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Clinical correlations of CT scan-detected calcifications of the basal ganglia.

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Clinical Correlations
of CT Scan-Detected Calcifications
of the Basal Ganglia
Martin J. Murphy, M D
A review of CT scans of 7,081 patients demonstrated calcifications of the basal ganglia in 53. The calcifications were
evident in the skull roentgenograms of only 4patients out of 40 in whom both CT scans and plain roentgenograms
were available, demonstrating the superior resolution of this new method. Seventy-five percent of the patients were
older than 50 years of age. Of the younger patients, 5 had had prior cranial irradiation; 1 had received cranial
irradiation and intrathecal methotrexate therapy for meningeal leukemia; and 2 others had deep-seated arteriovenous malformations. Serum concentrations of calcium and phosphorus were normal in all 46 patients in whom they
were measured.
We conclude that the detection of small calcifications of the basal ganglia in persons above 50 years of age is
infrequently associated with either clinical signs of basal ganglia dysfunction or calcium and phosphorus abnormalities. Calcium deposition in these patients may be related to vascular changes associated with aging. In younger
patients a specific pathogenetic factor or underlying process is frequently found.
Murphy MJ: Clinical correlations of CT scan-detected calcifications of the basal ganglia.
Ann Neurol 6:507-511, 1979
Microscopic basophilic deposits within and surrounding the cerebral blood vessels are frequently
found at postmortem examination in a characteristic
distribution involving the globus pallidus, putamen,
and caudate and dentate nuclei [18, 281. The deposits
are formed of small granules located within the media and adventitia of arterioles, in the perivascular
spaces and surrounding capillaries. As the deposits enlarge, the granules coalesce, encircling the vessels and extending into the adjacent parenchyma to
form microscopic “brain stones.” The lumen and endothelium of the vessels and the surrounding brain
parenchyma are not affected until late in the process
[6, 9, 14, 18, 201.
Initially, the deposits consist of an organic matrix
formed by protein and possibly mucopolysaccharides
[ 11 upon which mineral deposition later takes place.
As the deposits enlarge, first iron and then calcium
can be detected in histochemical stains [l, 14, 18, 29,
3 11. Progressive deposition of calcium and phosphorus in the form of hydroxyapatite results in enlargement of the deposits [30, 321. Although the deposits are composed largely of calcium, several other
minerals (manganese, aluminum, zinc, and fluorine)
have been found in small amounts [30].
Calcifications that advance sufficiently to be evident in plain roentgenograms often are associated
with hypoparathyroidism o r pseudohypoparathyroidism [2, 3, 6, 181. Others occur as an inherited
condition without either abnormalities of calcium
and phosphorus metabolism o r somatic features suggestive of pseudohypoparathyroidism or pseudopseudohypoparathyroidism [ 5 , 191.
This study reports the detection of basal ganglia
calcifications by CT scan together with an analysis of
the clinical characteristics of affected patients.
From the Department of Neurology, University of Iowa College
of Medicine, Iowa City, IA 52242.
Accepted for publication Apr 16, 1979.
Patients and Methods
The CT scan of 7,081 consecutive patients were visually
reviewed. Computerized tomography had been performed
using the EM1 scanner with an 80 x 80 matrix initially and
160 x 160 since 1976. The scans were done using the 8
mm collimator at 120 kvp and 33 ma. The window width
was set at 100 EM1 units (200 H) and window level at 15
units (30 H). The calcifications characteristically appear as
small, bilateral, discrete areas of increased density located
in the region corresponding to the globus pallidus (Fig 1).
When they are more extensive, the putamen and caudate
nucleus are also involved (Fig 2). If areas of increased density were found in a paraventricular location or in other
regions of the brain, not associated with similar findings in
the basal ganglia, the case was not included.
The clinical records of each patient were analyzed with
regard to age, sex, clinical features, final diagnosis, findings
o n plain skull roentgenograms, and serum calcium and
phosphorus levels.
