Rom J Morphol Embryol 2013, 54(1):195200 [609277]

Rom J Morphol Embryol 2013, 54(1):195–200
ISSN (print) 1220–0522 ISSN (on-line) 2066–8279 CCAASSEE RREEPPOORRTT
The Fahr syndrome and the chronic
lymphocytic thyroiditis
ANCA SAVA1), GABRIELA DUMITRESCU2), DANISIA HABA3),
DIANA HODOROG4), CLAUDIA MIHAILOV5), ELENA ȘAPTE6)
1)Discipline of Anatomy and Embryology,
“Grigore T. Popa” University of Medicine and Pharmacy, Iassy
2)Laboratory of Pathological Anatomy,
“Prof. Dr. Nicolae Oblu” Clinical Emergency Hospital, Iassy
3)Discipline of Radiology
4)Discipline of Neurology
“Grigore T. Popa” University of Medicine and Pharmacy, Iassy
5)Discipline of Internal Medicine
6)Discipline of Anatomy
Faculty of Medicine, “Ovidius” University, Constanta
Abstract
Fahr syndrome (FS) refers to bas al ganglia calcification that is associated with many neurolog ical and psychiatric abnormalitie s and
appears as secondary to other diseases. We described a case of FS patient who was admitted in the Department of Neurology of “Prof. Dr. Nicolae Oblu” Clinical Emergency Hospital, Iassy, Romania, with seizure and mood disorders. On CT, the cause of seiz ure was
found to be the bilateral calcifications of cerebellum, basal ganglia, thalamus and internal capsule. As the patient died after 15 days of
hospitalization due to new seizures and gastrointestinal infection, an autopsy was made. Grossly, there were bilateral symmetri cally gritty
yellow areas in basal ganglia, thalami, internal capsule, cerebral cortex, cerebellar folia, dentate nucleus, and brain stem. A detailed
histopathological examination revealed five types of calcium deposits within the walls of capillaries, small and medium-sized a rteries from
the
intracerebral affected areas, chronic lymphocytic thyroiditis and fibro-adipose tissue instead of parathyroids. We consider that intracerebral
symmetrical calcifications were the results of the hypoparathyroidism determined by an ancient autoimmune parathyroiditis that evolved to
fibrosis as at microscopy we found an autoimmune thyroiditis.
Keywords : Fahr syndrome, chronic lymphocytic thyroiditis, autopsy , symmetrical bilateral intracerebral calcification.
 Introduction
The Fahr syndrome is a degenerative neurological
disorder whose prevalence is probably less than 0.5%.
This condition is characterized by the presence of
bilateral intracranial calcifications with predilection for
the basal ganglia and dentate nuclei and is associated
with disturbances of calcium and phosphorus metabolism
[1–3].
The calcifications of vessels in basal ganglia have
been described since 1850 wh en Delacour [4] presented
a patient of 56-year-old who showed tremor, stiffness,
weakness and lower extremit y vascular calcification of
the basal ganglia. In 1855, Bamberger presented the
histopathological aspects of this type of calcification in
autopsy of a woman with mental retardation and seizures
[5]. Almost a century later, in 1930, the calcifications
of the basal ganglia named “Fahr’s disease”, after the
German neuropathologist Karl Theodor Fahr, although
he presented a 55-year-old patient with a history of
dementia and hypothyroidism, with the “immobility
without paralysis” but the histopathological aspects of
“rough granular cortex and calcifications in centrum
semiovale and striatum” and not in the basal ganglia
[5–7]. Since then, the literature uses the Fahr syndrome (FS)
term, which was applied to basal ganglia calcifications
regardless of etiology, but associated with low serum
levels of calcium and phosphorus, which usually occur
in hypoparathyroidism and the name of Fahr disease is
used for primary basal ganglia calcification, although
idiopathic, sometimes in the family without a known
cause, the calcium deposit is made on previous deposits
of mucopolysaccharides [1 , 8–10]. Both entities are
defined by bilateral intracranial calcification with neuro-
psychiatric symptoms and extrapyramidal disorder [11].
In both cases, the appearing granular deposits of free
calcospherits or in the vessel walls in the nervous tissue
[10].
In this study, we present the macroscopic and
microscopic aspects of a case with autopsy findings in
Fahr syndrome and discuss the etiopathogenesis of this
condition.
 Patient, Methods and Results
A 68-year-old female patient was admitted in the
Department of Neurology of “Prof. Dr. Nicolae Oblu”
Clinical Emergency Hospital, Iassy, Romania, with new
onset generalized tonic-clonic seizures. She was known
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for 23 years as having episodes of grand-mal seizures.
She was treated as a case of status epilepticus with
Carbamazepine. During the hospitalization, detailed
examination revealed postural static tremor of upper
limbs, cogwheel rigidity, more pronounced on right
side, mild muscular hypertonia, positive Babinski sign
on the left, dystonic and choreoathetoid movements of
upper limbs. The psychologi cal examination revealed
a depressive disorder on a dispositional background
of overall deterioration. General examination revealed
an anemic syndrome, ischem ic chronic cardiopathy and
hypothyroidism. X-ray showed pulmonary fibrosis.
She was treated for a cerebral infarct one year before.
Ophthalmologic examination showed partial optic
atrophy of both eyes. Laboratory studies revealed obvious
anemic syndrome (Hb 9.8, Ht 30%, erythrocytes
3 250 000/mm3). The sedimentation blood rate was
85 mm/h. Cholesterol 210 mg/mL, lipids 65 mg/mL,
triglycerides 88 mg/mL. Th e urinary sediment showed the presence of traces of albumin and amorphous
phosphates. Hormonal examination of the parathyroid
gland, serum calcium and phosphorus was not performed.
A non-contrast CT scan head revealed bilaterally
symmetrical hyperdense lesions of calcification, located
infra- and supratentorial, involving brainstem, cerebellar
hemispheres, globus pallidus, putamen, caudate nucleus,
thalami, periventricular and semioval centre (Figures 1
and 2).
Magnetic resonance imaging showed extensive
surfaces of T2 hypersignal and Flair on the peri-
ventricular white substance and on the semioval centre
which were interpreted as suggestive images of
degenerative cerebral vascular disease as well as T1 and
T2 hyposignal images of about 1 cm, located in the right
occipital horn (possibly calcification) and small lesions
in T2 hypersignal and Flair ,located symmetrically and
paramedian in the cerebellar lobes and in the brainstem
deck.

