MyPACS.net: Radiology Teaching Files > Case 3229176

never visited NEUROBLASTOMA WITH OPSOCLONUS-MYOCLONUS SYNDROME
Contributed by: J P.
Patient: 1 year 3 month old female
History: 15 month old female patient was brought in by parents because of ataxia, nystagmus, vomiting, and irritability.
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Fig. 13: I-131 MIBG Scan
Diagnosis: Neuroblastoma with Opsoclonus-myoclonus Syndrome
Discussion:

Differenital:
Nerve Tumor (Neuroblastoma, ganglioneuroma, etc.)
Lympadenopathy
Extramedullary Hematopoesis

Opsoclonus myoclonus is a rare neurological disorder characterized by unsteady gait, intention tremor (rhythmic, involuntary motions of the limbs during voluntary movements), myoclonus (brief, shock-like muscle spasms), and opsoclonus (irregular, rapid, horizontal and vertical eye movements). Other symptoms may include dysphasia (difficulty speaking), dysarthria (poorly articulated speech), mutism (inability to speak), hypotonia (decreased muscle tone), lethargy, irritability, or malaise (a vague feeling of bodily discomfort). Opsoclonus myoclonus may occur in association with tumors or viral infections. A full oncologic evaluation should be undertaken in all children with OMA because the paraneoplastic syndrome may precede the appearance of a systemic neoplasm.

The minimum requirements for staging include:

bullet Bilateral iliac crest bone marrow aspirate and biopsy; core biopsies must contain at least 1 cm of marrow, excluding cartilage, to be considered adequate
bullet Bone radiographs and either technetium radionuclide scan or MIBG scan
bullet Abdominal imaging by CT or MRI scan with three-dimensional tumor measurements
bullet Chest radiograph (anteroposterior [AP] and lateral); chest CT or MRI are necessary only if the chest radiograph is positive or if abdominal mass or lymph node disease extend into the chest
bullet Head CT is not necessary unless clinically indicated

MIBG is a chemical analog of norepinephrine that is selectively concentrated in sympathetic nervous tissues such as neuroblastoma. It can be labeled with radioactive iodine and imaged by scintigraphy. The MIBG scan is both sensitive and specific for neuroblastoma, and is recommended at diagnosis and repeat evaluations of the tumor. Because radioactive iodine is used, the thyroid gland must be protected by the simultaneous administration of non-radioactive iodine (eg, potassium iodide). Despite prophylaxis, thyroid dysfunction becomes a potential complication in up to 64 percent of patients. The addition of thyroxine and methimazole to potassium iodide, and continuation of thyroid protection for four weeks after the last dose of radiolabeled MIBG, may be more effective in preventing thyroid dysfunction.


References:

1. http://www.clevelandclinic.org/health/health-info/docs/1300/1313.asp?index=6110&src=news


2. http://www.utdol.com/application/topic.asp?file=ped_onco/6775&type=A&selectedTitle=1~26

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Additional Details:

Case Number: 3229176Last Updated: 12-14-2005
Anatomy: Spine and Peripheral Nervous System   Pathology: Neoplasm
Modality: CT, Nuc MedExam Date: Access Level: Readable by all users
Keywords: neuroblastoma

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