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Fahr's Disease: An Incidental Finding in a Case Presenting with Psychosis

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  • University College of Medical Sciences & Guru Teg Bahadur Hospital,Delhi,India

Abstract and Figures

Fahr's syndrome refers to a rare syndrome characterized by symmetrical and bilateral intracranial calcification. We present a 24-year-old male with Fahr disease, presenting with psychosis and recurrent seizures of generalized tonic clonic type, but lacking evidence of a metabolic disorder. His neurological examination was normal. MRI Brain of the patient revealed symmetrical large areas and foci of calcification in bilateral basal ganglia, thalami, cerebellar paren-chyma and subcortical regions of bilateral cerebral hemispheres. When screening other family members, we detected Fahr syndrome in his elder brother with hypocalcemia. Fahr disease may present with psychosis, have pronounced posi-tive brain imaging findings. Magnetic Resonance Imaging can also be effective screening tool for adult relatives (Ger-man J Psychiatry 2010; 13 (2): 86-90).
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Reprinted from the German Journal of Psychiatry · http://www. gjpsy. uni-goettingen. de · ISSN 1433-1055
Case Report
Fahr’s Disease: An Incidental Finding in a
Case Presenting with Psychosis
S. Srivastava, Manjeet S. Bhatia, V. Sharma, S. Mahajan, G. Rajender
Department of Psychiatry, University College of Medical Sciences (UCMS) and
G. T. B. Hospital, Dilshad Garden, University of Delhi, India
Corresponding author: Dr. M. S Bhatia, 1 Naraina Vihar, New Delhi-110028, India
Abstract
Fahr’s syndrome refers to a rare syndrome characterized by symmetrical and bilateral intracranial calcification. We
present a 24-year-old male with Fahr disease, presenting with psychosis and recurrent seizures of generalized tonic
clonic type, but lacking evidence of a metabolic disorder. His neurological examination was normal. MRI Brain of the
patient revealed symmetrical large areas and foci of calcification in bilateral basal ganglia, thalami, cerebellar paren-
chyma and subcortical regions of bilateral cerebral hemispheres. When screening other family members, we detected
Fahr syndrome in his elder brother with hypocalcemia. Fahr disease may present with psychosis, have pronounced posi-
tive brain imaging findings. Magnetic Resonance Imaging can also be effective screening tool for adult relatives (Ger-
man J Psychiatry 2010; 13 (2): 86-90).
Keywords:
Fahr’s disease, familial, psychosis
Received: 13.4.2009
Revised version: 26.3.2010
Published: 21.6.2010
Introduction
ahr’s disease (FD) is a rare, degenerative, neurological
condition characterized by idiopathic calcification of
the
basal ganglia. This condition has been known
since the middle
1800s. The clinical manifestations of Fahr’s
disease vary. One definition proposed by Trautner et al.,
1988 requires
bilateral calcifications with neuropsychiatric
and extrapyramidal
disorders with normal calcium and phos-
phorus metabolism. Bealle et al., 1989 gave another defini-
tion which had seizures, rigidity, and dementia with characte-
ristic
calcification of the basal ganglia.
Flint and Goldstein, 1992 opined that radiologists may view
basal ganglia calcification (BGC) as an incidental finding so
clinical findings associated with Fahr’s disease are important.
According to Rasmussen et al. (1991)
before age 50 inciden-
tal discovery of BGC merits diagnostic investigation. The
course
of Fahr’s disease is progressive as reported by Ni-
shiyama et al., 1991. In adult-onset FD, calcium deposition
generally begins in the third decade of life, with neurological
deterioration two decades later as reported by Manyam et al.,
1992, but BGC can also occur in
pediatric populations.
The basal ganglia and dentate nucleus are the most common
site of involvement and most cases present with extra pyra-
midal symptoms. This disease is mostly associated with a
phosphocalcic metabolism disorder, especially to hypopara-
thyroidism.
Defective iron transport and free radical production
may
damage tissue, initiating calcification. (Beall et al, 1989)In
adult-onset FD, calcium deposition
generally begins in the
third decade of life, with neurological
deterioration two
decades later, (Manyam et al, 1992) Reduced blood
flow to
calcified regions correlates with clinical signs. (Uygur et al,
1995) Symptoms develop when the deposits accumulate,
including progressive deterioration of mental function, loss
of previous motor development, spastic paralysis, and athe-
tosis. In addition, optic atrophy may occur.
