- Email: [email protected]
Fahr's disease: A rare neurological disease
apollo medicine 12 (2015) 148–151
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/apme
Case Report
Fahr's disease: A rare neurological disease Dinesh Chaudhari
a,
*, Pushpendra Nath Renjen b
a
Internal Medicine Resident, Institute of Neurosciences/Internal Medicine, Indraprastha Apollo Hospitals, New Delhi 110076, India b Sr. Consultant Neurologist & Academic Coordinator, Institute of Neurosciences, Indraprastha Apollo Hospitals, New Delhi 110076, India
article info
abstract
Article history:
A 40-year-old unmarried female presented with abnormal involuntary choreo-athetoid
Received 2 April 2015
movements involving both upper limbs for 5 years along with features, such as bouts of
Accepted 1 May 2015
disorientation, anxiety, personality changes, reckless behaviour, inappropriate laughter and
Available online 12 June 2015
progressive decline in the neurological status. On neurological examination, her speech was dysarthric with mild choreiform movements involving both upper limbs. She had MMSE
Keywords:
score of 20/30. MRI scans of the brain plain T1- and T2-weighted axial and flair coronal
Fahr's syndrome
images were obtained. It showed calcifications as hyper-intense lesions on T1W and hypo-
Dentate nucleus calcification
intense T2W lesions in bilateral basal ganglion and bilateral dentate nuclei of cerebellum,
Bilateral basal ganglia calcification
that is consistent with Fahr's syndrome.
Movement disorder
# 2015 Indraprastha Medical Corporation Ltd. Published by Elsevier B.V. All rights reserved.
1.
Case report
A 40-year-old unmarried female presented to our tertiary care hospital with abnormal involuntary movements involving both upper limbs for last 5 years, but no such movements were noticed in the lower limbs. These movements were suggestive of choreo-athetoid movements. The family also noticed that for the same period, the patient had features, such as bouts of disorientation, anxiety, personality changes, reckless behaviour and inappropriate laughter. For these symptoms, she was under the care of a psychiatrist, and was put under some medication. Despite this, she was having a progressive decline in the neurological status. There was no significant family history of mental illness, dementia or any other physical illness. She did not have any
features of depression. On neurological examination, the patient was alert, oriented to time, place and person. She had bursts of laughter. Cranial nerves were normal, as well as power and sensations were found to be normal. Her speech was dysarthric and mild choreic involving both upper limbs. She had a score of 20/30 on the mini mental state examination. No parietal lobe signs, Parkinson's features or any other features suggestive of long tract signs. In order to exclude other differentials that may lead to secondary intracranial calcification, the patient went through a series of blood tests that included completed blood picture, erythrocyte sedimentary rate, serum iron studies, serum calcium and phosphate level, serum parathyroid hormone level, thyroid hormone level, serum creatinine, serum ceruloplasmin and urinary copper, serum lactate and pyruvic acid, serological tests for syphilis, HIV and CSF study to rule out
* Corresponding author. Mobile: +91 8585940681. E-mail address: [email protected] (D. Chaudhari). http://dx.doi.org/10.1016/j.apme.2015.05.009 0976-0016/# 2015 Indraprastha Medical Corporation Ltd. Published by Elsevier B.V. All rights reserved.
149
apollo medicine 12 (2015) 148–151
other metabolic, infectious causes. All these tests were essentially normal in our patient. Her electroencephalogram was also done, which was normal. MRI of the brain plain T1 & T2 weighted axial and flair coronal images were obtained. It showed calcifications as hyper-intense lesions on T1W and hypo-intense T2W lesions in bilateral basal ganglion and bilateral dentate nuclei of cerebellum that is consistent with Fahr's syndrome (Fig. 1).
2.
