Case Report

Fahr's Disease Vs. Drug Induced Movement Disorder:

Case report and Literature Review
Abdulaziz Thamer Alshomrani MD, Raafat Mahmoud Shuqdar MD

Department of Psychiatry and Addiction Medicine, College of Medicine,

Imam University Riyadh, Kingdom of Saudi Arabia

Abstract
Fahr's disease is a rare idiopathic degenerative neurological disorder, which can be present in
different heterogeneous manifestations and characterized by bilateral symmetrical cerebral
calcification. These presentations usually involve basal ganglia, manifested with multiple
neuropsychiatric disorders. We described a patient with acute onset schizophrenia, who
developed abnormal choreoathetoid movement three years later. His brain imaging studies
showed extensive bilateral cerebral calcification, the patient was diagnosed with a sporadic
case of Fahr's disease. Brain imaging should be considered in acute onset schizophrenia to
rule out organic causes. Fahr’s disease can be a differential diagnosis when extrapyramidal
symptoms and abnormal movement appear and are not improved on treatment

Key Words: Fahr's disease, Bilateral cerebral calcification, Parkinsonism; Schizophrenia,

Saudi Arabia.

Journal of Taibah University Medical Sciences 2010; 5 (2): 120 - 126

Correspondence to:
Dr. Abdulaziz Alshomrani
Assistant Professor, College of Medicine
Imam University Consultant of Psychiatry and Addiction Medicine
7544 Riyadh 13317 Kingdom of Saudi Arabia
+966503298929
 +96612590209
 [email protected]

Introduction first to describe this kind of calcification nor

did he contribute significantly to the

F ahr's disease is Familial Idiopathic Basal

Ganglia Calcification characterized by
understanding of this disorder1. Most cases
of Fahr's disease are autosomal dominant,
bilateral intracranial calcification and but familial and sporadic cases had been
associated with neuropsychiatric documented and autosomal recessive cases
manifestations. Fahr's disease referred to had been reported2,3. In dominant cases,
Fahr's (German neurologist) who described most of afflicted family members by cerebral
a case with bilateral calcifications in the calcification are asymptomatic and even
basal ganglia and other parts of the brain in some of them may not show calcification
1930, despite the fact that he was not the changes till they get older. Although there is

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 Abdulaziz Thamer Alshomrani and Raafat Mahmoud Shuqdar

no strong evidence and some researchers didn't fulfill his medication and his
doubt it, TULIP1 gene on chromosome 14q condition deteriorated progressively inform
has been suggested to be the responsible of positive and negative symptoms' of
gene (For what? Farah's disease?) 4- 7. schizophrenia (and he has been retired?
Fahr's disease cases are usually diagnosed What does this mean? The patient retired
by exclusion of other causes of Fahr’s because of the negative effect his case had or
syndrome e.g. hypoparathyroidism, because of him not complying with his
neurobrucellosis, CNS TB, toxoplasmosis, medication?).
Wilson disease and Huntington’s disease. The patient is the 4th amongst his 10
siblings, 7 males and 3 females, whom were
Case Report all healthy with no history of psychiatric or
A 26-year-old single retired soldier, neurological disorders. His parents denied
schizophrenic patient, admitted to any heredity of similar conditions of
psychiatric ward on 2004 after seeing the abnormal movements in the family.
outpatient psychiatric clinics in the Military The examination of the patient's
Hospital with his family. The patient’s neurological and mental state showed a
family reported that he started to develop calm, cooperative, older than his stated age,
abnormal involuntary movement of the thin, unclean dressing with poor self
trunk and upper limbs which worsen hygiene patient.
progressively and interfered with his daily The patient demonstrated an abnormal
live activity during the last six months prior bizarre choreoathetoid movement of trunk
to his visit. They also admitted that he had and upper limbs, dysartheric speech and
low self care, aggressive behavior but euthymic mood with appropriate affect and
denied any recent history of thought, his perceptual feeling intact. Upon
perception or cognitive abnormalities. examinations of his thought and cognition
Actually, the patient was seen in the out- revealed no abnormalities. Other physical
patient clinics before, where his condition examinations were unremarkable.
was diagnosed as drug induced movement Upon the patient's admission at a
disorder -extrapyramidal side effect neurologist was consulted and an MRI scan
secondary to his antipsychotic medication- and the following investigations were done:
(Resperidone 4 m.g, Procyclidine 5 m.g and CBC, U&E, LFT, parathyroid hormone, HIV,
Fluanxol depot 30 mg every 4 weeks), syphilis, Hepatitis viruses screening& urine
antipsychotic discontinued and his drug screening.
anticholinergic drugs increased without any All of the patient's lab tests were within
improvement. normal limits and his MRI showed the
The patient was diagnosed as a case of following: Calcific changes, which involved
schizophrenia for 4 years after he suddenly basal ganglia bilaterally, both dentate nuclei
developed paranoid delusions with and subcortical area and centrum semiovale
aggressive behavior. During this period the of both hemispheres (Figure 1). After that, a
patient was admitted three times in three radiologist asked for a CT scan, which
different tertiary hospitals, diagnosed as a confirmed the finding and revealed
case of Paranoid schizophrenia, and extension of calcification to caudate nuclei,
prescribed different kinds of typical and inferior aspect of thalami and cerebellum
atypical antipsychotic medications, but he (Figure 2, 3).