About 40% of patients
with basal ganglia calcification
present initially with psychiatric features (Konig, 1989).
Cognitive, psychotic, and mood disorders are common.
Symptomatic features may change over time. More extensive
calcification and subarachnoid space dilatation are known to
correlate with
the presence of psychiatric manifestations
(Konig et al, 1989).
F
FAHRS DISEASE
87
Case report
A 24 year old Hindu male, presented to outpatient clinic of
tertiary care centre (Guru Tegh Bahadur Hospital & Univer-
sity College of Sciences, Delhi) with recent onset complaints
of aggressive behavior and use of abusive language with
friends and family. Relatives also revealed behaviours as
smiling to himself, talking to himself, talking to ghosts and
being afraid of other people. Recently his sleep pattern had
also become quite irregular, sleeping for only three hours a
day. On further evaluation, the patient also revealed history
suggestive of obsessive behaviors (repeatedly cleaning uten-
sils) four years back and history of seizure disorder since last
ten years. Patient had generalized tonic clonic seizures with
loss of consciousness. Apparently his scholaristic perfor-
mance was good till sixth grade but sub-
sequently there was gradual deterioration
and he had failed his tenth class examina-
tions. His neurological examination was
normal. His mental status examination
revealed delusion of persecution, auditory
hallucinations as two or more voices
discussing among themselves and com-
manding the patient. These findings were
suggestive of psychosis, resembling schi-
zophrenia and patient had difficulty in
describing these experiences.
He had three more siblings, an elder
brother and two sisters. His elder brother
(29 years old) also had similar complaints
of recurrent seizures attacks since age of
seven years and also had poor academic
performance. He had a complaint of
knock knee since the age of twelve years.
His social behavior was otherwise normal
to others. His other two sisters had no
neuro-psychiatric complaints. There was
no history of similar complaints in any
other relatives.
MRI Brain of the patient revealed symme-
trical large areas and foci of calcification
in bilateral basal ganglia, thalami, cerebel-
lar parenchyma and subcortical regions of
bilateral cerebral hemispheres which were
suggestive of Fahr’s disease in the patient
(Figure 1) MRI findings of elder brother
also showed symmetrical calcifications in
bilateral gangliothalamic complexes, both
cerebral and cerebellar hemispheres in-
cluding dentate nuclei with confluent
white matter hyper intensity in both cen-
trum semiovale region, findings sugges-
tive of most probably Fahr’s disease in
elder brother (Figure 2).
Blood investigations were within normal
range for the patient though elder brother
had low serum calcium levels of 6mg/dl
(normal range of 8mg/dl-11mg/dl) and high serum phos-
phate levels of 7.7mg/dl (normal range of 3.0mg/dl-
5.0mg/dl) for elder brother.
The patient was initially prescribed paliperidone (6 mg/day),
haloperidol decanoate (50 mg) and sodium valproate
(1500mg/day) and clonazepam (1 mg) for control of agita-
tion. The psychotic symptoms responded poorly to the
initial treatment and as subsequently the diagnosis was con-
firmed amisulpride 200 mg/day was added. The patient
showed marked improvement over period of three to four
weeks, with psychotic symptoms decreasing and seizures
were controlled. Later family members could engage the
patient in farming activities. Six months after the initiation of
treatment, patient is functioning well as a farmer in village
with support of his brothers in the same profession.
Figure 1: MRI Image of the Patient
Figure 2: MRI Image of the Patient’s Elder Brothe
r
SRIVASTAVA ET AL.
88
Discussion
Fahr’s disease may present in familial form with history
suggestive of psychosis and generalised tonic clonic epilepsy
with intellectual decline. There was no evidence of hypocal-
caemia and hyperphosphatemia in the patient, though the
elder brother had such evidence and was suggested calcium
supplements by endocrinologist.
The patient lacked extrapyramidal symptoms or a metabolic
disorder and had normal neurological examination. Similar
cases have been reported by Kotan et al. (2009). With such a
clinical findings, the presentation of patient in early twenties
with recent onset of first episode psychosis with schizophre-
niform symptomatology can lead to misdiagnosis of schi-
zophrenia or acute transient psychotic disorder (ATPD),
especially in Asian country like India where ATPD is far
more commoner than schizophrenia.
The clinical expression of Fahr’s disease can vary greatly.
Symptom scan include features of psychiatric disorders,
epileptic seizures and dementia (Modrego et al 2005). But
other presentations have also been noted, like Simone et al.