Table 1 – Etiological manifestations of Fahr's syndrome. S. no. 1
Endocrine disorders
Idiopathic hypoparathyroidism Secondary hypoparathyroidism Pseudohypoparathyroidism Pseudopseudohypoparathyroidism Hyperparathyroidism
2
Adult onset neurodegenerative conditions
Neurodegeneration with brain iron accumulation disease Neuroferritinopathy Polycystic lipomembranous Osteodysplasia with sclerosing Leukoencephalopathy
3
Infectious disease
Intrauterine and perinatal infection Cockayne syndrome Type 1 Cockayne syndrome Type 2
4
Inherited or early onset syndrome
Aicardi-Goutieres syndrome Tuberous sclerosis complex Brucellosis Coat's disease
Discussion
Basal ganglia calcification is also known as Fahr's disease or Fahr's syndrome. It is a rare inherited or sporadic neurological disorder with a worldwide prevalence of <1/1,000,000.1–3 It was first described by German neurologist Karl Theodor Fahr in 1930.4 It is characterized by abnormal deposition of calcium in areas of the brain that control movements including basal ganglia, thalamus, dentate nucleus, cerebral cortex, cerebellum, subcortical white matter and hippocampus.5 Most cases present with extra pyramidal symptoms initially. Additionally, they may present with cerebellar dysfunction, speech difficulty, dementia and neuropsychiatric symptoms.6 Fahr's syndrome typically manifests in third or fourth decade of life. 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.1,7 A locus at 14q (IBGC1) has been suggested to be involved commonly. At the molecular level, calcification generally develops within the vessel wall and in the perivascular space, ultimately extending to the neuron. Due to defective iron transport and free radical production, tissue damage occurs which leads to the initiation of calcification. It occurs secondarily around a Nidus composed of mucopolysaccharide and related substances. Progressive basal ganglia mineralization tends to compress the vessel lumen, thus initiating a cycle of impaired blood flow, neural tissue injury and mineral deposition.8 Endocrine disorders, particularly parathyroid disturbances are most commonly associated with Fahr's syndrome. Many neurodegenerative, inherited and infectious conditions have also been linked to etiological manifestations of Fahr's syndrome (Table 1).8 Presentation of disease could vary with the age and course of illness (Table 2). The clinical features usually include psychosis, cognition impairment, and symptoms of mood disorders, epileptic seizures and dementia.9,10 Excessive extensive intracranial calcification is also associated with psychiatric manifestation.10 Cummings et al. (1983) have described two psychiatric symptoms in basal ganglion mineralization, as cases with psychiatric symptoms appearing in the early period (mean 31 years) and in late period (mean 49 years). Motor and cognitive symptoms in cases with basal ganglion mineralization, which appears in the late period, are more significant.11 Pallidal lesions may cause disorders related to motivation, judgement and insight in humans. Idiopathic basal ganglia calcification may lead to various neuropsychiatric symptoms. Patients with basal ganglia calcification present initially with psychiatric symptoms. When psychosis occurs in Fahr's disease, it usually presents in persons 20–40 years of age as
Etiologies
Table 2 – Clinical features of Fahr's syndrome. S. no.
Clinical features
1
Neurological
Loss of consciousness Tetany Seizures Epileptic disorder Gait disorder Spasticity Speech impairment Dementia Myoclonus Coma Paroxysmal choreoathetosis Dystonic choreoathetosis Papilledema of intracranial hypertension Pleocytosis of CSF
2
Movement disorder
Clumsiness Fatigability Unsteady gait Involuntary movements and muscle cramping
3
Neuropsychiatric features
Psychosis Depression Apoplexia Deterioration of intelligence Inability to make decisions
a part of the so-called early adult-onset Fahr's disease. Classic schizophrenia-like symptoms have been described, including auditory hallucinations, paranoid delusions, delusions of reference and catatonia. Psychotic symptoms that are not typically associated with schizophrenia have also been described, including musical auditory hallucinations and complex visual hallucinations.12 The pathophysiology of psychosis in Fahr's disease remains unknown, though previous studies have found a
150
apollo medicine 12 (2015) 148–151
[(Fig._1)TD$IG]
Fig. 1 – CT head of Fahr's disease showing bilateral symmetrical calcification of basal ganglia, thalamus, cerebellum and frontal cortex.
decreased cerebral blood flow matching the distribution of calcification or decreased perfusion in the cortex, which may reflect secondary deficits due to calcification.13,14 Diagnosis of Fahr's disease is based on combinations of clinical features, brain imaging and exclusion of other causes of intracranial calcifications.15
with Fahr's syndrome. Great caution is indicated when using lithium, because it can further increase the risk of seizures in patients with Fahr's syndrome. Treatment strategies involving the use of carbamazepine, benzodiazepines and barbiturates may exacerbate underlying gait disorders.17
5. 3.
