranial MR: Normal
![Page 9: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/9.jpg)
![Page 10: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/10.jpg)
Thorax CT scan:
enlarged mediastinal lymph node
Node biopsy: SCLC
Diagnosis: CV2-associated chorea
![Page 11: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/11.jpg)
A 50 year-old man presented with a 13-month history of pruritus in left leg and trunk and progressive muscle rigidity and spasms in the same distribution
Previous medical problems: Type 2 DM
![Page 12: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/12.jpg)
- April ’05:Normal general examination
Neuroexam: Rigidity in both legs L>R (video) and lower trunk.
Blood and CSF analysis, cranial and spinal MR : Normal
Anti-GAD antibodies: negative
EMG:Continuous motor unit discharges simultaneously from opposite muscles
![Page 13: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/13.jpg)
Mediastinoscopy and biopsy showed normal lymph node
Patient made an important improvement of rigidity with oral steroids and IVIG
September ’05: severe pruritus involving left leg and trunk. More rigidity in left leg. Development of myoclonus (video) and rigidity in right arm.
![Page 14: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/14.jpg)
![Page 15: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/15.jpg)
A new thorax CT: enlarged lymph nodes in mediastinum
Biopsy: SCLC
Diagnosis:
Paraneoplastic SMS and spinal myoclonus
Amphiphysin antibody: positive
![Page 16: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/16.jpg)
A 76 year-old man presented with a 1 weekhistory of progressive confusion and severe memory deficit
![Page 17: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/17.jpg)
-August’04: Admitted to Hospital Clinic
Normal general examination
Neuroexam: Disoriented,severe short-term memory deficit with confabulations.
Detection of onconeural (Hu, Yo, Ri, Ta, CV2, amphiphysin) and VGKC antibodies: negative
CSF: Normal; Brain MR: Normal
![Page 18: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/18.jpg)
![Page 19: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/19.jpg)
General autopsy: SCLC CNS: neuronal loss in hippocampus with inflammatory infiltrates. Study of herpes virus: negative
Diagnosis:
Seronegative limbic encephalitis
![Page 20: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/20.jpg)
Anti-GABAR-B1 LCR Co-localización
![Page 21: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/21.jpg)
Antibodies against neuronal surface antigens
• Probably pathogenic
•Markers of the neurological syndrome
•Frequency of cancer variable
• Good response to immunotherapy
![Page 22: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/22.jpg)
Antibodies against neuronal surface antigens
Ab Syndrome % paraneo DetectionVGCC LEMS/PCD 50 RIAmGluR1 PCD 0? CBAVGKC LE 5 RIANMDAR Encephalitis 30 CBAAMPAR LE 70 CBAGABAR LE 47 CBA
LEMS: Lambert-Eaton; LE: limbic encephalitis; PCD: Paraneoplastic cerebellar degeneration. RIA: radioimmunoassay; CBA: “cell based assay”
![Page 23: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/23.jpg)
A 68 year-old man presented with a 2-month history of progressive gait instability and mild memory deficit
No previous medical problems
![Page 24: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/24.jpg)
- May ’05: Admitted to Hospital Clinic
Normal general examination
Cognitive evaluation: mild short-term memory deficit
Neuroexam: Normal except gait (video)
Blood analysis: Normal
Cranial MR: Questionable LE
![Page 25: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/25.jpg)
![Page 26: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/26.jpg)
Highest-level gait disorders
• Cautious gait• Subcortical disequilibrium• Frontal disequilibrium• Isolated gait ignition failure• Frontal gait disorder
Nutt et al. Neurology 1993;43:268-79
![Page 27: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/27.jpg)
PET PET-CT
![Page 28: Paraneoplastic Neurological Syndromes - academia.cat · Paraneoplastic Neurological Syndromes Neurological syndromes of unknown cause that often associate with cancer. ... Subacute](https://reader031.vdocuments.mx/reader031/viewer/2022012923/5af333037f8b9a8b4c9129ed/html5/thumbnails/28.jpg)