0909 Case 2

doi:10.1111/j.1750-3639.2009.00349.
x
COM SEPT 2009 CASE 2
A 61-YEAR-OLD MAN WITH INSTABILITY OF GAIT AND RIGHT

HAND CLUMSINESS bpa_349 273..274
Anne Vital, MD, PhD ; Emmanuel Ellie, MD2; Hugues Loiseau, MD3
1
1
CNRS UMR 5227, Victor Segalen-Bordeaux 2 University, Bordeaux, France
2
Neurology Department, Côte Basque General Hospital, Bayonne, France
3
Neurosurgery Department, Bordeaux University Hospital, Bordeaux, France
patient was able to resume his daily hobby of golfing. Total body
CLINICAL HISTORY PET scan using fluorodeoxyglucose was normal, as were motor
A 60 year-old man, without relevant medical history, noted a slight and sensory nerve conduction studies and echocardiography.
and progressive instability of gait for one month, and right hand Serum beta2 microglobulin was within normal limits and bone
clumsiness two weeks before admission. marrow biopsy showed no abnormality. No change in the patient
Initial examination showed wide-based gait, mild dysarthria and condition was noted over a follow-up period of more than one year.
right arm dysmetria. Strength and sensation were normal as was
body temperature. Brain MRI showed a unique cerebellar lesion,
posterior to the middle cerebellar peduncle, near the right dentate
MICROSCOPIC PATHOLOGY
nucleus. The lesion was heterogeneous and hyperintense on FLAIR Microscopic examination on haematoxylin and eosin stained sec-
sequences, isointense on T1-weighted images, and showed gado- tions from three specimens revealed amorphous eosinophilic mate-
linium enhancement (Figure 1). rial, either extra-vascular or thickening vessel walls (Figure 2a). In
Full blood count, erythrocyte sedimentation rate, C-reactive close proximity to the deposited material, the cerebellar paren-
protein level, liver function tests, serum electrolytes, and serum chyma was infiltrated by predominant mature plasma-cells (CD
protein electrophoresis were normal. Plasma and urine immuno- 138 immunopositive; Figure 2b) associated with a few mature T
electrophoresis showed no abnormality. Serologic tests for HIV, lymphocytes (CD3 immunopositive; Figure 2c) and macrophages
Listeria, Legionella, and Lyme disease were negative. CT scans of of the foreign body type (CD68 immunopositive; Figure 2d). The
the abdomen, chest and pelvis were normal as was bone scintigra- weak congophilia of the deposits failed to show any birefringence
phy. Cervical echography was unremarkable and thyroid was in polarized light microscopy. There was a strong immunostaining
normal. CSF analysis showed slightly elevated protein content of the deposited material as well as the plasma-cells with anti-k
(0.72 mg/dl), 5 white cells/mm3, and no oligoclonal banding on light chain (Figure 2e), while anti-l antibodies failed to label them.
CSF protein electrophoresis. The deposits were also immunonegative for transthyretin amyloid,
At surgery, a yellowish and firm lesion associated with abnormal Ab amyloid and AA amyloid. Under UV light, the deposits were
vessels was resected. slightly fluorescent after thioflavine staining, strongly immuno-
The patient’s recovery was good and two months later an iso- fluorescent for k light chain (figure 2f) and negative for l light
lated mild clumsiness of the right hand was noted. However the chain. Electron microscopy revealed the non-fibrillar ultrastructure
of the deposits which presented as finely granular aggregates
(Figure 3).
What is the diagnosis?
Figure 1.
Figure 3. Figure 2.
Brain Pathology 20 (2010) 273–274 273

© 2010 The Authors; Journal Compilation © 2010 International Society of Neuropathology
Correspondence
3. Fischer L, Korfel A, Stoltenburg-Didinger G, Ransco C, Thiel E