Conclusions
Diagnostic criteria
Diagnostic criteria of Fahr's syndrome have been modified and derived from Moskowitz et al. (1971), Ellie et al. (1989) and Manyam (2005), and that can be stated as follows: – Bilateral calcification of the basal ganglia visualized on neuroimaging. Other brain regions may also be observed. – Progressive neurologic dysfunction, which generally includes a movement disorder and/or neuropsychiatric manifestations. Age of onset is typically in the fourth or fifth decade, although this dysfunction may also be present in childhood. – Absence of biochemical abnormalities and somatic features suggestive of a mitochondrial or metabolic disease or other systemic disorder. – Absence of an infectious, toxic or traumatic cause. – Family history consistent with autosomal dominant inheritance.
1. The present paper emphasizes that diagnosis of Fahr's disease can be thought of in a patient presenting with neuropsychiatric manifestations along with a brain calcification pattern which is suggestive of Fahr's disease, in the absence of biochemical abnormalities, infectious, toxic or traumatic causes. 2. Bilateral basal ganglia calcification along with bilateral cerebellar (particularly dentate nucleus) calcification strongly favours the diagnosis of Fahr's disease. 3. Family history should be sought for in all such patients. 4. Clonazepam and atypical antipsychotics such as quetiapine also offer a distinct advantage in treating patients with Fahr's syndrome.
Conflicts of interest The authors have none to declare.
4.
Treatment
Several approaches based on diverse biological theories and small-scale clinical experiences have been proposed. Pharmacological treatment should be used to improve anxiety, depression and obsessive compulsive disorder and to alleviate dystonia.16 Clonazepam and atypical antipsychotics such as quetiapine also offer a distinct advantage in treating patients
references
1. Bilateral striopallidodentate calcinosis. http://www.orpha. net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=1980. 2. Manyam BV, Walters AS, Narla KR. Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry. Mov Disord. 2001;16(2): 258–264.
apollo medicine 12 (2015) 148–151
3. Ellie E, Julien J, Ferrer X. Familial idiopathic striopallidodentate calcifications. Neurology. 1989;39(3): 381–385. 4. Fahr T. Idiopathische verkalkung der hirngefässe. Zentrabl Allg Pathol. 1930;50:129–133. 5. Ahad MA, Bala C, Karim S. Fahr's syndrome. Bangladesh Med J Khulna. 2013;45(1–2):33–35. 6. Chiu H, Lam L, Shum P, Li K. Idiopathic calcification of the basal ganglia. Postgrad Med J. 1993;69(807):68–70. 7. Yamada N, Hayashi T. Asymptomatic familial basal ganglia calcification with autosomal dominant inheritance: a family report. No to hattatsu. Brain Dev. 2000;32(6):515–519. 8. Saleem S, Aslam HM, Anwar M. Fahr's syndrome: literature review of current evidence. Orphanet J Rare Dis. 2013;8:156. 9. Modrego PJ, Mojonero J, Serrano M, Fayed N. Fahr's syndrome presenting with pure and progressive presenile dementia. Neurol Sci. 2005;26:367–369. 10. König P. Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry. 1989;25:459–468. 11. Cummings JL, Gosenfeld LF, Houlihan JP, McCaffrey T. Neuropsychiatric disturbances associated with idiopathic
12.
13.
14.
15.
16.
17.
151
calcification of the basal ganglia. Biol Psychiatry. 1983;18: 591–601. Lauterbach EC, Cummings JL, Duffy J, 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. J Neuropsychiatry Clin Neurosci. 1998;10:249–266. Shoyama M, Kitabata Y, Kaku T, Shinosaki K. Evaluation of regional cerebral blood flow in Fahr disease with schizophrenia-like psychosis: a case report. Am J Neuroradiol. 2005;26:2527–2529. Hempel A, Henze M, Berghoff C, Garcia N, Ody R, Schröder J. PET findings and neuropsychological deficits in a case of Fahr's disease. Psychiatry Res. 2001;108:133–140. Lam JS, Fong SY, Yiu GC, Wing YK. Fahr's disease: a differential diagnosis of frontal lobe syndrome. Hong Kong Med J. 2007;13:75–77. Sobrid MJ, Hopfer S, Geschwind DH. Familial Idiopathic Basal Ganglia Calcification: GeneReviewsTM [Internet]. Seattle (WA): University of Washington; 2004 [Updated 2007 Sep 20]. Lauterbach EC. Psychiatric Management in Neurological Disease. American Psychiatric Press; 2005 In: http://books.google.com.pk/ books?id=sE1qlE751XcC.