Figure 1 – Microcalcifications
conglomerates near the
cerebellar tonsils and
punctiform calcification
at the bulbopontine
junction.
Figure 2 – “Sun ray”
pulverulent calcification
around the bilateral
dentate nuclei.

The patient received antiepileptic treatment
(Carbamazepine), but seizures reappeared. The patient
became febrile due to bronchopneumonia and gastro-
intestinal infection and died in two days.
An autopsy was made. Brain showed diffuse atrophy
(weight 900 g). Grossly, after multiple coronal sections,
the brain showed bilateral symmetrical gritty and yellow areas in globus pallidus, putamen, thalamus, hypo-
thalamus, semioval centre, cerebral cortex, cerebellar
cortex, dentate nuclei, pons and medulla oblongata. In
the right occipital lobe and in left cerebellar hemisphere,
one
cystic area filled with a colorless liquid was observed.
The thyroid gland enlarged and contained numerous colloid cysts while the parathyroid glands could not be
identified, being replaced by a conjunctive-adipose
tissue. The heart had small pear ly white areas of fibrosis
in the interventricular septum. On sectioning, both lungs
showed small yellowish nodular formations, slightly
raised and separated by relatively normal lung tissue. The small intestine and the colon had blackish-red wall
with gray-yellowish free running mucus lining it. The
liver had a slightly increased volume and had a yellowish color. The kidn eys showed numerous areas
retracted on the external surface. The adrenal glands
showed post mortem autolysis.
Samples of brain and other organs were taken.
Tissues were fixed in 10% buffered formalin, and paraffin sections were made. Sections were stained with
Hematoxylin and Eosin (HE).
Light microscopic examination revealed various
degrees
of granular basophilic material (calcium deposits) in the vessels walls of the nervous tissue. The calcification always occurre d bilaterally, in cerebral
cortex, semioval centre, caudate nucleus, globus
pallidus, thalamus, hypothalamus, cerebellar cortex,
dentate nucleus, pons and medulla oblongata.
The calcification occurr ed in five clearly
differentiated patterns varying in shape and size:
▪ Type 1: Fine basophil granules looking like
“strings of pearls”, located in the walls of capillaries
(Figure 3);
▪ Type 2: Larger, irregular limestone deposits,
randomly arranged in small vessel walls (Figure 4);
▪ Type 3: Basophilic concentric lamellar structures
located in the tunica media of small and medium vessels
leading to obvious thickening and narrowing of the
vascular lumen (Figure 5);
▪ Type 4: Massive calcium deposits in artery walls
with appearance of ossificatio n in the walls of small and
medium-sized arterial walls (Figure 6);
▪ Type 5: Spherical limestone concretions of various
sizes located in the nervous parenchyma, apparently
unrelated to a blood vessel (Figures 7 and 8).
The white substance showed diffuse reactive gliosis
more obvious around the larger calcium deposits while
in the gray substance areas a nerve cell loss was found,
the remaining neurons having a pyknotic nucleus and an
intense eosinophilic cytoplasm.
In the neurohypophysis, only one vessel was
identified having a massive presence of concentric
calcification in the wall and a massive reduction of the
lumen (calcification Type 4).

