Paraneoplastic cerebellar degeneration due to Hodgkin's lymphoma

Case contributed by Dr Holly Gilbert

Presentation

Dizzyness, slurred speech, difficulty with pronunciation, bilateral nystagmus, intention tremor and high stepping broad gait. Previous HL.

Patient Data

Age: 45 years
Gender: Female

Axial views reveal that supra tentorially there is normal gyral and sulcal appearance for the patients age with no demonstrable evidence of significant atrophy of the gyrus.

However infra tentorially the cerebellum is symmetrically atrophic. 

The folia are significantly atrophic and shrunken with too much spacing between the folia. Additionally, the inter-fissure distance between all fissures is increased.

These atrophic changes are symmetrical, in keeping with the symmetrical cerebellar signs, and out of keeping for the patients age.

The MRI study reveals the same symmetrical bilateral cerebellar degeneration with normal supra-tentorial appearances.

In addition there is no evidence of any cerebellitis, ischemia or space occupying lesion within the cerebellum to account for the clinical presentation of bilateral cerebellar signs.

Case Discussion

This case demonstrated typical clinical progressive cerebellar symptoms that preceded the diagnosis of relapse Hodgkins disease. CT and MRI demonstrated cerebellar degeneration and lymph node biopsy confirmed relapse Hodgkin's lymphoma. In addition, we did paraneoplastic antibodies and the Hodgkin's specific antibody (anti-Tr) was positive which further confirmed the diagnosis. The patient was treated with chemotherapy and steroids.

Paraneoplastic cerebellar degeneration is associated with multiple cancers such as ovarian, breast, small cell lung cancer as well as Hodgkin's lymphoma1. Paraeneoplastic cerebellar degeneration can present up to 5 years prior to oncology diagnosis. The hallmark is lost of cerebellar Purkinje cells. Brain imaging can often be normal initially with cerebellar atrophy developing over time. Occasionally there may be transient cerebellar enlargement or cortical meningeal enhancement prior to atrophy2. CSF protein is usually high. As there may be diagnostic uncertainty, testing for a wide range of paraneoplastic antibodies in CSF and peripheral blood is useful2. Management relies on the diagnosis and treatment of the underlying cancer although immunomodulation is also used to try and suppress the immune process. In particular, these include steroids, IVIg, cyclophosphamide and rituximab. However, as the condition results in destruction of neurons, prompt reduction of paraneoplastic antibiotics is critical3.

Key Learning Points

  • symptoms can present up to 5 years prior to diagnosis of malignancy
  • CT/MRI imaging maybe normal or subtle initially but results in eventual atrophy
  • paraneoplastic antibody testing can be very helpful in confirming a diagnosis
  • management includes treating the underlying malignancy and immunomodulation.

Case presented with:
Dr Nolan Walker MuDr FRCR

Acknowledgements
Dr Effie Liakopoulou MD, PhD, FRCP (Glasg), FRCPath, CMM
Dr Robert Lown MBBS MD(Res) MRCP FRCPath
Dr William Townsend MB ChB MRCP FRCPath

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