| A 61-year-old man with instability of gait and right hand clumsiness. | |
| | |
MedLine Citation:
|
PMID: 20438489 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
60 year-old man, without relevant medical history, noted a slight and progressive instability of gait for one month and right hand clumsiness. Brain MRI showed a cerebellar lesion, posterior to the middle cerebellar peduncle. This lesion was heterogeneous and hyperintense on FLAIR sequences, isointense on T1-weighted images, and showed gadolinium enhancement. Hematological and biological serum analyses were normal as were plasma and urine immunoelectrophoresis. CSF analysis including protein electrophoresis was unremarkable. CT scans of the abdomen, chest and pelvis were normal as were cervical echography and bone scintigraphy. A yellowish and firm lesion was surgically resected. The patient's recovery was good, with normal total body PET scan and bone marrow biopsy. Pathological study evidenced kappa light chain deposits and kappa-immunopositive mature plasma-cells in the vicinity. The deposits failed to show any birefringence in polarized light microscopy after Congo red staining, and electron microscopy revealed their granular ultrastructure. Light chains are well known for their amyloidogenic properties, but in a few cases, they are non amyloidogenic and may cause tissue deposits histologically similar to amyloid but Congo red-negative and non fibrillary at ultrastructural examination. Occurrence of light chain deposits in the brain is rare and the tumor-like MRI presentation is reminiscent of primary intracerebral amyloidoma presenting as a mass lesion. This is the first report of intracerebral kappa light chain deposits which presumably derived from local synthesis by mature plasma cells. |
| | |
Authors:
|
Anne Vital; Emmanuel Ellie; Hugues Loiseau |
Related Documents
:
|
11606679 - Extensive metabolic and neuropsychological abnormalities associated with discrete infar... 9539329 - A case of central pontine and extrapontine myelinolysis with early hypermetabolism on 1... 15060269 - Cerebellar infarction in adolescent males associated with acute marijuana use. 12445909 - Motor and psycho-cognitive clinical types in adult metachromatic leukodystrophy: genoty... 6538649 - Central pontine myelinolysis: demonstration by nuclear magnetic resonance. 21135879 - Assessment of stone composition in the management of urinary stones. 17676459 - Radio-guided neurosurgery (rgns): early experience with its use in brain tumour surgery. 11587519 - Pseudotumor deltoideus: a previously undescribed anatomic variant at the deltoid insert... 9179229 - Single photon emission computerized tomography increases the sensitivity of indium-111-... |
Publication Detail:
|
Type: Case Reports; Letter |
Journal Detail:
|
Title: Brain pathology (Zurich, Switzerland) Volume: 20 ISSN: 1750-3639 ISO Abbreviation: Brain Pathol. Publication Date: 2010 Jan |
Date Detail:
|
Created Date: 2010-05-04 Completed Date: 2010-07-30 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 9216781 Medline TA: Brain Pathol Country: Switzerland |
Other Details:
|
Languages: eng Pagination: 273-4 Citation Subset: IM |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Cerebellar Diseases
/
pathology Functional Laterality / physiology Gait Disorders, Neurologic / pathology* Hand / physiology Humans Immunoelectrophoresis Immunoglobulin kappa-Chains / metabolism* Magnetic Resonance Imaging Male Microscopy, Electron Middle Aged Positron-Emission Tomography Tegmentum Mesencephali / pathology Tomography, X-Ray Computed |
| Chemical | |
Reg. No./Substance:
|
0/Immunoglobulin kappa-Chains |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: A 6-year-old girl with an extra-axial mass in the middle cranial fossa.
Next Document: Sheep genetic diversity in Bhutan using microsatellite markers.