The Fahr syndrome and the chronic lymphocytic thyroiditis
197
The thyroid had large follicles filled with weak
eosinophilic colloid. In some areas, in the thyroid
stroma, small lymphocytic conglomerates were observed sometimes centered by small thyroid follicles with
necrosis of thyroid follicular cells (Figure 9).

Figure 3 – Cerebral cortex: finely granular or coarse
calcium deposits, arranged as “strings of pearls” in the
walls of capillaries (calcification Type 1), the number
of nearby neurons is reduced visible (HE stain, ×200). Figure 4 – Globus pallidus: segmentary deposits of
basophilic material (calcium) in the wall of a small
artery (calcification Type 2) with marked reduction of
the surrounding neurons (HE stain, ×100).

Figure 5 – Dentate nucleus (cerebellum) lamellar
massive calcification in the walls of small arteries
(calcification Type 3) (HE stain, ×40). Figure 6 – Globus pallidus: massive calcification in the
walls of small vessels with marked reduction in lumen
(calcification Type 3), reducing number of surrounding
neurons, the remaining on es taking a pyknotic
appearance (HE stain, ×200).

Figure 7 – Cerebellar white substance: massive calcium
deposit with an aspect of ossification (calcification
Type 4) in the arterial wall of a small vessel (HE stain,
×100). Figure 8 – Cerebellar cortex: amorphous calcifications
masses with an aspect of “limestone concretions”
(calcification Type 5) in th e granular layer, formed by
evolution towards the conglomeration of small original
limestone deposits (HE stain, ×200).