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/apme
Case Report
Fahr's disease: A rare neurological disease Dinesh Chaudhari
a,
*, Pushpendra Nath Renjen b
a
Internal Medicine Resident, Institute of Neurosciences/Internal Medicine, Indraprastha Apollo Hospitals, New Delhi 110076, India b Sr. Consultant Neurologist & Academic Coordinator, Institute of Neurosciences, Indraprastha Apollo Hospitals, New Delhi 110076, India
article info
abstract
Article history:
A 40-year-old unmarried female presented with abnormal involuntary choreo-athetoid
Received 2 April 2015
movements involving both upper limbs for 5 years along with features, such as bouts of
Accepted 1 May 2015
disorientation, anxiety, personality changes, reckless behaviour, inappropriate laughter and
Available online 12 June 2015
progressive decline in the neurological status. On neurological examination, her speech was dysarthric with mild choreiform movements involving both upper limbs. She had MMSE
Keywords:
score of 20/30. MRI scans of the brain plain T1- and T2-weighted axial and flair coronal
Fahr's syndrome
images were obtained. It showed calcifications as hyper-intense lesions on T1W and hypo-
Dentate nucleus calcification
intense T2W lesions in bilateral basal ganglion and bilateral dentate nuclei of cerebellum,
Bilateral basal ganglia calcification
that is consistent with Fahr's syndrome.
Movement disorder
# 2015 Indraprastha Medical Corporation Ltd. Published by Elsevier B.V. All rights reserved.
1.
Case report
A 40-year-old unmarried female presented to our tertiary care hospital with abnormal involuntary movements involving both upper limbs for last 5 years, but no such movements were noticed in the lower limbs. These movements were suggestive of choreo-athetoid movements. The family also noticed that for the same period, the patient had features, such as bouts of disorientation, anxiety, personality changes, reckless behaviour and inappropriate laughter. For these symptoms, she was under the care of a psychiatrist, and was put under some medication. Despite this, she was having a progressive decline in the neurological status. There was no significant family history of mental illness, dementia or any other physical illness. She did not have any
features of depression. On neurological examination, the patient was alert, oriented to time, place and person. She had bursts of laughter. Cranial nerves were normal, as well as power and sensations were found to be normal. Her speech was dysarthric and mild choreic involving both upper limbs. She had a score of 20/30 on the mini mental state examination. No parietal lobe signs, Parkinson's features or any other features suggestive of long tract signs. In order to exclude other differentials that may lead to secondary intracranial calcification, the patient went through a series of blood tests that included completed blood picture, erythrocyte sedimentary rate, serum iron studies, serum calcium and phosphate level, serum parathyroid hormone level, thyroid hormone level, serum creatinine, serum ceruloplasmin and urinary copper, serum lactate and pyruvic acid, serological tests for syphilis, HIV and CSF study to rule out
* Corresponding author. Mobile: +91 8585940681. E-mail address: [email protected] (D. Chaudhari). http://dx.doi.org/10.1016/j.apme.2015.05.009 0976-0016/# 2015 Indraprastha Medical Corporation Ltd. Published by Elsevier B.V. All rights reserved.
149
apollo medicine 12 (2015) 148–151
other metabolic, infectious causes. All these tests were essentially normal in our patient. Her electroencephalogram was also done, which was normal. MRI of the brain plain T1 & T2 weighted axial and flair coronal images were obtained. It showed calcifications as hyper-intense lesions on T1W and hypo-intense T2W lesions in bilateral basal ganglion and bilateral dentate nuclei of cerebellum that is consistent with Fahr's syndrome (Fig. 1).
2.
