Diagnostic Correlates of Psychiatric Presenting Symptoms of Idiopathic Basal Ganglian Calcification (Fahr’s Disease)
Shalan Joodah Rhemah Al Abbudi*
Department of Psychiatry, Consultant Psychiatrist, Imamain Kadhimain Medical City, Iraq
Submission: October 17, 2019; Published: January 8, 2020
*Corresponding author: Department of Psychiatry, Consultant Psychiatrist, Imamain Kadhimain Medical City, Baghdad, Iraq
How to cite this article: Shalan Joodah Rhemah Al Abbudi. Diagnostic Correlates of Psychiatric Presenting Symptoms of Idiopathic Basal Ganglian Calcification (Fahr’s Disease). Open Access J Neurol Neurosurg. 2020; 12(4): 555843.
Background: Psychiatric disturbances were the major presenting symptoms of idiopathic basal ganglion calcification
Objectives: evaluation of psychological presenting symptoms, clinical and sociodemographic variables
Methods: Fahr’s disease patients were assessed by a consultant psychiatrist. Sociodemographic and clinical variables were assessed. Semi-structured clinical interview and mini-mental state examination was done. Data analysis and significance was done.
Results: total 40 patients with Idiopathic basal ganglia calcification were assessed. The age range 35-78 years mean 56.4±13 years, 64% married, 88% of higher education. Psychiatric presenting symptoms were; schizophrenia 32.1%, depression 18.9%, mania17.0%, bipolar disorder 17.0% and dementia 15.1%. Negative investigation was 3%, cognitive impairment 17%, seizures11.3%, movement disorders.5%, vertigo 26.4%, headache 9.4%, unsteadiness and difficulty of swallowing 56.6%. Psychiatric morbidity was significantly associated with; cognitive impairment (P<0.001), movement disorders (P=0.034), unsteadiness (P=0.019), difficulty of swallowing (P=0.019).
Conclusion: Fahr’s disease diagnosis could be challenging, due to discrepancy between clinical presentations and radio-imaging findings. Fahr’s disease should be kept in mind in any patients with late onset neuropsychiatric disorders
Idiopathic basal ganglia calcification which known as Fahr’s disease  is chronic, slow, neurodegeneration with deposition of calcium in the basal ganglia , cerebellum subcortical white matter, cerebral cortex, thalamus, dentate nucleus, and hippocampus [3,4]. Globus pallidus is frequent site of the calcification. Calcification may involve other nuclei; putamen, caudate, internal capsule, dentate, thalamus, cerebellum, and cerebral white matter . Histologically, the media layer of the small vessels contains proteins and polysaccharides in the perivascular space . Pathogenesis may be due to blood brain barrier impairment or to a disorder of metabolism of neuronal calcium . The clinical picture may accompanied by extra pyramidal and cerebellar symptoms, Parkinsonian features,
convulsive seizures, speech disorders and dementia. Disease appears during age of 40-60 years [2,7,8] give a prevalence <1/1,000,000 . the disease is usually of insidious onset and frequently misdiagnosed as a psychiatric illness or dementia . Most cases initially presented with extra pyramidal symptoms,
cerebellar dysfunction, speech difficulty, and dementia [9,10]. Fahr’s disease is most commonly transmitted as an Autosomal Dominant trait; but it may also be passed on as an autosomal recessive trait or it may occur sporadically [9,11]. The differential diagnoses of Fahr’s disease are infections, enchapalitis, calcified angiomas, and Addison’s disease. Common clinical picture of the disease were headache, movement disorders such as Parkinsonism, dystonia, chorea and ataxia, syncope, seizures and psychiatric symptoms .
All patients with Fahr’s disease, both genders who have consulted during the period of study, agree to participate, wereincluded. Approval of psychiatric department was taken. A
questionnaire was prepared and administered to each patient
consulted during the study period.
All patients attended psychiatric clinic with suspicion of
Fahr’s disease were send for brain radio-imaging and blood
investigations to confirm the diagnosis. A questionnaire filled out
by a consultant psychiatrist, which included two parts. The first
part consists of collection of clinical and sociodemographic data.
The second part consists of the Mini-Mental State Examination
(MMSE) to assess the cognitive impairment for each patient.
Cutoff scores were 27-30 normal, 21-26 mild impairment, 11-
20 moderate impairment and ≤10 is severe impairment . All
patients were selected for administration of the semi-structured
clinical interview for DSM-IV-TR (SCID)  by a consultant
psychiatrist to assess the psychiatric morbidity. Other clinical
symptoms were collected through the same interview.