(2008) reported case of Fahr’s disease with syncope and
pseudohypoparathyroidism.
About 40% of patients are known to present initially with
psychiatric features like this case, cognitive, psychotic, and
mood disorders are common Konig et al, 1989). Paranoid
and psychotic features often present between the ages of 20
and 40 in FD (Cummings, 1985). Two patterns of psychotic
presentation in FD are known, including early onset (mean
age 30.7 years) with minimal movement disorder and late
onset (mean age 49.4 years) attended by dementia and
movement disorder (Cummings et. al., 1983. This patient
had presented at 24 years with psychosis and no extra pyra-
midal involvement. Symptoms included auditory hallucina-
tions, perceptual distortions and paranoid delusions which
have been associated with FD (Rosenberg et al 1991). As in
our case schizophreniform psychoses have been reported to
present in familial FD (Francis et al, 1984).
On initial brief evaluation in walk in clinic, the patient was
initially prescribed paliperidone and haloperidol depot by
senior registrar, as there was possibility of poor compliance.
As mentioned earlier, the presentation of patient in early
twenties with recent onset of first episode psychosis with
schizophreniform symptomatology in India raises possibility
of diagnosis of ATPD which is far more commonly seen in
outpatient clinics than schizophrenia. But as psychosis in
setting of FD is known to respond variably to treatment and
is sometimes unresponsive (Cummings et. al., 1983). This
patient also responded poorly to initial treatment and as
diagnosis was confirmed, amisulpiride was added as these
patients are known to have high propensity of developing
extrapyramidal symptoms due to basal ganglia involvement
(Francis, 1979. Subsequently the psychotic symptoms re-
sponded well to treatment over three to four weeks with
improvement of occupational functioning. Six months later
patient is on maintainance treatment, has no psychotic fea-
tures or seizures and is functioning well in less challenging
occupation of farming in village with good social support.
The correction of phospho-calcium metabolism disorder led
to clinical improvement in brother, as also noted in other
cases (Abe et al., 1996). This case study emphasizes that
cases presenting with schizophreniform symptomatology,
even in countries with known high incidence of ATPD, must
be thoroughly investigated, family history be given due im-
portance and possibility of disorders presenting with protean
clinical manifestations as Fahr’s disease be considered.
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... It is an inherited or sporadic neurological disorder with a prevalence of <1/10000002. This syndrome is mostly associated with a disorder of calcium and phosphate metabolism, especially to hypoparathyroidism and pseudohypoparathyroidism [2,[7][8][9], but can also be attributed to other different etiologies, including infectious, metabolic, and genetic diseases [1]. PHP is a group of heterogeneous disorders with end-organ resistance of various hormones, especially parathyroid hormone (PTH) [10]. ...
... Although the most common cause of BGC are disorders of calcium and phosphate metabolism, more than fifty clinical conditions have been associated with BGC, including inflammatory, infectious, tumoral, endocrine, hypoxic, vascular, toxic, metabolic, and genetic disorders [1,18]. Among these conditions, the endocrine diseases, hypoparathyroidism, hyperparathyroidism and pseudohypoparathyroidism are the most common causes of Fahr's syndrome [2,[7][8][9]. ...
... Other neurological symptoms consist of stroke like events, speech disorders, coma, cognitive impairment, and syncope [18][19][20][21]. The psychiatric symptoms are usually more prominent than the neurologic symptoms in the onset of the disease [8,9]. Forty percent of patients with BGC present themselves with psychiatric symptoms at the beginning of the disease, and usually precede ...
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Full-text available
Physiological intracranial calcification occurs in about 0.3-1.5% of cases. It is asymptomatic and detected incidentally by neuroimaging. Pathological basal ganglia calcification is due to various causes, such as: metabolic disorders, infectious and genetic diseases. Hypoparathyroidism and pseudohypoparathyroidism are the one of the causes of pathological basal ganglia calcification. Besides tetany and seizures this condition is presented by parkinsonism and dementia. Infections (toxoplasmosis, rubella, cytomegalovirus, cysticercosis, AIDS) give multiple and asymmetric intracranial calcification. Inherited and neurodegenerative diseases cause symmetrical, bilateral basal ganglia calcification which is not related to metabolic disorders. Fahr's syndrome is a rare entity characterized by the presence of bilateral intracranial calcifications with predilection for the basal ganglia and dentate nuclei. It is commonly associated with endocrine disorders, particularly parathyroid and Vitamin D disturbances. Herein we report a case of pseudohypoparathyroidism revealed by Fahr's disease.