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 Fahr's disease vs. drug induced movement disorder

Figure 1: MRI Brain demonstrated calcific changes which involved basal ganglia bilaterally.

Figure 2: CT Brain revealed calcification of caudate nuclei.

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 Abdulaziz Thamer Alshomrani and Raafat Mahmoud Shuqdar

Figure 3: CT Brain revealed extension of calcification to cerebellum

Therefore, the patient's condition was cerebellum. Hence, one of the other names
diagnosed as Fahr’s Disease after the of the disease is Bilateral Striopallidodentate
exclusion of other disorders like, Wilson Calcification (BSPDC). However,
disease and metabolic disorder related to calcification can involve centrum ovale.
parathyroid hormone. Thalami, cerebral cortex, subcortex and no
Upon admission to (Where?), the patient part of the brain could be spared. The
received a small dose of clozapine, which extension of calcification is directly related
was discontinued, then Tetrabenazine was to the psychiatric manifestations, but not to
prescribed by neurologist, who added the neurological ones, which could explain
Sodium valproate later. The patient's the presentation of the acute psychosis in
condition did not show any marked changes the patient in this study8. Functional brain
and was discharged in order to be admitted neuroimaging showed different inconsistent
to a long-term rehabilitation facility. findings according to the clinical
However, after that, patient was seen once presentation, showing part of the brain
at an ER with the same presentation, but no affected by calcification9- 11.
more visits were made since then Postmortem histology studies of the lesions
showed mainly calcium compositions, but
Discussion other items like phosphorus, iron,
magnesium, aluminum and zinc had been
The most common sites of calcifications are identified as well. However, no important
basal ganglia and dentate nuclei of

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 Fahr's disease vs. drug induced movement disorder

clinical issues were related to these presentation and progressive course of the
components 12,13. illness over long period) or just an early
Fahr's disease has a broad spectrum of psychiatric presentation of Fahr's disease
clinical neurological presentation. Patients that preceded the movement disorder. Since
can be presented with movement disorders, the presentation is sudden, no family history
executive and cognitive impairment, of schizophrenia and because of the
seizures, pain, pyramidal symptoms, extensive calcification that was most likely
cerebellar signs and other different to be there at the start of psychosis, the
presentation, depending on which part of researchers suggest the later diagnosis,
the brain was affected by lesion 1,14. Manyam starting psychotic patients on antipsychotic
BV et al. 2001 analyzed presentations of need close monitoring and observation for
sixty one symptomatic cases and found that any side effect development like movement
movement disorders were the most common abnormalities especially if there is no
presentation, which accounted for 55% of response to anticholinergic medications.
clinical presentations Parkinsonism 57%, Cerebral imaging is strongly indicated when
chorea 19%, tremor 8%, dystonia 8%, patients without a family history of
athetosis 5% and orofacial dyskinesia 3% 15. psychosis are presented with sudden
About 40% of patients with Fahr's disease psychosis or a case of unordinary movement
present initially with psychiatric features16. complication to antipsychotic medication
Cognitive, psychotic, and mood disorders not responding to the usual interventions.
are the most common presentations. Fahr's Professionals in the psychiatric field should
disease may present as a progressive be more educated about other differentials
subcortical dementia in elder patients and and causes of medications' side effects in
those who were presented earlier with other advance.
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