DIAGNOSIS (2006) A 19-year-old male with generalized seizures,
Tumor-like deposits of k light chain surrounded by mature kappa unconsciousness and a deviation of gaze. Brain Pathol 16:185–186.
chain-expressing plasma cells. 4. Grassi MP, Clerici F, Perin C, Borella M, Gendarini A, Quattrini A,
Nemni R, Mangoni A (1998) Light chain deposition disease
neuropathy resembling amyloid neuropathy in a multiple myeloma
patient. Ital J Neurol Sci 19:229–233.
DISCUSSION
5. Laeng RH, Altermatt HJ, Scheithauer BW, Zimmermann DR (1998)
Light chains are well known for their amyloidogenic properties, but Amyloidomas of the nervous system. A monoclonal B-cell disorder
in a few cases, they are non amyloidogenic and may cause tissue with monotypic amyloid light chain l amyloid production. Cancer
deposits histologically similar to amyloid but Congo red-negative 82:362–374.
and non fibrillary at ultrastructural examination. Non amyloid light 6. McMillion L, Melton DM, Erickson JC (2008) Teaching neuroimage:
Primary cerebral amyloidoma mimicking CNS neoplasm. Neurology
chain deposits have been reported as a complication of plasma cell
71:e68.
dyscrasia and particularly of multiple myeloma and the condition 7. Popovic M, Tavcar R, Glavac D, Volavsek M, Pirtosek Z, Vizjack A
has been designated light chain deposition (LCD) disease (9). The (2007) Light chain deposition disease restricted to the brain: the first
kidney is usually the first and most severely affected organ but case report. Hum Pathol 38:179–184.
others may be involved, including the peripheral nervous system 8. Ragel BT, Blumenthal DT, Browd SR, Salzman KL, Jensen RL
(4) and ocular globes (1). However, the blood-brain barrier likely (2006) Intracerebral amyloidoma can mimic high-grade glioma on
prevents entrance of protein macromolecules into the central magnetic resonance imaging and spectroscopy. Arch Neurol
nervous system and the brain is not usually affected by systemic 63:906–907.
light chain disorders, excepted in sites missing a tight blood-brain 9. Randall RE, Williamson WC, Mullinax F, Tung MY, Still WJS
barrier such as the choroids plexus and the periventricular areas (1976) Manifestations of systemic light chain deposition. Am J Med
60:293–299.
where they have no effect on the brain (10).
10. Schröder R, Linke RP (1999). Cerebrovascular involvement in
Occurrence of light chain deposits in the brain is rare, with only
systemic AA and AL amyloidosis: a clear hematogenic pattern.
two cases previously reported in the literature (3, 7). In one of these Virchows Arch 434:551–560.
cases, it was suggested that the light chain deposition originated
from a low-grade cerebral lymphoma diagnosed 3 months earlier ABSTRACT
(3), while in the other case a monoclonal B-cell proliferation of
“neoplastic or other undefined nature” was found in close proxim- A 60 year-old man, without relevant medical history, noted a slight
ity to the deposits (7). The cell infiltrate observed in the present and progressive instability of gait for one month and right hand
case was composed of monotypic k producing plasma cells clumsiness. Brain MRI showed a cerebellar lesion, posterior to the
showing no cytologic atypia, associated with a few mature T lym- middle cerebellar peduncle. This lesion was heterogeneous and
phocytes and macrophages of the foreign body type. There was hyperintense on FLAIR sequences, isointense on T1-weighted
no evidence of an aggressive or systemic lymphoplasmacytic images, and showed gadolinium enhancement. Hematological and
neoplasm. biological serum analyses were normal as were plasma and urine
The tumor-like MRI presentation of the light chain disease in immunoelectrophoresis. CSF analysis including protein electro-
this case is reminiscent of primary intracerebral amyloidoma pre- phoresis was unremarkable. CT scans of the abdomen, chest and
senting as a mass lesion (6, 8) and likely produced by B-cell clone. pelvis were normal as were cervical echography and bone scintig-
Nevertheless, most of such cases lack clinical evidence of an raphy. A yellowish and firm lesion was surgically resected. The
aggressive or systemic lymphoplasmacytic neoplasm and are char- patient’s recovery was good, with normal total body PET scan and
acterized by a relatively indolent course (2, 5). Intracerebral light bone marrow biopsy.
chain amyloidomas are almost invariably of l type, as for the two Pathological study evidenced k light chain deposits and
previously reported cases of LCD (3, 7). We report the first case of k-immunopositive mature plasma-cells in the vicinity. The deposits
intracerebral k LCD presumably derived from local synthesis by failed to show any birefringence in polarized light microscopy
mature plasma cells and mimicking CNS neoplasm on MRI. 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 amy-
REFERENCES loidogenic and may cause tissue deposits histologically similar to
amyloid but Congo red-negative and non fibrillary at ultrastructural
1. Daicker BC, Mihatsch MJ, Strøm EH, Fogazzi GB (1995). Ocular
pathology in light chain deposition disease. Eur J Ophtalmol 5:75–81.
examination. Occurrence of light chain deposits in the brain is rare
2. Fischer B, Palkovic S, Rickert C, Weckesser M, Wassmann H (2007) and the tumor-like MRI presentation is reminiscent of primary
Cerebral AL l-amyloidoma: clinical and pathomorphological intracerebral amyloidoma presenting as a mass lesion. This is the
characteristics. Review of the literature and of a patient. Amyloid first report of intracerebral k light chain deposits which presumably
14:11–19. derived from local synthesis by mature plasma cells.
274 Brain Pathology 20 (2010) 273–274

© 2010 The Authors; Journal Compilation © 2010 International Society of Neuropathology

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doi:10.1111/j.1750-3639.2009.00349.

A 61-YEAR-OLD MAN WITH INSTABILITY OF GAIT AND RIGHT

Brain Pathology 20 (2010) 273–274 273

3. Fischer L, Korfel A, Stoltenburg-Didinger G, Ransco C, Thiel E

274 Brain Pathology 20 (2010) 273–274

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