0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Images in Neurology |

Primary Brain Amyloidoma Long-term Follow-up

Dimitri Renard, MD; Chantal Campello, MD; Valerie Rigau, MD; Nicolas de Champfleur, MD; Pierre Labauge, MD, PhD
Arch Neurol. 2008;65(7):979-980. doi:10.1001/archneur.65.7.979.
Text Size: A A A
Published online

Extract

A 39-year-old woman presented with generalized seizures. Clinical examination findings were normal. Brain computed tomography showed 2 hyperdense subcortical lesions in the right temporal and frontal lobes. Enhancement was seen in the temporal lesion after contrast injection. Magnetic resonance imaging patterns were as follows: the temporal lesion was slightly hypointense on the T1-weighted sequence, clearly enhanced by gadolinium administration (Figure 1A), and hypointense surrounded by a hyperintense signal on the T2-weighted sequence; the frontal lesion was slightly hypointense on the T1-weighted sequence, enhanced after gadolinium injection (Figure 1A), and mixed isointense/hyperintense on the T2-weighted sequence. Findings from the cerebral spinal fluid examination, cerebral angiography, mammography, and thoracic, abdominal, and pelvic computed tomography were normal. A multifocal brain tumor was suspected. Carbamazepine therapy was started. Four years later, the signal characteristics of the frontal lesion had changed and it was seen as a central hypointensity surrounded by a hyperintense signal on the T2-weighted sequence (Figure 1B). A new lesion close to the Monro foramen was visualized only after gadolinium injection (Figure 1C). A frontal lesion biopsy was noncontributive. Another 4 years later, depression and minor cognitive impairment occurred. All 3 lesions showed hyperintense components on unenhanced T1-weighted sequences and increased size after gadolinium injection (Figure 1D). A new biopsy of the temporal lesion found extracellular deposits of amorphous acidophil eosinophilic material, staining positive with Congo red (Figure 2), with apple-green birefringence under polarized light, signaling amyloid deposits. Immunohistochemical stains showed λ light-chain predominance. Repeated immunoelectrophoresis, echocardiography, electromyography, and multiple biopsies (salivary gland, skin, nerve, muscle, bone marrow, rectosigmoid, stomach, and duodenum) did not reveal any systemic amyloidosis. Primary brain amyloidoma was diagnosed. Seventeen years after onset, clinical evaluation findings remained unchanged and all the lesions showed a further increase in size on gadolinium-enhanced T1-weighted sequences.

Figures in this Article

Topics

brain

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

First Page Preview

View Large
First page PDF preview

Figures

Place holder to copy figure label and caption
Figure 1.

Magnetic resonance imaging patterns: the initial T1-weighted sequence showed gadolinium enhancement of the temporal (arrowhead) and frontal (arrows) lesions (A); 4 years later, on the T2-weighted sequence, both lesions were seen as a central hypointensity (black arrowheads) surrounded by a hyperintense signal (white arrowheads) (B); a new lesion close to the Monro foramen (arrow) was visualized on the gadolinium-enhanced T1-weighted sequence only (C); and another 4 years later, the gadolinium-enhanced T1-weighted sequence showed an increase in size of all 3 lesions (arrowheads) (D).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

Histopathologic examination of the temporal lesion revealed amyloid deposition (arrows) (A), with λ light-chain predominance (arrowheads) on immunohistochemical stains (B).

Graphic Jump Location

Tables

References

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.

Multimedia

Some tools below are only available to our subscribers or users with an online account.

174 Views
4 Citations
×

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles
Jobs
JAMAevidence.com

Users' Guides to the Medical Literature: A Manual for Evidence-Based Clinical Practice, 3rd ed
Clarifying Your Question

Users' Guides to the Medical Literature: A Manual for Evidence-Based Clinical Practice, 3rd ed
Three Examples of Question Clarification

brightcove.createExperiences();