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Year : 2020  |  Volume : 4  |  Issue : 1  |  Page : 90-93

Obsessive compulsive disorder with psychotic features as neuropsychiatric manifestation of fahr's disease

Department of Psychiatry, Datta Meghe Institute of Medical Sciences, Sawangi, Wardha, Maharashtra, India

Date of Submission03-Oct-2019
Date of Decision22-Oct-2019
Date of Acceptance06-Nov-2019
Date of Web Publication30-May-2020

Correspondence Address:
Dr. Ajinkya Sureshrao Ghogare
House Number 4, Shree Colony, Daryapur - 444 803, Amravati, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aip.aip_65_19

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Fahr's disease (FD) is a rare neurological disorder characterized by idiopathic bilateral basal ganglia calcification. It manifests as psychiatric, cognitive, and neurological manifestations. FD is distinct from Fahr's syndrome, in that the latter is having the symptoms generated by preexisting disease and abnormalities evident on blood investigations. In this case report, we present a case of FD presenting as obsessive compulsive disorder with psychotic features.

Keywords: Fahr's disease, Fahr's syndrome, idiopathic bilateral basal ganglia calcification, obsessive compulsive disorder with psychotic features

How to cite this article:
Ghogare AS, Agrawal SR, Patil PS, Vankar GK. Obsessive compulsive disorder with psychotic features as neuropsychiatric manifestation of fahr's disease. Ann Indian Psychiatry 2020;4:90-3

How to cite this URL:
Ghogare AS, Agrawal SR, Patil PS, Vankar GK. Obsessive compulsive disorder with psychotic features as neuropsychiatric manifestation of fahr's disease. Ann Indian Psychiatry [serial online] 2020 [cited 2020 Oct 20];4:90-3. Available from: https://www.anip.co.in/text.asp?2020/4/1/90/285509

  Introduction Top

Fahr's disease (FD) is a rare neurological disorder characterized by bilateral symmetrical idiopathic basal ganglia calcification. FD manifests as psychiatric, cognitive, and neurological manifestations.[1] FD is also known as idiopathic basal ganglia calcification. It also affects other brain areas such as dentate nucleus of cerebellum, lateral thalamus, and frontal cortex.[2],[3] FD is distinguished from Fahr's syndrome by the occurrence of the latter secondary to preexisting diseases such as hypoparathyroidism which can be detected as abnormality on blood investigations. The prevalence of FD is < 1/1,000,000.[4] In routine computed tomography (CT) of brain, basal ganglia calcification has been reported at a frequency of 0.3%–1.2%.[5]

Common clinical features of FD may include cognitive dysfunction, cerebellar signs, dysarthria, pyramidal signs, psychiatric illness, gait disorder, and sensory impairment.[6] Of these clinical features, the index patient is having psychiatric illness as the main presentation.

More extensive calcification correlates with the presence of psychiatric manifestations, but not with neurologic manifestations,[7] which could explain the presentation of the index patient with psychiatric manifestations. Nearly 50% of the patients suffering from FD present with movement disorders, among which  Parkinsonism More Details and chorea occur more commonly. Fewer than 10% of the patients present with tremor, dystonia, athetosis, or orofacial dyskinesia. Presence of symptoms is directly related to the amount of calcification in brain regions.[8]

There is no clear explanation for the calcification of bilateral basal ganglia in FD. The possible causes may include a variety of conditions such as metastatic deposition, calcium deposition secondary to the disruption of blood–brain barrier, or disturbance of the neuronal calcium metabolism.[9]

A whole-genome scan has revealed a locus for FD on chromosome 14q.[10] A study of regional blood flow to areas of calcification has revealed decreased blood flow to basal ganglia as well as cerebral cortex.[11] Disorders of calcium and phosphorus metabolism can cause bilateral basal ganglia calcification, especially in hypoparathyroidism.[12] If the basal ganglia calcification is secondary to hypoparathyroidism or any other metabolic abnormality, then it is labeled as Fahr's syndrome. However, if it is idiopathic, then it is better known as FD.[1] The values summarized in [Table 1] suggest the diagnosis of FD in the index patient, as all blood investigations were normal and CT brain was suggestive of idiopathic bilateral symmetrical basal ganglia calcification [Figure 1] and [Figure 2].
Table 1: The blood investigation findings of the index patient