The independent variables assumed to explain the psychiatric
morbidity were socio- clinical and demographic variables.
Sociodemographic includes; age, gender, marital status, education,
occupation. Clinical variables include the associated features like;
investigation, movement disorders, headache, vertigo, seizures
unsteadiness, and difficulty of swallowing.
Data processing and analysis was conducted using a Statistical
Package for Social Sciences (SPSS) version 24. Results are
expressed as percentages for qualitative variables. Association
between variables was assessed by Chi‑square test. P≤0.05 was
considering for significant.
The study includes 40 patients with idiopathic calcification
of basal ganglia. age range was 35-78 years, mean 56.4±13 years,
half of cases fall into the age group 40 - 60 years, about 80% was
male, 70% married, 87% of lower education, about three forth not
working. MMSE reveals no cognitive dysfunction. Clinical interview
for DSM-IV-TR (SCID) found that; psychosis and schizophrenia
14 (35%), depression 8 (20%), mania (9) 22.5%, and bipolar
disorder (9) 22.5%. Investigation regarding parathyroid
hormone, calcium and phosphate were negative. Abnormal
movements Parkinson like symptoms; tremor, rigidity, was 30%
of patients. Four percent have history of seizures. Vertigo present
in 22.5% of patients. Headache present in 12.5% of patients.
Unsteadiness and difficulty of swallowing found among 50% of
patients (Table 1). Correlation of sociodemographic variables
with psychiatric presentation was done. Psychiatric presentation
of patients with basal ganglia calcification was significantly
associated with age (P<0.001), movement disorders (P=0.034),
unsteadiness (P=0.019), difficulty of swallowing (P=0.019) (Table
2). Correlation of sociodemographic and neurological symptoms
of patients found significant association of age with movement
disorders (P=0.021), unsteadiness (P<0.001), and difficulty of
swallowing (P<0.001). Marital status was significantly associated
with unsteadiness (P=0.011), difficulty of swallowing (P=0.011).
Occupation was significantly associated with movement disorder
(P=0.004) (Table 3).
The psychiatric presentation of patients with Fahr’s disease
was psychosis and schizophrenia 35%, mania 22.5%, bipolar
disorder 22.5%, and depression 20%. The neurological symptoms
were; abnormal movements; Parkinson like symptoms; tremor,
rigidity, 30%, unsteadiness 50%, difficulty of swallowing 50%,
vertigo 22.5%, headache 12.5%, and seizures 4%, Around 40%
of patients with basal ganglia calcifications presented with
psychiatric symptoms . Studies suggest criteria for the
diagnosis of Fahr’s disease include ; bilateral basal ganglia
calcification; progressive neuropsychiatric symptoms; late onset
in the fourth or fifth decade but earlier onset can also occur. Fahr’s
disease symptoms are reported individual case reports because
of the clinical rarity of the disease  thus no exact figures to
compare this study results with it. Psychiatric symptomatology
of Fahr’s disease might be the results of the interaction of basal
ganglia and cerebellum. Fahr’s disease patients of this study
showed negative results of investigations. MMSE results were no
cognitive dysfunction in patients with Fahr’s disease. Movement
disorders account for 55% of the total symptomatic Fahr’s
disease patients . The dentate nucleus of the cerebellum
provides disynaptic inputs to the basal ganglia , and
motor and non-motor domains of the substantia nigra provide
disynaptic inputs to the cerebellar cortex. These interactions
may have clinical implications for neuropsychiatric disorders
such as schizophrenia . The basal ganglia may be involved
in generating neuropsychiatric symptoms in major psychiatric
disorders such as schizophrenia and depression . Several
basal ganglia disorders can have psychiatric manifestations .
The common psychiatric features are cognitive deterioration,
psychotic symptoms, and mood disorders . Organic affective
symptoms were found more common in chronic cases of basal
calcification15. Calcifications sites, clinical features and relevant
investigations are important particularly upon incidental cases
presenting with disorganised behavior, as there may be no other
significant clinical features. Calcifications should be evaluated
with clinical pictures for proper diagnosis .
Fahr’s disease diagnosis could be challenging, due to
discrepancy between clinical presentations and radioimaging
findings. Clinical presentation includes psychiatric
and neurological symptoms. Psychiatric symptoms include;schizophrenia, psychosis, bipolar disorder, mania and depression.
Neurological symptoms include Parkinson’s like movement,
seizures, vertigo, headache, unsteadiness, and difficulty of
swallowing. Fahr’s disease should be kept in mind in any patients
with neuropsychiatric disorders.
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