A 33-year-old female patient presented with complaints of loose teeth and was found to have oligodontia, gingival inflammation, and advanced periodontitis. She had a history of brain fever and meningitis as a child. Imaging found calcification in the basal ganglia consistent with Fahr syndrome. The patient was diagnosed with Fahr syndrome based on her clinical history and imaging findings. She received full-mouth tooth extraction and is undergoing dental rehabilitation.

1. Gauri Kapila
MDS student
Department of Periodontology and Oral Implantology

2.  A33-year-old female patient reported to the Department of
Periodontics, Oxford Dental College, Bangalore, India, in May 2013
with the complaint of loose maxillary and mandibular teeth.
 Mobility was noticed 6 months previously > gradually increased >
associated with pain and difficulty when eating.
 Written informed consent was obtained for additional examination
and treatment on the same day.

3.  The patient had normal growth and development until the age of 4
years, at which time the patient experienced brain fever and
meningitis.
 This was followed by altered development, including stunted height,
altered gait, and slow speech and learning.
 After the treatment for brain fever and meningitis, the patient had no
history of epilepsy or any other health problems.
 The patient attained puberty at a normal age and reported a history
of regular menstrual cycles.

4.  Figure 1 illustrates patient’s short stature (141 cm height)
 Figure 2 shows the short digits on the hands and feet.
 The patient weighed 34.7 kg and had a spastic gait and
poor motor coordination.

5.  Figure 3 illustrates the oral findings, which include oligodontia with only 13 teeth
present.
 The missing teeth included teeth #2, #6 through #11, #14 through #19, #22 through
#27, #30, and #32.
 The gingiva of the remaining teeth was reddish-pink in color, soft, and edematous in
nature, with increased size and absent stippling.
 Generalized bleeding on probing, generalized Miller Class IV recession, and Glickman
Grade III mobility were noted.

6.  A panoramic radiograph and cone-beam computed tomography (CBCT) of the maxilla
and mandible showed a generalized advanced horizontal bone loss involving the
alveolar and basal bones (Figs. 4 and 5).

7.  After additional investigation, routine blood examination showed all
the parameters within normal range, except hemoglobin (9 mg/dL),
red blood cells (3.2 million/m), and packed cell volume (29%).
 Other blood investigations revealed that alkaline phosphatase (83
U/L) and triiodothyronine (168 ng/dL) levels were normal.

8.  The thyroxine level (12.5 mg/dL) was slightly elevated (normal is 4.5
to 12 mg/dL), but the thyroid stimulating hormone level (7.44 mIU/L)
was markedly elevated.
 The bone mineral density was low (SD of 2.4), indicating
osteoporosis and increased risk of fracture.
 Considering the history of brain fever, stunted growth, and poor
motor coordination, a CT scan of the brain was advised.

9.  The CT scan showed areas of hyperdensity, indicating the presence of calcification of
the basal ganglia (Fig. 6).

10.  These findings are in accordance with the diagnostic criteria for Fahr
syndrome, which include bilateral calcification of the basal ganglia as
visualized on neuroimaging.
 There was an absence of biochemical abnormalities, and any
infectious, toxic, or traumatic causes were ruled out.
 Meningitis does have neurologic manifestations, but they are not
always present, and these neurologic symptoms in the patient are a
result of bilateral calcifications seen in the basal ganglia.
Saleem S, Aslam HM, Anwar M, et al. Fahr’s syndrome: Literature review of
current evidence. Orphanet J Rare Dis 2013;8:156.

11.  Based on the above findings, the authors, along with the physician
(Dr. G. M. Arvind, Manipal Hospital, Bangalore, India), neurologist
(Dr. E. V. Joshy, Sri Sai Hospital, Bangalore, India), and
neuroradiologist (Dr. Zarina Aziz, Sri Sai Hospital), arrived at a
diagnosis of Fahr syndrome.
 Full-mouth extraction was completed, and replacement of the teeth
(maxillary and mandibular dentures) is currently in progress.

12.  Fahr syndrome was first noted by the German neurologist Karl
Theodor Fahr in 1930.
 It is a rare degenerative neurologic disorder characterized by
calcifications and cell loss within the basal ganglia.
 The calcium deposits in the brain may occur before the onset of the
symptoms, usually in the third decade of life.
 Fahr syndrome can be sporadic or familial and demonstrates no
abnormalities in calcium metabolism and kinetics.
Ogi S, Fukumitsu N, Tsuchida D, Uchiyama M, Mori Y, Matsui K. Imaging of
bilateral striopallidodentate calcinosis. Clin Nucl Med 2002;27:721-724.
Amin OS. Fahr’s disease: A case series. CukurovaMed J 2013;38:823-831.