0364-5134/~~/120507-05$01.25
@ 1979 by Martin J. Murphy
507
F i g 1. CT scan of a 6J-year-old woman with head trauma.
Bilateral basal ganglia calcifications are present i n an
area corresponding to the globus pallidus. Roentgenograms of
the skull were normal.
Results
Calcifications within the basal ganglia were identified
in 53 patients, 29 female and 24 male. The calcifications were bilateral and symmetrical in 41 and unilateral in 12. Two patients also had calcifications in the
occipital and frontal lobes and 1 had them in the
dentate nucleus. Skull roentgenograms demonstrated
calcifications in 4 out of 40 patients in whom both C T
scans and plain films were available.
The patients' ages ranged from 5 to 84 years, 7 5 %
being above 5 0 years of age at the time of C T scanning (Fig 3 ) . The clinical diagnoses were cerebrovascular disease (17), intracranial neoplasms ( 8 ) , dementia ( 8 ) ,craniocerebral trauma ( 3 ) , CNS infections
(3, including 1 patient with possible congenital toxoplasmosis), and miscellaneous, including headache,
motor neuron disease, psychiatric disorders, and
poorly defined gait disturbances. O n e patient had
Parkinson disease. Slowness of movement and
stooped posture were present in 2 other patients.
Two patients had an abnormal gait suggesting cerebellar involvement.
Among the younger patients, 4 of the 6 under 20
years of age and a 32-year-old had had prior cranial
irradiation (Table). In addition, an 8-year-old patient,
Fig 2. CT scan of an 18-year-old woman with a hypothalamic
tumor that was irradiated when she was 7 . Bilateral
calcifications involve the pallidurn, putamen, and head of the
caudate nucleus. The calczjcations were n o t demonstrated in
plain skull roentgenograms.
14 12 10 -
16
h.
0
864 2 -
0
10
20
30
40
50
60
70
80
AGE
F i g 3 . Age distribution of patients with basal ganglia
calcijcatio ns .
508 Annals of Neurology Vol 6 No 6 December 1977
90
Data on 5 Patients with Basal Ganglia Calcifications Who Previousb Received Cranial Irradiation"
Patient No.,
Age (yd,
and Sex
Diagnosis
1. 19, F
Right optic nerve glioma with
chiasma involvement
2. 18, M
4. 32, F
Right frontal arteriovenous
malformation
Hypothalamic tumor (no tissue
diagnosis)
Malignant pinealoma
5. 15, F
Atypical suprasellar teratoma
3 . 18, F
Age at Irradiation, Source,
Fields, and Tumor Dose
Interval between
Radiation Therapy
and C T Scan (yr)
9 mo-200
18
kv (orthovoltage), right
and left lateral skull; 7 x 7y2 cm;
4,250 R14 wk
5 yr-"Co,
right and left lateral
skull; 6 x 6 cm; 5,000 FU9 wk
6 yr--"OCo, right and left lateral skull;
10 x 10 cm; 4,657 R18 wk
7 yr--"'Co, right and left lateral skull;
8 x 8 cm; 4,530 R18 wk
12 yr-250 kv (orthovoltage), right
and left lateral skull, frontal, vertex, and occipital; 6 cm; 5,789 R16
wk
5 yr--""Co, right and left lateral skull;
6 x 6 cm; 4,054 FU7 wk
10
11
20
10
"All 5 patients had normal serum calcium and phosphorus levels.
previously reported by Mueller et a1 [20], had received cranial irradiation and systemic and intrathecal
methotrexate therapy for acute lymphocytic leukemia with meningeal involvement. In 2 other young
patients, aged 8 and 24 years, the calcifications were
associated with arteriovenous malformations.
Serum calcium and phosphorus concentrations
were normal in all 46 patients in whom these determinations were available.
Three of the patients died. Sections from the basal
ganglia were available in 1. Basophilic perivascular
deposits were evident, with minimal parenchymal involvement.
