CLINICAL IMAGE

Primary clinical manifestation of systemic
amyloidosis in the central nervous system
Anetta Lasek-Bal1, Michał Holecki2 , Anita Czapska3 , Aleksandra Karuga1, Jan Duława2
1
2
3

Department of Neurology, Medical University of Silesia, University Hospital No. 7, The Upper-Silesian Medical Centre, Katowice, Poland
Department of Internal Medicine and Metabolic Diseases, Medical University of Silesia, University Hospital No. 7, The Upper-Silesian Medical Centre, Katowice, Poland
Department of Radiology, Medical University of Silesia, University Hospital No. 7, The Upper-Silesian Medical Centre, Katowice, Poland

A 67-year-old male patient was admitted to the
Department of Neurology, Medical University
of Silesia, because of progressive weakness of
the left limbs persisting for several days. The patient’s relatives reported memory disturbances
that had started a few months earlier. The patient had a history of untreated cardiac arrhythmia and a neurological event of unknown origin (12 months earlier, he experienced right arm
weakness that resolved spontaneously 3 days after the onset). Moreover, he experienced disorientation, and neurological examination on admission revealed left limb paresis and ataxia. A neuropsychological examination revealed cognitive
impairment in memory and visual spatial orientation. A transcranial Doppler ultrasound showed
reduced cerebrovascular reactivity to changes in
partial CO2 pressure. Magnetic resonance imaging (MRI) of the head showed heterogeneous lesions corresponding to intracranial hemorrhages in the course of amyloid angiopathy (FIGURE 1 ).
Other diagnostic procedures did not reveal any

neurological symptoms or abnormalities. A preliminary diagnosis of cerebral amyloid angiopathy (CAA) according to the Boston criteria1 was
established, and the patient was transferred to
the Department of Internal Medicine for further
diagnostic workup. A biopsy of oral and rectum
mucosa was nonspecific; however, a biopsy of the
abdominal fat tissue suggested amyloid deposits.
Serum immunofixation revealed an abnormal ratio of lambda and kappa light chains. Scintigraphy with 123I-labeled serum amyloid P component
(SAP) showed abnormal SAP levels in the kidney.
The patient was diagnosed with amyloidosis and
treated with bortezomib.
The term “amyloidosis” is used to describe hyaline eosinophilic extracellular deposits in various tissues. The clinical presentation is nonspecific and may vary depending on the site of deposition. Neurological involvement is observed in
about 20% of the cases, and the median duration
of neuropathic symptoms before the diagnosis of
amyloidosis is 2 years.2 CAA is a cerebrovascular

A

B

Correspondence to:
Anetta Lasek-Bal, MD, PhD, Oddział
Neurologii, Samodzielny Publiczny
Szpital Kliniczny Nr 7, Śląski
Uniwersytet Medyczny, Górnośląskie
Centrum Medyczne im. Prof. Leszka
Gieca, ul. Ziołowa 45/47, Katowice,
Poland, phone: +48-32-359-83-06,
fax: +48-32-202-95-92,
e-mail: alasek@gcm.pl
Received: January 19, 2014.
Revision accepted:
January 23, 2014.
Published online: January 24, 2014.
Conflict of interest: none declared.
Pol Arch Med Wewn. 2014;
124 (3): 138-140
Copyright by Medycyna Praktyczna,
Kraków 2014

FIGURE 1 A – numerous low-signal intensity foci representing multiple old microhemorrhages (small arrows);
B – numerous low-signal intensity foci representing disseminated old microhemorrhages (large arrow, subacute
hemorrhage; small arrows, foci of old microhemorrhages)

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2014; 124 (3)

disease in which amyloid is deposited in the leptomeningeal, cortical, and subcortical arteries as
well as in the capillaries. Most individuals with
CAA are asymptomatic but they can present cognitive impairment and other neurological deficits caused by vascular wall amyloid deposition
leading to hemorrhage.3 Amyloidosis results in
intracranial hemorrhages in 15% to 20% of the
patients aged over 60 years.4 An MRI scan of the
hemorrhages in patients with CAA needs to be
differentiated with hemorrhages due to hypertension, brain changes in posterior reversible encephalopathy syndrome, or, rarely, hemorrhagic metastases.
In our case, which is quite unique, amyloidosis initially occurred in the central nervous system without systemic manifestation. Cognitive
deterioration, left limb weakness, and previously
undiagnosed neurological symptoms were caused
by amyloid pathology in the central nervous system. MRI of the head suggested CAA as the main
etiology of the hemorrhage, which prompted further investigation. Of note, we did not observe
neuropathy, which is the most characteristic neurological symptom of amyloidosis. The final diagnosis was based on abdominal fat biopsy and
SAP scintigraphy, which allowed to initiate treatment before systemic manifestations of amyloidosis occurred.
REFERENCES
1 Renard D, Castelnovo G, Wacongne A, et al. Interest of CSF biomarker
analysis in possible cerebral amyloid angiopathy cases defined by the modified Boston criteria. J Neurol. 2012; 259: 2429-2433.
2 Viswanathan A, Greenberg SM. Cerebral amyloid angiopathy in the elderly. Ann Neurol. 2011; 70: 871-880.
3 Skeik N, Porten BR, Delgado Almandoz JE, et al. A unique case report
of cerebral amyloid angiopathy with literature review. Ann Vasc Surg. 2013.
doi: 10.1016/j.avsg.2013.12.001.
4 Salzman K. Stroke. In: Diagnostic Imaging: Brain. Osborn A, ed. Salt
Lake City, Utah: Amirsys Publishing, Inc.; 2010: I72-I75.

CLINICAL IMAGE

Primary clinical manifestation of systemic amyloidosis in the central nervous system

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