Table 1 – Etiological manifestations of Fahr's syndrome. S. no. 1
Endocrine disorders
Idiopathic hypoparathyroidism Secondary hypoparathyroidism Pseudohypoparathyroidism Pseudopseudohypoparathyroidism Hyperparathyroidism
2
Adult onset neurodegenerative conditions
Neurodegeneration with brain iron accumulation disease Neuroferritinopathy Polycystic lipomembranous Osteodysplasia with sclerosing Leukoencephalopathy
3
Infectious disease
Intrauterine and perinatal infection Cockayne syndrome Type 1 Cockayne syndrome Type 2
4
Inherited or early onset syndrome
Aicardi-Goutieres syndrome Tuberous sclerosis complex Brucellosis Coat's disease
Discussion
Basal ganglia calcification is also known as Fahr's disease or Fahr's syndrome. It is a rare inherited or sporadic neurological disorder with a worldwide prevalence of <1/1,000,000.1–3 It was first described by German neurologist Karl Theodor Fahr in 1930.4 It is characterized by abnormal deposition of calcium in areas of the brain that control movements including basal ganglia, thalamus, dentate nucleus, cerebral cortex, cerebellum, subcortical white matter and hippocampus.5 Most cases present with extra pyramidal symptoms initially. Additionally, they may present with cerebellar dysfunction, speech difficulty, dementia and neuropsychiatric symptoms.6 Fahr's syndrome typically manifests in third or fourth decade of life. 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.1,7 A locus at 14q (IBGC1) has been suggested to be involved commonly. At the molecular level, calcification generally develops within the vessel wall and in the perivascular space, ultimately extending to the neuron. Due to defective iron transport and free radical production, tissue damage occurs which leads to the initiation of calcification. It occurs secondarily around a Nidus composed of mucopolysaccharide and related substances. Progressive basal ganglia mineralization tends to compress the vessel lumen, thus initiating a cycle of impaired blood flow, neural tissue injury and mineral deposition.8 Endocrine disorders, particularly parathyroid disturbances are most commonly associated with Fahr's syndrome. Many neurodegenerative, inherited and infectious conditions have also been linked to etiological manifestations of Fahr's syndrome (Table 1).8 Presentation of disease could vary with the age and course of illness (Table 2). The clinical features usually include psychosis, cognition impairment, and symptoms of mood disorders, epileptic seizures and dementia.9,10 Excessive extensive intracranial calcification is also associated with psychiatric manifestation.10 Cummings et al. (1983) have described two psychiatric symptoms in basal ganglion mineralization, as cases with psychiatric symptoms appearing in the early period (mean 31 years) and in late period (mean 49 years). Motor and cognitive symptoms in cases with basal ganglion mineralization, which appears in the late period, are more significant.11 Pallidal lesions may cause disorders related to motivation, judgement and insight in humans. Idiopathic basal ganglia calcification may lead to various neuropsychiatric symptoms. Patients with basal ganglia calcification present initially with psychiatric symptoms. When psychosis occurs in Fahr's disease, it usually presents in persons 20–40 years of age as
Etiologies
Table 2 – Clinical features of Fahr's syndrome. S. no.
Clinical features
1
Neurological
Loss of consciousness Tetany Seizures Epileptic disorder Gait disorder Spasticity Speech impairment Dementia Myoclonus Coma Paroxysmal choreoathetosis Dystonic choreoathetosis Papilledema of intracranial hypertension Pleocytosis of CSF
2
Movement disorder
Clumsiness Fatigability Unsteady gait Involuntary movements and muscle cramping
3
Neuropsychiatric features
Psychosis Depression Apoplexia Deterioration of intelligence Inability to make decisions
a part of the so-called early adult-onset Fahr's disease. Classic schizophrenia-like symptoms have been described, including auditory hallucinations, paranoid delusions, delusions of reference and catatonia. Psychotic symptoms that are not typically associated with schizophrenia have also been described, including musical auditory hallucinations and complex visual hallucinations.12 The pathophysiology of psychosis in Fahr's disease remains unknown, though previous studies have found a
150
apollo medicine 12 (2015) 148–151
[(Fig._1)TD$IG]
Fig. 1 – CT head of Fahr's disease showing bilateral symmetrical calcification of basal ganglia, thalamus, cerebellum and frontal cortex.
decreased cerebral blood flow matching the distribution of calcification or decreased perfusion in the cortex, which may reflect secondary deficits due to calcification.13,14 Diagnosis of Fahr's disease is based on combinations of clinical features, brain imaging and exclusion of other causes of intracranial calcifications.15
with Fahr's syndrome. Great caution is indicated when using lithium, because it can further increase the risk of seizures in patients with Fahr's syndrome. Treatment strategies involving the use of carbamazepine, benzodiazepines and barbiturates may exacerbate underlying gait disorders.17
5. 3.