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Figure 1: Computed tomography brain showing bilateral symmetrical basal ganglia calcification

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Figure 2: Computed tomography brain showing bilateral symmetrical basal ganglia calcification (highlighted)

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There are no specific diagnostic criteria for FD. Diagnosis depends on clinical history, clinical examination, neuroimaging mainly CT brain, and exclusion of any abnormality on blood biochemistry. According to Trautner et al., diagnosis of FD requires the presence of bilateral calcification of basal ganglia along with neuropsychiatric and extrapyramidal disorders with normal calcium and phosphorus metabolism.[13] Beall et al. proposed another definition for FD which includes seizures, rigidity, and dementia, with characteristic calcification of the basal ganglia.[14]

There are no specific clinical markers for FD. Pronicka et al. found that some patients have resistance to parathyroid hormone (PTH), which is manifested by a decreased phosphaturic effect of this hormone. This effect can be improved by beta-blockers, suggesting that the disease may be the result of the defect of adrenergic receptors and their impaired relationship with PTH receptors.[15]

Another biochemical abnormality noted is raised alkaline phosphatase (ALP) activity in the basal ganglia in the background of normal serum level of ALP. It may lead to precipitation of insoluble calcium phosphate salts in the brain tissue.[16]

Obsessive compulsive disorder (OCD) is seen in about 33% of patients with FD.[17] About 40% of patients with FD present with psychotic features.[7] Here, we report a rare case of OCD with psychotic features in the context of FD.

  Case Report Top

The index patient, an 18-year-old Muslim, unmarried female, belonging to a nuclear family, studying in 12th standard, premorbidly introvert, and having an insignificant family history of psychiatric disorders, presented with a duration of symptoms of 1½ years in the form of obsessive thoughts of hands and body being contaminated with dirt and germs, a compulsive act of repetitive hand washing about 15–20 times a day, spending about 5 min on hand washing each time, and taking bath for about 45 min to get rid of germs and to get relieved of anxiety associated with the thoughts of hands and body being contaminated. It would take her only 2 days to finish one soap for washing hands and taking bath. She also had obsessive thoughts about house doors being left open during night and would feel anxious. To relieve anxiety, she would get up four to five times every night to check whether the doors were closed or not. After doing it repetitively, she would feel relief. She had been on treatment from few psychiatrists, but old documents regarding the details of treatment history were lost and so were not available. The patient and the parents were not aware of the names of the medicines which the patient was receiving. A history of poor compliance to treatment was present due to financial issues. Her academic performance was also affected in the last 1½ years. Since last 4 months, the severity of symptoms increased. She began washing her hands about 25–30 times/day and was spending about 10 min to wash her hands each time, taking bath for about 1 to 1½ h every day. She was finishing one bathing soap every day. Her door-checking behavior also increased from four to five times each night to seven to eight times. Fifteen days back, the patient started muttering to self and started complaining of hearing of voices of many known and unknown people of both gender, talking with each other, discussing about her obsessions and compulsions, and planning to kill her if she went outside her house. She started refusing to go outside of the house. She became fearful and suspicious that her neighbors were talking about her and making plans to kill her. She became socially withdrawn, and her interaction with the family members drastically reduced. Her sleep and appetite were markedly disturbed for 8 days as she reported hearing “Azan” during night hours. At times, she would also complain of hearing sounds of footwear and bangles. Mental Status Examination revealed withdrawn behavior, increased reaction time with speech of low tone and volume, fearful mood, blunt affect, delusion of persecution and reference, third-person auditory hallucination, impaired concentration, impaired test and personal judgments, and grade 2 insight. She was admitted in the psychiatry ward and was started on capsule fluoxetine 20 mg once a day and tablet risperidone 2 mg at bedtime. The doses of fluoxetine and risperidone were increased gradually to 40 mg/day and 4 mg/day, respectively. Within 3 weeks of starting the treatment, the patient was denying having obsessive thoughts and auditory hallucinations. Her compulsive acts and hallucinatory behavior completely stopped. Her fearfulness reduced. The Yale Brown Obsessive Compulsive Scale (YBOCS) was used to categorize the severity of OCD.[18] YBOCS score reduced from 32 to 10, indicating reduction of OCD severity from extreme to mild. The Nurses' Observation Scale for Inpatient Evaluation (NOSIE) was used to assess an improvement in psychotic features.[19] The NOSIE consists of a total of thirty items with six factors, of which three are positive and three are negative factors. Positive factors consist of social competence, social interest, and personal neatness. Negative factors consist of irritability, manifest psychosis, and retardation. The NOSIE is usually administered 72 h after initial hospitalization and weekly thereafter. Each item is rated from 0 to 4, but item numbers 3, 14, 18, and 23 do not usually contribute to the total score. The total scale score ranges from 0 to 208. For total scale score or final score, either the sum of total scores of two raters or doubling the score of a single rater is recommended. On NOSIE, total patient assets (TOT) are calculated by the following equation: 96 + Total Positive Factors Score – Total Negative Factors Score.[19] In the index patient, on NOSIE, TOT progressively increased indicative of improvement in symptoms with time. Scores at initial assessment, at the end of the 1st week, at the end of the 2nd week, and at the end of the 3 week were 43, 68, 90, and 112, respectively [Table 2]. Progressively, scores of positive factors increased, whereas scores of negative factors decreased.
Table 2: Nurses' Observation Scale for Inpatient Evaluation scoring in the index patient