Anca Sava et al.
198

Figure 9 – Thyroid gland: small lymphocytic conglo-
merates in the stroma of thyroid follicles showing
necrosis of thyroid follicul ar cells (HE stain, ×100).
The parathyroids could not be identified, only a
conjunctive-adipose tissue being visible in those areas.
The heart presented widespr ead fibrosis replacing the
myocardial fibers. The lungs showed acute supurative
exudates (neutrophils) in the alveolar lumen and
bronchiole. Kidneys showed tubular necrosis with
eosinophilic material in the tubular lumen. In the small
intestine adventitia several small vessels basophilic deposits were detected in the walls. The epithelium of
the small intestine had ulcers covered by fibrin while in
the neighborhood polymorphic inflammatory infiltrates were observed, predominantly lymphocytic in the
epithelium and lamina propria.
The final pathological diagnosis included the
following: multiple cerebral and cerebellar infarcts,
Fahr’s cerebral calcification, chronic lymphocytic
thyroiditis, myocardial ischemic fibrosis, broncho-pneumonia, enterocolitis.
 Discussion
The Fahr syndrome (FS) defines over 30 conditions
that result in metastatic or bilateral dystrophic calcification in the basal ganglia and can be classified as
inflammatory (CMV infection, neurocysticercosis,
toxoplasmosis, neurobrucellosis, tuberculosis, HIV infection),
tumoral (astrocytomas), hypoxic and vascular
(arteriovenous malformations, calcified infarct, ischemic
encephalopathy), endocrinal (hypoparathyroidism,
pseudohypoparathyroidism, hyperparathyroidism), toxic
(CO and Pb intoxication, hypervitaminosis D,
radiotherapy), metabolic and degenerative (senility, mitochondrial encephalopathies, leukodystrophic
diseases, idiopathic familial, motor neuron disease,
myotonic muscular dystrophy, carbonic anhydrase deficit, biopterin deficit) and other (malabsorption,
Down syndrome, lupus, tuberous sclerosis, arthro-
griposis) [7, 12, 13].
Lowenthal (1986) established the defining criteria
to the Fahr syndrome (including Fahr disease): (1) Calcifications should have a characteristic distribution,
or to inquire at least globus pallidus, with or without
cerebellar calcification; (2) The calcifications should
be obvious on the computed tomography; (3) The calcifications should be large enough to be detected at
macroscopic examination. As pects that support this
diagnosis, but are non-diagno stic, include the following:
(1)
Determinable clinical presentation, and (2) Existence
of a metabolic abnormality, particularly hypopara-thyroidism [14].
The onset age of clinical symptoms is usually
between 30–60 years [15, 16]. Fahr syndrome patients (including those with Fahr disease) may exhibit
movement disorders such as Parkinsonism, chorea,
tremor, paresis, dystonia, sp eech disorders [1, 2, 11].
Other neurological symptoms may include events such
as stroke, seizures, syncope, often associated with
psychiatric conditions such as psychosis or dementia [9, 15, 16].
The Fahr syndrome diagnosis in this case was based
on clinical data (disease onse t at the age of 45 years;
the disease initially manifested by episodes of grand
mal seizures, which were later associated with other
neurological
and psychiatric symptoms) imagistic aspects
(bilateral intracerebral calcifications in almost all the
intracranial nerve structur es) and autopsy aspects
(atrophic brain that weighed only 900 g, bilateral
calcification of various size s and shapes, located in
the walls of capillaries, small and medium arteries of all intracranial nerve struct ures). There could not be
determined the serum levels of calcium, phosphorus,
alkaline phosphatase and of the parathyroid hormone.
The most common area of calcification in Fahr
disease is globus pallidus, but the Fahr syndrome also
involves other intracerebra l areas that may include
putamen, caudate nucleus, dentate nucleus, lateral
thalamus (striopallidodentate calcification) and cerebral
cortex, internal capsule, cerebellar film [13, 17–19]. This pattern of arrangemen t of bilateral intracerebral
calcifications was also identified in our case as the
computed tomography and magnetic resonance imaging revealed symmetrical and extensive calcifications in
the putamen, globus pallidus, caudate, dentate nucleus,
thalamus, cortex, cerebellum and internal capsule. The
microscopic
examination confirmed postmortem vascular
calcifications in all these area s, but also in the brainstem
situation, which is rarely reported in other studies [20].
Neuropathological studies have shown that intra-
cerebral bilateral mineral deposits are composed of non-atherosclerotic calcium locat ed in the walls of small
vessels, especially those in areas that control movement
[5]. Those patients with dementia show evidence of neuronal loss in the fronto-temporal cortex and in the
basal nucleus of Meynert. The calcifications affecting
the average of small and medium arteries and the perivascular tissue of arterioles and capillaries are
associated with neuronal degeneration and gliosis [21].
Fujita D et al. have classified how calcium deposits
are
made on intracerebral vessel walls into three patterns:
diffuse deposition within the tunica media of small and
medium-sized vessels (Type 1 deposition), free spherical
or lobulated concretions (Type 2 deposition) in the
parenchyma, and rows of small calcospherites lying
along capillaries (Type 3 deposition) [22]. The detailed
histological examination of those sections taken from the
affected areas in this case we have shown that calcium

