Clinical Neurology and Neurosurgery 190 (2020) 105664

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Clinical Neurology and Neurosurgery
journal homepage: www.elsevier.com/locate/clineuro

Case Report

Nine-years follow-up of cavernoma located in basal ganglia mimicking
Parkinson’s disease

T

Raluca Mihaela Modreanua,*, Carsten Buhmannb, Björn Hauptmanna,c
a

Parkinson’s Disease and Movement Disorders Unit, Department of Neurology, Segeberger Kliniken, Hamdorfer Weg 3, 23795 Bad Segeberg, Germany
Department of Neurology, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany
c
Department Performance, Neuroscience, Therapy and Health, Medical School Hamburg, Am Kaiserkai 1, 20457 Hamburg, Germany
b

A R T I C LE I N FO
Keywords:
Secondary parkinsonism
Brain cavernoma
DaTscan
Vascular parkinsonism
Apathy
Right caudate nucleus

1. Introduction
Secondary parkinsonism is a well described entity with a large
variety of causes. However, there is a lack of data about parkinsonism
caused by cavernoma in the basal ganglia, given its low prevalence
(0,46 %) in the general old population, and even lower prevalence of
symptomatic-ones (0.037 %) [1]. We report a 75-years-old female patient with 9-year history of non-progressive left parkinsonism and
apathy due to a cavernoma in the right striatum.
2. Case report
Our patient was referred to neurologist in 2009 due to acute gait
impairment after a severe coughing episode. The neurological examination assessed bradykinesia and rigidity of her left limbs, cogwheel
phenomenon of the right upper-limb only by contralateral hand-activation, hypomimia and unsteady gait. The patient referred to a loss of
interest in her hobbies in the preceding years and difficulties in initiating speech and movements. Because of her apathy she had been an
inpatient in a psychosomatic clinic and received a high dose of a serotonin-norepinephrine reuptake inhibitor, without improvement.
Under the clinical suspicion of Parkinson’s disease (PD) she underwent Magnetic Resonance Imaging (MRI), a Dopamine Transporter
Single Photon Emission Computerized Tomography (DaTscan) and an
olfactory-test. The Brain MRI revealed a 2 cm diameter cavernoma with

signs of subacute hemorrhage involving mainly the right caudate nucleus but also the right putamen, as well as bilateral microangiopathy in
the basal ganglia and white matter. The DaTscan showed a severly reduced dopamine transporter uptake in the right striatum, predominately in the caudate nucleus, and only mildly diminished left
striatal binding. The olfactory-test ruled out hyposmia. Because of severe dizzy spells and nausea after 300 mg levodopa-intake, the levodopa-challenge-test was interrupted and therefore could not be assessed. Vascular risk factors such as hypertension, hyperglycemia and
dyslipidemia were assessed by routine tests. The patient had no family
history of neurodegenerative diseases and she has never taken medication causing secondary parkinsonism. Risk factors for cavernoma
such as positive family history or cerebral radiotherapy were also inconspicuous.
She was diagnosed with PD based on her clinical picture and positive DaTscan and medicated with low-dose levodopa.
Since 2011 she has been seen as outpatient annually by the same
specialist in movement disorders who has recorded the lack of progress
of her left parkinsonism over the years. Only her apathy and gait-unsteadiness have got progressively worse. No rest tremor, motor fluctuations or dyskinesia have ever occurred. The patient claimed a lack of
response to levodopa, even after the dosage was gradually raised to 750
mg per day. Symptomatic orthostasis, urinary incontinence, constipation, rapid eye movement behavior disorder (RBD), supranuclear oculomotor disturbance, falls, apraxia or alien limb were absent. There was

⁎
Corresponding author at: Parkinson’s Disease and Movement Disorders Unit, Segeberger Kliniken, Neurologisches Zentrum, Hamdorfer Weg 3, 23795, Bad
Segeberg, Germany.
E-mail address: raluca.modreanu@segebergerkliniken.de (R.M. Modreanu).

https://doi.org/10.1016/j.clineuro.2020.105664
Received 23 July 2019; Received in revised form 22 October 2019; Accepted 1 January 2020
Available online 02 January 2020
0303-8467/ © 2020 Elsevier B.V. All rights reserved.

Clinical Neurology and Neurosurgery 190 (2020) 105664

R.M. Modreanu, et al.

Fig. 1. A:DaTSCAN™. DaTSCAN™ shows severe decreased tracer (ioflupane I123) uptake in the right striatum (predominantly in the right caudate nucleus) and only
mild decreased tracer in the left striatum. B: MRI. MRIFLAIR axial cutshows the cavernoma located in the right basal ganglia affecting mostly the right caudate
nucleus, multiple microangiopathic lesions and lacunar infarcts in the striatum and white matter bilateral. C: Fusion. Fusion showsnarrow correlation between MRI
findings and DaTSCAN™.