... It is an inherited or sporadic neurological disorder with a prevalence of <1/1 000 0002. This syndrome is mostly associated with a disorder of calcium and phosphate metabolism, especially to hypoparathyroidism and pseudo hypoparathyroidism (2,(7)(8)(9) , but can also be attributed to other different etiologies, including infectious, metabolic, and genetic diseases (1) .PHP is a group of heterogeneous disorders with end-organ resistance of various hormones, especially parathyroid hormone (PTH) (10) . ...
... Although the most common cause of BGC are disorders of calcium and phosphate metabolism, more than fifty clinical conditions have been associated with BGC, including inflammatory, infectious, tumoral, endocrine, hypoxic, vascular, toxic, metabolic, and genetic disorders (1,18) . Among these conditions, the endocrine diseases, hypoparathyroidism, hyperparathyroidism and pseudohypoparathyroidism are the most common causes of Fahr's syndrome (2,7,8,9) . ...
... Other neurological symptoms consist of stroke like events, speech disorders, coma, cognitive impairment, and syncope (18,19,20,21) . The psychiatric symptoms are usually more prominent than the neurologic symptoms in the onset of the disease (8,9) . Forty percent of patients with BGC present themselves with psychiatric symptoms at the beginning of the disease, and usually precede neurological manifestations (8,9) . ...
Article
Full-text available
Physiological intracranial calcification occurs in about 0.3-1.5% of cases. It is asymptomatic and detected incidentally by neuroimaging. Pathological basal ganglia calcification is due to various causes, such as: metabolic disorders, infectious and genetic diseases. Hypoparathyroidism and pseudohypoparathyroidism are the one of the causes of pathological basal ganglia calcification. Besides tetany and seizures this condition is presented by parkinsonism and dementia. Infections (toxoplasmosis, rubella, cytomegalovirus, cysticercosis, AIDS) give multiple and asymmetric intracranial calcification. Inherited and neurodegenerative diseases cause symmetrical, bilateral basal ganglia calcification which is not related to metabolic disorders. Fahr’ssyndrome is a rare entity characterized by the presence of bilateral intracranial calcifications with predilection for the basal ganglia and dentate nuclei. It is commonly associated with endocrine disorders, particularly parathyroid and Vitamin D disturbances. Herein we report a case of pseudohypoparathyroidism revealed by Fahr’s disease.
... A number of scientific literatures documented different forms of FD -autosomal dominant [18], sporadic [1] and autosomal recessive [19]. There are just a few reports of this condition from the Indian subcontinent [20][21][22]. We report two varied cases to highlight the heterogeneous nature of this rare disorder. ...
... In the earlier days there was possibility of diagnosis of a non affective psychosis [16]. The poor response to medication is seen in psychosis related to FD [21]. This patient also responded poorly to initial treatment and as diagnosis was confirmed, amisulpiride was added in the first case, the choice of medication was determined by the high propensity of developing extrapyramidal symptoms due to basal ganglia involvement and the likelihood of developing seizure disorder [1]. ...
... Calcifications are more commonly reported in the globus pallidus; additional reported sites [44]. Several cases were diagnosed incidentally [25,61,65,68,72] during routine assessment of psychiatric or somatic symptoms, which may suggest the possibility of underestimated diagnosis of BSPDC. ...
... Bilateral striopallidodentate calcinosis is mostly associated with a disorder of calcium and phosphate metabolism, especially hypoparathyroidism (HPT) [2,20,55,60]; however, different aetiology must be considered, including infectious, metabolic, and genetic diseases [65]. ...
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Introduction: Fahr’s disease is characterized by bilateral calcium deposition within the basal ganglia, cerebellar dentate nucleus and subcortical brain white matter. The main clinical manifestations are rigid or hyperkinetic syndrome, mood disorders and cognitive impairment. The correlation between neurological impairment and symmetrical basal ganglia calcification is not so frequent. Aim of the study was to report the results of neurological assessment of three sporadic cases of Fahr’s disease highlighting a correlation between the clinical syndrome and neuroimaging. Case reports: Three adults of aged 32, 55 and 70, were studied. They all showed a heterogeneous clinical spectrum. One case developed neuropsychiatric symptoms, whereas the others complained of the tremorigen syndrome. Brain computed tomography scans revealed several calcifications in basal ganglia, cerebellar white matter and dentate nuclei. Conclusions: The pathogenesis of Fahr’s disease is probably secondary to the dysfunction of cortico-basal connections and their interhemispheric relations. No significant correlation between calcifications and neurological symptoms is proved.