Discussion
CT scanning is considerably more sensitive than plain
roentgenograms in the detection of basal ganglia
calcifications. The paucity of clinical signs suggesting
basal ganglia dysfunction is at variance with the incidence of such signs when basal ganglia calcifications
are visible in plain roentgenograms. Muenter and
Whisnant [2 11 found that involuntary movementschorea, athetosis, or tremor-and altered coordination or abnormal muscle tone were present in 25% of
their patients with calcifications visible on roentgenograms. In familial idiopathic calcification of the
basal ganglia the reported incidence of neurological
abnormalities is even higher. In the review by Boller
et a1 [5], 23 of 36 patients demonstrated abnormalities, which included progressive intellectual deterioration, dysarthria, seizures, parkinsonian features, and spasticity. It is likely that the paucity of
signs of basal ganglia dysfunction in the patients reported here reflects detection of the deposits at a
time when parenchymal involvement is minimal. This
inference is supported by pathological studies that
demonstrate minimal or no neuronal involvement
until large concretions are formed [ 14, 2 1, 291 and
absence of clinical abnormalities in young members
of families with idiopathic calcifications, even when
the calcifications are demonstrated in skull roentgenograms [51.
The absence of clinical or chemical abnormalities
suggesting idiopathic hypoparathyroidism and pseudohypoparathyroidism in our patients is in contrast to their presence in two-thirds of the patients
with basal ganglia calcifications visible in skull roentgenograms [3, 181. The present results are similar to
those of Koller et a1 [151.
The presence of basal ganglia calcifications following radiation therapy has been described in several reports [3, 12, 17, 231. The total radiation dose
has varied from 4,000 to over 9,000 rads. The interval between the initial radiation treatment and detection of the calcification has ranged from 3 years to 20
years, as in our fourth patient (see the Table). Delayed radiation injury to the central nervous system is
a well-recognized complication, usually producing
symptoms after a variable period with a peak within
one and one-half years [16]. Arterioles and capillaries
are particularly sensitive to radiation, which can result in fibrinoid necrosis involving the blood vessel
walls, endothelial and periadventitial fibroblastic
proliferation, and telangiectatic dilatation of the capillaries [26]. Calcification occurs within these areas of
degeneration [13, 22, 261.
The patient in whom calcifications were found
after intrathecal methotrexate therapy and cranial ir-
Murphy: Basal Ganglia Calcifications
509
radiation for meningeal leukemia represents an
example of necrotizing leukoencephalopathy subsequent to this combination of therapy [7, 24, 25,
271. Microscopically, the principal changes consist of
multiple noninflammatory necrotic foci within the
cerebral white matter with varying degrees of mineral
deposition and diffuse reactive astrocytosis. The
calcifications have been found in degenerating axons
and cellular debris, and in a perivascular distribution.
Vascular changes indicative of radiation injury have
rarely been found [27]. Some of the patients described were asymptomatic, but the majority developed a subacute disorder with seizures, progressive intellectual impairment, and focal neurological
deficits. It has been postulated that this process is
secondary to the action of methotrexate [24, 271,
with radiation therapy increasing the diffusion of
methotrexate through an impaired blood-brain barrier 1251.
Most of the present patients had no identifiable
condition to explain the presence of calcifications. It
is suggested that the small calcifications detected in
individuals above 50 years of age result from agerelated vascular changes. Slager and Wagner [29], in
their histological study of calcareous deposits within
the basal ganglia in unselected autopsy material,
found an increased incidence with aging. The finding
correlates with the demonstration of calcium deposition in association with the progressive structural
and biochemical changes that take place in vessel
walls with advancing age [8, 101. Whether these vascular changes are the result of aging per se, arteriosclerosis, or both, is not settled [4, 111.
Presented in part at the 103rd Annual Meeting of the American
Neurological Association, Washington, DC, September, 1978.
The author wishes to thank Dr S . Cornell for his assistance in the
selection of cases, Dr M. W. Van Allen for his advice and encouragement, and Mrs J. Hulme for secretarial assistance.
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Murphy: Basal Ganglia Calcifications 5 11
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