Conclusions
Diagnostic criteria
Diagnostic criteria of Fahr's syndrome have been modified and derived from Moskowitz et al. (1971), Ellie et al. (1989) and Manyam (2005), and that can be stated as follows: – Bilateral calcification of the basal ganglia visualized on neuroimaging. Other brain regions may also be observed. – Progressive neurologic dysfunction, which generally includes a movement disorder and/or neuropsychiatric manifestations. Age of onset is typically in the fourth or fifth decade, although this dysfunction may also be present in childhood. – Absence of biochemical abnormalities and somatic features suggestive of a mitochondrial or metabolic disease or other systemic disorder. – Absence of an infectious, toxic or traumatic cause. – Family history consistent with autosomal dominant inheritance.
1. The present paper emphasizes that diagnosis of Fahr's disease can be thought of in a patient presenting with neuropsychiatric manifestations along with a brain calcification pattern which is suggestive of Fahr's disease, in the absence of biochemical abnormalities, infectious, toxic or traumatic causes. 2. Bilateral basal ganglia calcification along with bilateral cerebellar (particularly dentate nucleus) calcification strongly favours the diagnosis of Fahr's disease. 3. Family history should be sought for in all such patients. 4. Clonazepam and atypical antipsychotics such as quetiapine also offer a distinct advantage in treating patients with Fahr's syndrome.
Conflicts of interest The authors have none to declare.
4.
Treatment
Several approaches based on diverse biological theories and small-scale clinical experiences have been proposed. Pharmacological treatment should be used to improve anxiety, depression and obsessive compulsive disorder and to alleviate dystonia.16 Clonazepam and atypical antipsychotics such as quetiapine also offer a distinct advantage in treating patients
references
1. Bilateral striopallidodentate calcinosis. http://www.orpha. net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=1980. 2. Manyam BV, Walters AS, Narla KR. Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry. Mov Disord. 2001;16(2): 258–264.
apollo medicine 12 (2015) 148–151
3. Ellie E, Julien J, Ferrer X. Familial idiopathic striopallidodentate calcifications. Neurology. 1989;39(3): 381–385. 4. Fahr T. Idiopathische verkalkung der hirngefässe. Zentrabl Allg Pathol. 1930;50:129–133. 5. Ahad MA, Bala C, Karim S. Fahr's syndrome. Bangladesh Med J Khulna. 2013;45(1–2):33–35. 6. Chiu H, Lam L, Shum P, Li K. Idiopathic calcification of the basal ganglia. Postgrad Med J. 1993;69(807):68–70. 7. Yamada N, Hayashi T. Asymptomatic familial basal ganglia calcification with autosomal dominant inheritance: a family report. No to hattatsu. Brain Dev. 2000;32(6):515–519. 8. Saleem S, Aslam HM, Anwar M. Fahr's syndrome: literature review of current evidence. Orphanet J Rare Dis. 2013;8:156. 9. Modrego PJ, Mojonero J, Serrano M, Fayed N. Fahr's syndrome presenting with pure and progressive presenile dementia. Neurol Sci. 2005;26:367–369. 10. König P. Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry. 1989;25:459–468. 11. Cummings JL, Gosenfeld LF, Houlihan JP, McCaffrey T. Neuropsychiatric disturbances associated with idiopathic
12.
13.
14.
15.
16.
17.
151
calcification of the basal ganglia. Biol Psychiatry. 1983;18: 591–601. Lauterbach EC, Cummings JL, Duffy J, 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. J Neuropsychiatry Clin Neurosci. 1998;10:249–266. Shoyama M, Kitabata Y, Kaku T, Shinosaki K. Evaluation of regional cerebral blood flow in Fahr disease with schizophrenia-like psychosis: a case report. Am J Neuroradiol. 2005;26:2527–2529. Hempel A, Henze M, Berghoff C, Garcia N, Ody R, Schröder J. PET findings and neuropsychological deficits in a case of Fahr's disease. Psychiatry Res. 2001;108:133–140. Lam JS, Fong SY, Yiu GC, Wing YK. Fahr's disease: a differential diagnosis of frontal lobe syndrome. Hong Kong Med J. 2007;13:75–77. Sobrid MJ, Hopfer S, Geschwind DH. Familial Idiopathic Basal Ganglia Calcification: GeneReviewsTM [Internet]. Seattle (WA): University of Washington; 2004 [Updated 2007 Sep 20]. Lauterbach EC. Psychiatric Management in Neurological Disease. American Psychiatric Press; 2005 In: http://books.google.com.pk/ books?id=sE1qlE751XcC.