... Epilepsy is present in some cases, including different seizure types, as well as syncope, stroke, and strokelike episodes (Demir et al., 2020;Khalid et al., 2020;Rangaswamy et al., 2016). Up to one-third of patients may be asymptomatic, even at advanced age and with extensive calcified areas visible in the neuroimage, and this implies that the incidental diagnosis in patients undergoing brain computed tomography (CT) scan for other reasons is possible (Modrego et al., 2005;Srivastava et al., 2010). ...
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Objective: Calcifications in basal ganglia could be an incidental finding up to 20% of asymptomatic patients undergoing computed tomography (CT) or magnetic resonance imaging (MRI) scan. The presence of neuropsychiatric symptomatology associated with basal ganglia calcifications identifies a clinical entity defined as Fahr's Disease. This term is used in presence of calcifications secondary to a specific cause, but the variability of etiology, pathogenesis, and clinical picture underlying this condition have raised the question of the real existence of a syndrome. Several classifications based on the etiology, the location of brain calcifications and the clinical presentation have been proposed. Method: In the present study, we describe the case of a 52 years old man with a Bipolar I disorder diagnosis and a recent onset of behavioral disinhibition and alcohol misuse. The patient came to our center, specialized for bipolar disorder, as a consequence of a progressive worsening of the clinical picture associated to behavioral disturbances (sexual disinhibition, episodes of binge-eating, alcohol misuse), initial degrees of deterioration in cognitive function, peculiar psychotic symptoms and a resistance to various psychopharmacological treatment. The patient underwent neuro-psychologic evaluation, laboratory examinations and neuroimaging. Results and conclusions: CT and MRI revealed basal ganglia calcification and, in presence of normal blood tests, a diagnosis of Fahr's syndrome was suggested. During the hospitalization, the patient showed a good clinical response to a psychopharmacological therapy constituted by two mood stabilizers (lithium carbonate and oxcarbazepine) and mild antipsychotics doses (quetiapine and aripiprazole). Finally, we performed a literature review on the complex and multifaceted neuropsychiatric clinical manifestations of Fahr's disease in order to provide useful elements in terms of etiology, clinical manifestation, diagnosis, and treatment.
... Widespread neuropsychiatric and cognitive symptoms with extensive calcification on neuroimaging has been detected by several workers in the past. 12 A study by Shouyama et al also revealed that more extensive calcification, but not the distribution, correlates with the presence of higher frequency of psychiatric manifestations which could explain the presentation of the index patient with neuropsychiatric manifestations. 13 Current report points to the rare association of FD, FND and extensive calcification which might be the first case reported in the literature. ...
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Fahr’s disease is also known as familial idiopathic basal ganglia calcification affecting the cerebral microvessels and presenting with numerous neuropsychiatric manifestations. Functional neurological disorder (FND), a pure neuropsychiatric phenomenon occurs rarely in Fahr’s disease. Herein, we have presented a case of 17 year old female presenting neuropsychiatric symptoms like pseudoseizure, left sensorineural deficit and mild headache for last 4 months. Mental status examination revealed impaired attention and concentration. Laboratory examinations were within normal limits except CT scan and MRI Brain which revealed bilateral and symmetrical basal ganglia calcification. This is a rare case of FND associated with Fahr’s Disease, never reported in the literature. This case highlights the need for evaluating the biological factors in causation of FND where psychological factors were not assessed.
... It is believed to have autosomal dominant inheritance 3 but a few cases have been reported to have autosomal recessive inheritance and even some sporadic cases have been reported in literature 4 . Idiopathic calcification of the basal ganglia, also known as Fahr's disease, is a rare neurologic disorder of unknown etiology characterized by neuropsychiatric abnormalities, 5,6,7 Parkinsonian or choreoathetotic-type movement disturbance, and extensive symmetrical calcification of the basal ganglia and dentate nuclei in the cerebellum. These symptoms cannot be explained by any other particular disorder of the calcium phosphorus metabolism or any other disease. ...