The Fahr syndrome and the chronic lymphocytic thyroiditis
199
deposits are present in all intracranial nerve structures
and that they can take five morphological aspects, from
early lesions to ossification and concretion images. These
morphological aspects may suggest the evolutionary
picture of Fahr type dystro phic calcification and at the
same time, they explain the polymorphism of clinical
manifestations. The initial calcification vessel walls
showed thickening by means of a homogeneous material,
slightly eosinophilic, possibly a degenerative protein substance that allows the initiation of calcification,
followed by a slowly progressive deposit of calcium
and probably other minerals, possibly related to the metabolic disturbance of calcium/phosphate. Our study
also demonstrated that along with the calcium deposits
identified in various forms and degrees in the walls of capillaries and small and medium arteries, the nervous
tissue develops an important reactive gliosis and a
significant reduction of the number of neurons in the
corresponding areas, probably due to a progressive
decrease of the corresponding blood flow.
Several researchers have investigated the mineral
elements shown in the vascular calcifications in the
Fahr syndrome and proposed the term “mineralization” for these deposits since it has been shown that they
contain, apart from calcium, a wide range of other
minerals: P, Fe, Cl, S, Al, K, and Zn [23, 24].
The pathophysiology of the Fahr syndrome is still
unknown, but there are hypotheses that the intracerebral
calcium deposit is a metastat ic type, secondary to local
disruption of blood-brain barrier being or to a calcium
neuronal metabolism disorder [15]. Other authors have
found that a defective tran sport of iron or increased
production of free radicals may be possible factors to
initiate calcification [16].
Recent allegations sustain that the bilateral basal
ganglia calcifications may be the result of a latent
viral infection, possibly with Epstein–Barr virus (EB).
Morishima T et al. (2002) analyzed the lymphocyte
subsets and the cytokines in the peripheral blood of 10
adults with calcification of the basal ganglia concluding that the natural killer cells which release cytokines in
circulation,
particularly the tumor necrosis factor-α, may
be involved in the pathophysiology of this syndrome and
that the EB virus and other viruses may be associated
with the etiology of basal ganglia calcifications [25].
Apart from the family cases, considered to be
genetically determined, such as Fahr disease, the
accumulation of calcium in Fahr syndrome is due to the
abnormal seric levels of this mineral (associated with changes in vascular permeability due to local calcium
concentration) or to th e dystrophic calcifications
associated with local circ ulatory disturbances and
metabolic disorders (hypoxia, hypoglycemia, disorders
of fluid and electrolyte balance) [12].
Vega
MG et al. demonstrated the correlation between
the bilateral basal ganglia calcification, hypopara-
thyroidism and extrapyramidal syndrome [26].
Morgante L et al. reported three cases of Fahr’s
syndrome with disorders of calcium metabolism and
had had meningoencephalitis during their childhood.
They hypothesized that gliovascular changes caused by cerebral inflammation may facilitate the emergence of calcifications
within the striopallidodentate system when
there is a disruption of the calcium metabolism [27].
The pathophysiologic mech anism of calcification
in Fahr syndrome is also discussed by Guseo A et al.
who stress the role of increased permeability and the dysfunction of mesenchymal cells in the vessel walls
[19]. Somehow, the same hypothesis is also issued
by Kobayashi S et al. who, by means of transmission
electron microscopy, revealed small deposits, especially in the adventitious cells cytoplasm of the blood vessels and sometimes by means of cytoplasmic processes of the glial cells, which led the researchers to believe that
the pericytes could play an important role in the onset of
calcification in Fah r’s syndrome [23].
The etiopathogenesis of symmetric intracerebral
calcifications and of multiorgan calcifications (neuro-hypophysis, thyroid, small in testine) identified in the
presented case still remains uncertain. However, some
speculations are possible. Our case presented a chronic
lymphocytic thyroiditis. Pa tients with autoimmune
endocrine disease are at increased risk of developing other autoimmune diseases, both in the other endocrine glands and the other non-endocrine organ, which is defined as the polyglandular autoimmune syndrome.
Autoimmune hypoparathyroidism and autoimmune
thyroid disorders are included in the autoimmune polyglandular syndrome [28], which was classified into four types according to the affected organs [29]. From a histopathological point of vi ew, the glands affected by
this syndrome present in their structure mononuclear
infiltrates consisting mainly of lymphocytes that are
located in glandular stro ma, but aggressing the
parenchyma and leading to cell necrosis. As the disease progresses, atrophy and fibrosis install [30, 31].
In this case, although there are no neuro-
endocrinology
or calcemia and phosphate investigations,
given the presence of chroni c lymphocytic thyroiditis,
one can advance the hypothesis that autoimmune hypo-parathyroidism is a possible cause of extensive symmetrical intracerebral calcifications. This hypothesis is supported by the fact that at autopsy, in the location of the parathyroid glands only fibro-adipose tissue was
found, suggesting an ancient autoimmune pathology of
the parathyroid glands. A similar situation was reported by Mori K et al. , in 1991, but the hypoparathyroidism
associated to the chronic thyroiditis was considered to be idiopathic [32].
 Conclusions
Due to severe complications of hypoparathyroidism,
especially significant being the extensive intracerebral
calcification leading to neuropsychiatric symptoms resistant to treatment, it is important that in the presence
of hypothyroidism determined by chronic lymphocytic
thyroiditis,
doctors should also consider the investigation
of the parathyroid glands.
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Corresponding author
Anca Sava, MD, PhD, Discipline of Anatomy and Embryo logy, Morphofunctional Department, “Grigore T. Popa”
University of Medicine and Pharmacy, 16 Universit ății Street, 700115 Iassy, Romania; Phone +40744–303 678,
Fax +40232–301 633, e-mail: dr_anca_sava@yahoo.com

Received: November 16th, 2012
Accepted: February 13th, 2013