Türkiye Psikiyatri Derneği Yıllık Toplantısı ve 1. Uluslararası 25. Ulusal Klinik Eğitim Sempozyumu, İzmir, Türkiye, 19 - 22 Mayıs 2022, ss.127
OBJECTIVE: Fahr Syndrome is a rare neurological disorder characterized
by abnormal calcium deposition in the basal ganglia (lenticular nucleus and
the internal globus pallidus) and hypocalcemia in the brain region where
movements are controlled and fine tuned. Associated neuropsychiatric
symptoms with Fahr Syndrome are generally difficulty in concentration,
personality and memory changes, behavioral problems, psychosis, and
dementia. About 40% of patients with basal ganglia calcification may
present with psychiatric symptoms such as mania, apathy, psychosis, etc.
In this case, a patient with a psychotic episode caused by hypocalcemia
due to Fahr's syndrome is mentioned. Oral consent was obtained from
the patient for the case presentation.
CASE: 41-year-old housewife patient, 5th grade dropout, has been
followed up with a diagnosis of psychosis suggested to be related with
Fahr's syndrome diagnosed for 8 years is hospitalized. In her anamnesis,
it was learned that the signs and symptoms appeared about 8 years ago
with presenting deterioration in mental functions, disorganized and
hallucinatory behaviors, tremor and psychotic findings. She has been
in remission with clozapine 200 mg/perday for 2 years. She has been
taking clozapine regularly but cessed calcium supplement for about 6
months because of constipation that has been used for the treatment
of hypocalcemia caused by Fahr's syndrome. She was admitted to our
clinic with the diagnosis of psychotic exacerbation with psychomotor
agitation and excitation, persecution delusions, disorganized behaviors,
and auditory and visual hallucinations. A chvostek sign was detected on
physical examination. In laboratory findings serum calcium level was
measured as 6.03 mg/dl (normal reference range of our hospital is 8.6 -
10.2 mg/dl). There were no other pathological findings in blood tests.
In brain magnetic resonance imaging; calcification in bilateral caudate
nucleus head, lentiform nucleus, internal globus palidus and posterior
part of thalamus was seen. In addition to the patient's current clozapine
200 mg/perday treatment, calcium and calcitriol treatment was
administered. It was observed that the serum calcium level increased to
8.65 mg/dl after approximately 2 weeks of hospitalization, the psychotic
symptoms subsided when the calcium treatment returned to normal
limits, and the PANNS score regressed from 83 to 40.
DISCUSSION:Treatment of clinical signs and symptoms of Fahr
Syndrome depends on the correction of the underlying cause. It
has been observed that with appropriate approach and treatment
psychotic symptoms possibly caused by hypocalcemia was relieved
by normalisation of serum calcium level. As seen in our case, organic
causes that may cause the psychiatric conditions should be investigated
if suspected for optimal treatment.
Keywords: psychosis, neuropsychiatry, fahr syndrome