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Hence, on the NOSIE, there was an improvement in psychotic features. The patient was also having intermittent, bilateral, holocranial, throbbing headache of mild severity for the last 1½ years, with frequency of about once to twice a week, with each episode of headache lasting for about 15–20 min. Hence, CT brain was advised which was suggestive of “bilateral symmetrical basal ganglia calcification.” No other abnormality was detected on CT brain [Figure 1] and [Figure 2]. Neurologist's opinion was taken including taking blood investigations. The patient's blood investigations were normal [Table 1], which supported the diagnosis of FD. Routine investigations including complete blood count, random blood sugar, and liver and kidney function tests were also normal.

  Discussion Top

The above case has represented a rare occurrence of OCD with psychotic features in patients with FD. Among the anxiety disorders in patients suffering from FD, OCD is seen in about 33% of the cases. Less commonly seen psychiatric disorders in FD include personality change and substance abuse.[17]

The index patient was also having mild, throbbing headache for 1½ years. However, despite involvement of the basal ganglia bilaterally, the patient was not having any movement disorders and focal neurological signs. Nearly 50% of the patients with basal ganglia calcification exhibit neurological features such as headache, vertigo, movement disorders, stroke-like events, paresis, seizures, and syncope. Almost 40% of the patients with basal ganglia calcification initially present with psychiatric features, of which psychotic, mood, and cognitive symptoms occur commonly.[7] Patients with basal ganglia calcification in FD present with paranoid features between the ages of 20 and 40 years.[20] The main clinical features may include auditory hallucinations with occasional musical types, complex visual hallucinations, paranoid delusions, perceptual distortions, catatonia, and referential ideas.[17] König observed that 56% of cases with FD can present with extrapyramidal movement disorder.[7] Lauterbach et al. concluded that patients suffering from FD are more susceptible to neuroleptic malignant syndrome when treated with antipsychotic drugs.[17] Hence, there should be prudent use of psychotropic medications while managing the neuropsychiatric manifestations of FD.