13.  Fahr syndrome is characterized by clinical heterogeneity and can be
asymptomatic (frequent among middle-aged patients) or associated
with neuropsychiatric manifestations.
 The true prevalence is unknown, but an incidence of basal ganglia
calcifications ranging from 0.24% to 2% was reported in consecutive
radiologic studies, showing an evident relationship with increasing
age.
Manyam BV. What is and what is not “Fahr’s disease.” Parkinsonism Relat
Disord 2005;11:73-80.

14.  Tedrus et al. reported an incidence of 0.68% among 3,662 cranial CT
scans analyzed.
 Although bilateral and symmetric basal ganglia calcification is known
to be associated with multiple medical conditions, the exact etiology
is still unknown.
 Genetic alterations were attributed to genes in the region of
chromosome 14.
Tedrus GM, Fonseca LC, Nogueira EJ Jr. Basal ganglia calcification on computed
tomography: Clinical characteristics in 25 patients (in Portuguese). Arq Neuropsiquiatr
2006;64:104-107.

15.  Many of these conditions involve basal ganglia only or
predominantly.
 The condition that was closely described with diffuse, bilateral,
symmetric striopallidodentate calcinosis is primary
hypoparathyroidism.
 When there is no explainable cause for striopallidodentate
calcinosis, the condition is termed Fahr syndrome.
Rastogi R, Singh AK, Rastogi UC, Mohan C, Rastogi V. Fahr’s syndrome: A rare
clinico-radiologic entity. Med J Armed Forces India 2011;67:159-161.

16.  According to Manyam et al. Fahr syndrome is found more often in
males than in females, and movement disorders account for 55% of
the total symptomatic patients.
 Most cases of Fahr syndrome present with extrapyramidal
symptoms.
 Clinical expression of Fahr syndrome varies greatly. Symptoms
include psychiatric disorders, epileptic seizures, and extrapyramidal
syndrome and various neurologic conditions.
 Statistics showed that the prevalence of neurologic symptoms may
vary in Fahr syndrome.

17.  Oral examination of a patient with Fahr syndrome revealed
oligodontia and Grade III mobility of the remaining teeth.
 Generalized gingival inflammation and Class III gingival recession
was also seen.
 The patient in the current case report presented most of these
symptoms, which are similar to those reported by Aditya et al.and
Ahad et al.

18.  The differential diagnosis considered was Hajdu-Cheney syndrome
(HCS).
 The main clinical features of HCS include short stature, scoliosis and
kyphosis, elongation of the skull, small chin, clubbing of the fingers,
coarse hair, and thick eyebrows.
 Oral and dental manifestations of HCS are as follows:
 1) premature exfoliation of the teeth;
 2) dental maleruption and malocclusion;
 3) increased tooth mobility;
 4) impaction of teeth;
 5) hypoplastic dental roots;
 6) atrophy of the alveolar processes; and
 7) structural changes in the dentin and cementum of teeth.

19.  Radiographically, the most frequent findings include persistent wide
cranial sutures, absence of the frontal and maxillary sinuses, and
osteolysis of the distal phalanges.
 Another syndrome that can be considered for differential diagnosis is
Kenny-Caffey syndrome, which is characterized by growth delay,
cortical thickening of the long bones, hypocalcemia, hypothyroidism,
and calcification of the basal ganglia.
 This was excluded in the current case because the majority of these
features were absent in the patient.

20.  Fahr syndrome is diagnosed based on clinical aspects,
neuroimaging findings, and the exclusion of other primary causes.
 Final diagnosis in the present patient was given by the radiographic
and laboratory findings and bilateral calcifications seen in the CBCT
of the brain, specifically in the basal ganglia.
 Imaging also revealed progressive cerebral atrophy, pointing to a
diagnosis of Fahr syndrome.

21.  To the best of the authors’ knowledge, this is only the second case
report that describes oral findings of a patient with Fahr syndrome.
 This case report will increase awareness of Fahr syndrome and help
clinicians arrive at the correct diagnosis.
 Early presentation and diagnosis can help in early treatment and
result in less mortality and morbidity

22.  Fahr's disease with oral manifestations: report of a rare case.
 Aditya A, Lele S, Aditya P., 2012
 CLINICAL PRESENTATION AND INTERVENTION:
 A patient presented with the complaint of partial anodontia, but further
clinical and radiographic investigations showed a myriad of findings
including stunted growth, osteoporosis and pathological calcifications. Oral
findings included oligodontia and advanced periodontitis in relation to the
present teeth. Full-mouth rehabilitation was eventually planned for the
patient.
 CONCLUSION:
 This case shows the necessity for dentists to be aware of symptoms
associated with Fahr's syndrome in order to make appropriate referrals and
to enable diagnosis and treatment.