movement disorders or RBD [2–6]. Only two of them are about secondary parkinsonism induced by cavernoma in basal ganglia [2,3],
presumably due to the compression of the cavernoma on the near
structures, as we hypothesize in our patient. Unlike our patient, none of
these cases has been clinically and paraclinically followed up over nine
years.
By the first neurological assessment, the clinical presentation and
the positive DaTscan made the differential diagnosis between the
idiopathic PD and secondary parkinsonism with mood and gait disturbance due to cavernoma and microangiopathy, difficult. However,
the lack of bilateralization and the non-progressive course of the parkinsonism over the years, as well as no response to levodopa, pointed
out to the secondary genesis due to cavernoma. There were no acute
episodes of worsening as expected by bleeding from cavernoma. There
was no progression of her parkinsonism, as expected in PD. After levodopa-withdrawal there was no worsening of her motor symptoms.
Her apathy, present throughout the course of the disease, did not
improve with a serotonin-norepinephrine reuptake inhibitor or levodopa, even when the dose was increased to 750 mg, and did not worsen
after levodopa-withdrawal, either. We hypothesize that her increasing
apathy over the years could be a consequence of the compression on the
right caudate nucleus due to the enlargement of the cavernoma, leading
to a loss of prefrontal-cortex activation. It has been already demonstrated that a hypoperfusion of the right caudate nucleus is related to
the presence of apathy in patients with normal pressure hydrocephalus
[7]. Furthermore, Levy et al. hypothesized that a lesion of the caudate
nucleus would lead to a loss of activation of the prefrontal-cortex,
consequently causing apathy [8].
We consider the progressive gait disturbance, without any change in
her parkinsonism, to be at least partially a consequence of the increasing microangiopathy causing leukoaraiosis.
We hypothesize that the pathological DaTscan findings are explained by a tissue lesion caused by cavernoma and bilateral microangiopathy. There is one report about pathological DaTscan by vascular

no spasticity or other pyramidal signs bilateral.
In 2018 she was admitted to our movement disorders clinic as an
inpatient. Because of the lack of progression of her parkinsonism over
nine years and the referred lack of response to levodopa, we decided to
re-evaluate her diagnostically. A 300 mg levodopa- as well as up to 6
mg apomorphin-challenge-tests were performed and both showed no
improvement by UPDRS Scale Part 3. In off-medication we assessed
bradykinesia and rigor of her left upper- and lower-limb. Besides cogwheel-phenomenon by contralateral activation maneuver, no rigidity or
bradykinesia of her right limbs were assessed in off-medication. Rest
tremor was absent bilateral. A Brain MRI showed a slight enlargement
of her cavernoma, affecting predominantly the right caudate nucleus,
and a moderate increment of the microangiopathy. A hyperechogenicity of the substantia nigra was excluded sonografically. Her
medication was checked again to exclude any drugs which could cause
secondary parkinsonism. We decided to repeat a DaTscan to assess a
possible disease progression, which showed a mild-to-moderate worsening of the dopamine transporter uptake bilateral compared to 2011,
affecting mostly the right caudate nucleus. Because of the negative levodopa- and apomorphin-test we decided to discontinue progressively
her levodopa. After a week without levodopa, no worsening of her
symptoms occurred.
Because of the negative levodopa- and apomorphin-test, no worsening of the symptoms after levodopa-discontinuation, one-sided
parkinsonism contralateral to the hemangioma without bilateralization
nine years after the first neurological assessment, no progression of her
rigor and bradykinesia over nine years, negative brainstem sonography
and absence of red-flags suggesting atypical parkinsonism, we diagnosed her with secondary parkinsonism caused by cavernoma.

3. Discussion
There are few descriptions in the medical literature about clinically
manifested cavernoma of the central nervous system leading to
2

Clinical Neurology and Neurosurgery 190 (2020) 105664

R.M. Modreanu, et al.

diagnostic work-up as well as S. Geeson for the English editing.

parkinsonism, which pointed out the association between the reduced
dopamine transporter uptake in basal ganglia bilateral and the extensive microangiopathy assessed by Brain MRI [9]. These findings are
similar to ours and could explain the distorted shape of the left basal
ganglia contralateral to the cavernoma by DaTscan.
However, there is no description in the literature about pathological
DaTscan by secondary parkinsonism caused by cavernoma in the basal
ganglia. The narrow correlation between Brain MRI and DaTscan
(Fig. 1) and the exclusion of other causes for her parkinsonism, led us to
the conclusion that DaTscan can also be pathological by cavernomainduced secondary parkinsonism. Interestingly, Chun et al. reported a
focal dopamine transporter uptake in a cavernous hemangioma located
in the right frontal bone as an incidental finding by a patient who underwent F-FP CIT PET/CT [10] for her left hand and leg tremor.
However, this brief report does not provide further information about
the rest of the work-up, neither about the underlying cause of the
tremor.

References
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4. Conclusion
We conclude that a cavernoma in the striatum can cause secondary
parkinsonism and apathy mimicking PD at the first neurological assessment. However, unlike PD, its natural history can remain unchanged over many years. Furthermore, DaTscan can be pathological in
secondary parkinsonism caused by both cavernoma and microangiopathy in the striatum.
Acknowledgements
We thank Dr. R. Buchert and Dr. C Gaebel for supporting the clinical

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