  Conclusion Top

With the use of neuroimaging techniques in the field of psychiatry, more number of cases of FDs as well as other neurological disorders with neuropsychiatric manifestations can be detected. The present case depicts that prior to making the diagnosis of any psychiatric disorder, the psychiatrist needs to rule out the organic causes of psychiatric signs and symptoms. With an appropriate use of psychotropic drugs, it is possible to manage the neuropsychiatric manifestations of FD.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient and her parent have given their consent for the patient's images and other clinical information to be reported in the journal. The patient and her parent understand that the patient's name and initial will not be published, and due efforts will be made to conceal the patient's identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Burns K, Brodat H. Fahr's disease and psychosis. In: Sachdev PS, Keshavan MS, editors. Secondary Schizophrenia. 1st ed. Cambridge: Cambridge University Press; 2010. p. 358-66.  Back to cited text no. 1
Manyam BV. What is and what is not “Fahr's disease.” Parkinsonism Relat Disord 2005;11:73-80.  Back to cited text no. 2
Hecser L, Croitorescu L, Biris D, Palfi Siklodi K, Jung H. Fahr's Disease: Bilateral symmetrical striatopallidodentate calcification. Rom J Leg Med 2010;1:25-6.  Back to cited text no. 3
Manyam BV, Walters AS, Narla KR. Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry. Mov Disord 2001;16:258-64.  Back to cited text no. 4
Kobari M, Nogawa S, Sugimoto Y, Fukuuchi Y. Familial idiopathic brain calcification with autosomal dominant inheritance. Neurology 1997;48:645-9.  Back to cited text no. 5
Jankovic J. Parkinson Disease and Other Movement Disorders. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, editors. Bradley's Neurology in Clinical Practice. 7th ed. London: Elsevier; 2016. p. 1442.  Back to cited text no. 6
König P. Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry 1989;25:459-68.  Back to cited text no. 7
Shouyama M, Kitabata Y, Kaku T, Shinosaki K. Evaluation of regional cerebral blood flow in Fahr's disease with schizophrenia-like psychosis: A case report. AJNR Am J Neuroradiol 2005;26:2527-9.  Back to cited text no. 8
Malik R, Pandya VK, Naik D. Fahr's disease – A rare neurodegenerative disorder. Indian J Radiol Imaging 2004;14:383-4.  Back to cited text no. 9
  [Full text]  
Geschwind DH, Loginov M, Stern JM. Identification of a locus on chromosome 14q for idiopathic basal ganglia calcification (Fahr's disease). Am J Hum Genet 1999;65:764-72.  Back to cited text no. 10
Uygur GA, Liu Y, Hellman RS, Tikofsky RS, Collier BD. Evaluation of regional cerebral blood flow in massive intracerebral calcifications. J Nucl Med 1995;36:610-2.  Back to cited text no. 11
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Beall SS, Patten BM, Mallette L, Jankovic J. Abnormal systemic metabolism of iron, porphyrin, and calcium in Fahr's syndrome. Ann Neurol 1989;26:569-75.  Back to cited text no. 14
Pronicka E, Kulczycki J, Rowińska E, Kuran W. Abolished phosphaturic response to parathormone in adult patients with Fahr's disease and its restoration after propranolol administration. J Neurol 1988;235:185-7.  Back to cited text no. 15
Brannan TS, Burger AA, Chaudhary MY. Bilateral basal ganglia calcifications visualised on CT scan. J Neurol Neurosurg Psychiatry 1980;43:403-6.  Back to cited text no. 16
Lauterbach EC, Cummings JL, Duffy J, Coffey CE, Kaufer D, Lovell M, et al. Neuropsychiatric correlates and treatment of lenticulostriatal diseases: A review of the literature and overview of research opportunities in Huntington's, Wilson's, and Fahr's diseases. A report of the ANPA committee on research. American neuropsychiatric association. J Neuropsychiatry Clin Neurosci 1998;10:249-66.  Back to cited text no. 17
Rapp AM, Bergman RL, Piacentini J, McGuire JF. Evidence-based assessment of obsessive-compulsive disorder. J Cent Nerv Syst Dis 2016;8:13-29.  Back to cited text no. 18
Honigfeld G, Klett CJ. The nurses' observation scale for inpatient evaluation: A new scale for measuring improvement in chronic schizophrenia. J Clin Psychol 1965;21:65-71.  Back to cited text no. 19
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  [Figure 1], [Figure 2]

  [Table 1], [Table 2]


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