Paraneoplastic Neurologic Disorders

[mkur]

I saw this article and was wondering if PANDAS is a group 2 paraneoplastic neurologic disorder. Another new medical word for me.

 

CURRENT TREATMENT OPTIONS IN NEUROLOGY

Volume 12, Number 3, 212-230, DOI: 10.1007/s11940-010-0066-9

NEUROIMMUNOLOGY

Treatment of Paraneoplastic Neurologic Disorders

John E. Greenlee

 

 

Abstract

Paraneoplastic neurologic disorders are rare, autoimmune disorders, which can be broken down into two groups: those in which antibody response is directed against intracellular neuronal or neuroglial proteins (Group 1) and those in which the immune response is directed against antigens within or subjacent to the neuronal cell membrane (Group 2). In both groups, detection and treatment of the underlying neoplasm is critical and carries the best chance of clinical stabilization or remission.

Syndromes in Group 2 frequently respond to therapy. This may involve corticosteroids, plasma exchange (PE), or intravenous immunoglobulin G (IgG), depending on the specific paraneoplastic syndrome. Cyclophosphamide or rituximab may be helpful in patients who fail to stabilize or improve on less aggressive therapies.

Treatment of syndromes in Group 1 is far more difficult, and proven treatment strategies do not exist. Younger men (< 40 years of age) with limbic or brainstem syndromes, testicular or germ cell tumors, and anti-Ma2 antibodies may respond to specific tumor treatment together with immunotherapy. Patients with paraneoplastic syndromes and anti-Ri antibodies may respond to corticosteroids and/or cyclophosphamide. Evidence-based treatment guidelines do not exist for patients with other central paraneoplastic syndromes such as cerebellar degeneration or encephalomyeloneuritis. Approaches to therapy, apart from treating the underlying tumor, are thus speculative.

In patients with rapidly progressive symptoms classically suggestive of a paraneoplastic neurologic syndrome, time is of the essence in arresting neurologic deterioration. Clinical improvement in patients with longstanding symptoms is unlikely. At the outset, one should move rapidly to define the antibody response involved, as this may also assist tumor diagnosis. Treatment may include prednisone, intravenous IgG, and cyclophosphamide; rituximab plus prednisone may be an alternative, either initially or in the face of continued disease progression despite treatment with intravenous IgG or cyclophosphamide. Although PE is of questionable benefit, a single cycle of PE may be considered before other treatment, to achieve rapid lowering of circulating paraneoplastic autoantibodies.

[PowPow]

Paraneoplastic by definition means that there is a cancer in the body.

I think the description of treatments fits many PANDAS cases, though, certainly. I imagine that there is an overlap of kids who are have PANS and may really have a paraneoplastic syndrome. Maybe the sickest kids on here, who get better and relapse, have more going on than meets the eye.

In fact, when one of my daughters' had pex, I spent some time with a mom of a little boy who was being treated for a recurrence of cancer. He had a PANS episode, right as his cancer recurred, after a febrile illness. They determined it was not strep-caused. They could not find a direct cause, after testing for anti-NMDA-R and other known types of encephalitis. He was treated with IVIG or pex, I cannot remember- as we talked at length about both. His PANS symptoms resolved and now that sweet family is fighting something tougher than PANS.

Thanks for mentioning this to the forum.

I think it is something that parents should keep in mind as a possible diagnosis for kids presenting with severe PANS.

[rjayne]

I have discussed in a few posts on this forum the paraneoplastic neurological disease that my daughter has. It's called opsoclonus myoclonus syndrome and the behavioral symptoms of the disease are nearly identical to what I have read parents here describing. Interestingly enough, not all cases of OMS are caused by neuroblastoma, there are cases that have been caused by gluten intolerance, swine flu, and strep to name a few.

Rebecca

[PowPow]

How was your daughert diagnosed with oms without a neuroblastoma? Not questioning, just wondering ,as I feel I am dealing with more than pandas . the mom I met whose son had OMA from neuroblastoma asked if my child had ben checked for cancer. no one has- how would they know? Thanks for any info.

[rjayne]

My daughter did have neuroblastoma but was it missed for a few years, so the diagnosis was made w/o the cancer finding. The diagnosis is based on symptoms alone, the presence of ataxia, myoclonus, and opsoclonus. She also had high b cell and slightly elevated t cell population in her CSF. Also, she went from perfectly normal to jerking, non-verbal, not walking, not sitting, eyes darting and screaming all the time in about three days-very acute onset.

Is your child exhibiting ataxia, myoclonus, or opsoclonus?

[PowPow]

I will PM you. thanks so much for sharing about this