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p>Idiopathic Basal Ganglia Calcification, also known as Fahr disease or Fahr's Syndrome or Bilateral StriatoPallidoDentate Calcinosis (BSPDC) is a rare, genetically dominant, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement, including the basal ganglia and the cerebral cortex. A rare idiopathic disease which manifests in middle age characterized by punctate areas of non-arteriosclerotic calcination in parts of the gray and dentate nuclei, particularly of smaller brain vessels. The symptoms include mental and growth retardation, dystonic movements, and athetosis. May be caused by a malfunction of the glandula parathyreoidea. The term Fahr triad consists of symmetrical calcification of the basal ganglia, neuropsychiatric symptoms, and hypofunction of the parathyroid gland. Treatment is directed toward minimizing symptoms. The prognosis for any individual with Fahr's Syndrome is variable and hard to predict. Progressive neurological deterioration generally results in disability and death. Medicine Today 2017 Vol.29(1): 39-41</p
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Calcification in the basal ganglia in the brain is, as a rule, asymptomatic and is therefore observed as an incidental finding at CT examination for other cerebral conditions e.g. tumours or haemorrhage. The question is whether incidental finding of calcification in the basal ganglia indicates the need for further supplementary investigations. Three case histories and study of the literature are quoted to illustrate this. It is concluded that investigation is not indicated in patients over the age of 50 years with limited, bilateral, symmetrical calcifications in the globi pallidi. In patients under 50 years, investigation in the form of detailed history taking, neurological examination and haematological investigation is indicated.
Article
Striopallidodentate calcinosis (Fahr's disease) is characterized clinically by seizures, rigidity, and dementia and pathologically by mineral deposition in the basal ganglia, dentate nucleus, and cerebral cortex. Disorders of iron and calcium-phosphate metabolism are thought to play a role in its pathogenesis. We present the case of a patient with familial striopallidodentate calcinosis who had porphyria cutanea tarda, refractory anemia, and pseudohypoparathyroidism type 2. The serum level of ferritin was markedly increased, serum iron and iron-binding capacity were below normal, and at autopsy she had deposition of iron in liver, spleen, bone marrow, and brain. She showed intermittent mild hypocalcemia, increased serum values of parathyroid hormone, elevated renal tubular reabsorption of phosphate, and low serum levels of 1,25-dihydroxyvitamin D, suggesting blunted renal responsiveness to endogenous parathyroid hormone. Pseudohypoparathyroidism type 2 was confirmed by infusion of synthetic parathyroid hormone, which gave a normal urinary cyclic adenosine monophosphate response, but a blunted phosphaturic response. After splenectomy for hypersplenism and weekly phlebotomies, she showed progressive improvement in function, mental status, weight, and seizure control. The hypothesis advanced is that the underlying pathophysiology of the separate diseases contributed to the formation of the brain stones through mechanisms of defective iron transport and free radical production.
Article
Psychopathological alterations caused by symmetrical basal ganglia sclerosis of different etiologies are described, involving cases with parathyroid gland/hormone dysfunction (some of them familial), patients after thyroidectomy, and patients with basal ganglia calcification of uncertain etiology. Initial symptomatology in a group of 62 patients is reported; chronic symptoms in another group of 35 patients were evaluated. Estimates of volume of the basal ganglia calcifications were made, in addition to precise topographical localizations by CT. In 40% the initial symptoms noted were psychiatric, compared with 50% who first presented neurological symptoms. In the group of chronic cases practically all showed intellectual impairment. There was a marked preponderance of organic affective syndromes (initially 21%, chronic 65%): the affective chronic patients can be subdivided into 37% depressive, 20% bipolar, 11% manic cases. We could find no direct relationships with regard to etiology, localization, volume or symptoms, except that extensive calcifications occur after parathyroid hormone deficiencies due to thyroidectomy and lead to more severe mental deterioration.
Article
Idiopathic basal ganglia calcification is a syndrome consisting of bilateral basal ganglia calcifications, neuropsychiatric abnormalities, disturbances of movement, and normal calcium and phosphorus metabolism. The best described neuropsychiatric alterations are dementia and an organic psychosis. Organic mood disorder has been reported less often, and mania secondary to idiopathic basal ganglia calcification has not been noted previously. The authors describe five patients with idiopathic basal ganglia calcification and organic mood changes, including one patient with secondary mania. Symptoms of idiopathic basal ganglia calcification resemble those of other disorders affecting subcortical structures and support an association between mood, affect, cognition, and the extrapyramidal nuclear system. Treatment may ameliorate the mood disorder.