Article

Clinical early symptoms and CT-Scan findings in Fahr syndrome

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Abstract

We present the results of an investigation on initial symptomatology of patients with bilateral, symmetrical intracerebral calcification of the basal ganglia (Fahr's syndrome). 62 patients, who because of various neurological or psychiatric symptoms or other reasons were referred to cranial computer-tomography, revealed clear manifestation of Fahr's syndrome. In these cases estimations of the volume of the opaque bodies were made, based on computer-integrated programmes (SO-MATOM D2). In 9 cases there was a history of thyroidectomy, on average 25 years previously. As a rule patients were referred in the age range between 40 to 60 years, those who had undergone thyroidectomy being on average somewhat older than the others. As presenting symptoms extrapyramidal syndromes, apoplexias, cephalea, affective organic alterations, alcoholism and dementia were noted. Cerebral localisation of bilateral, symmetrical calcification was most frequent in the pallidum, though this localisation may be responsible for numerous different neuropsychiatric symptoms. The dimensions of the opaque structures can be very greatly. Symptomatology seems to be practically independent of either cerebral localisation or volume, except that greater volumes of calcification seem to cause more pronounced neurological (extrapyramidal) symptomatology and dementia. The time-span between manifestation of the initial symptoms and diagnosis was shorter in neurological syndromes than in psychiatric cases. Like earlier workers, we found a high incidence (21%) of organic affective syndromes as an initial manifestation of Fahr's syndrome.

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... Although calcifications can involve other structures as well, the globus pallidus is most commonly involved. 181,182 The lateral pallidum tended to be more affected than the medial pallidum in one study. 183 Defective iron transport and free radical production may damage tissue, initiating calcification. ...
... The frequencies of calcifications have been determined in various endocrinologic disorders, 188,189 and the relationship of endocrine dysfunction to BGC has been assessed. [174][175][176]178,181,184,190 These associations are detailed elsewhere. 173 CT has greater diagnostic specificity for BGC, whereas MRI correlates better with functional impairment. ...
... Symptomatic features may change over time. 234,238 More extensive calcification and subarachnoid space dilatation correlate with the presence of psychiatric manifestations, 178,179,181 but calcific distribution and etiology do not. 179 Konig analyzed the frequency of psychiatric disorders in patients with BGC on both initial presentation and follow-up. ...
Article
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This report reviews clinical neuropsychiatric findings and opportunities for research in Huntington's, Wilson's, and Fahr's diseases. Consistent, systematic methodology is lacking among neuropsychiatric studies in these lenticulostriatal diseases. Systematic cross-sectional and longitudinal assessments are needed to ascertain the prevalence of psychiatric disorders as a function of disease course. Preliminary synthesis of existing data suggests the following heuristic relationships in these diseases: depression with parkinsonian states; personality changes with caudate or putamen disease; psychosis, impulsivity, and sexual disorders with caudate disease; dementia and mania with caudate and pallidal diseases; and compulsions with pallidal disease. Correlation of neuropsychiatric findings with disease stage, clinical signs, and radiologic, metabolic, physiologic, and pathologic markers of disease will add to our understanding of these conditions.
... These statistics suggest that the prevalence of neurological symptoms may vary in FS. It has been suggested that extent or side of the lesion have an effect on manifestation but when it comes to dementia and extrapyramidal symptoms, they become worse with more extensive calcification [53]. Clinical findings may correlate with site of calcification as Lopes-Villega reported that out of 18 Fahr's syndrome patients, 2(11.1%) had parkinsonism (with Pallidal thalamic and Cerebellal dentate calcification), 4 (22.2%) had transient ischemic attacks ( Pallidal and Putamen calcification), 1(5.6%) had dysarthria and orthostatic hypotension (caudate and putamen calcification). ...
... In another case, a 50 year old female patient with unimpaired basic and higher motor function had grossly compromised memory and attentional function but she remained neurologically asymptomatic. Neuropsychiatric illness may be present in the form of Schizophrenia like Psychosis, Dementia, Depression, Apoplexia, Deterioration of intelligence, inability to make decisions and effective organic alterations [28,53,[59][60][61][62]. In one case report it was reported that reduced glucose uptake in PET scan was not only confined to the Putamen and Globus Pallidus, but extended to involve the temporal and parietal cortices, bilaterally. ...
Article
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Fahr's disease or Fahr's syndrome is a rare, neurological disorder characterized by abnormal calcified deposits in basal ganglia and cerebral cortex. Calcified deposits are made up of calcium carbonate and calcium phosphate, and are commonly located in the Basal Ganglia, Thalamus, Hippocampus, Cerebral cortex, Cerebellar Subcortical white matter and Dentate Nucleus. Molecular genetics of this disease haven't been studied extensively; hence evidence at the molecular and genetic level is limited. Fahr's disease commonly affects young to middle aged adults. Etiology of this syndrome does not identify a specific agent but associations with a number of conditions have been noted; most common of which are endocrine disorders, mitochondrial myopathies, dermatological abnormalities and infectious diseases. Clinical manifestations of this disease incorporate a wide variety of symptoms, ranging from neurological symptoms of extrapyramidal system to neuropsychiatric abnormalities of memory and concentration to movement disorders including Parkinsonism, chorea and tremors amongst others. Diagnostic criteria for this disease has been formulated after modifications from previous evidence and can be stated briefly, it consist of bilateral calcification of basal ganglia, progressive neurologic dysfunction, absence of biochemical abnormalities, absence of an infectious, traumatic or toxic cause and a significant family history. Imaging modalities for the diagnosis include CT, MRI, and plain radiography of skull. Other investigations include blood and urine testing for hematologic and biochemical indices. Disease is as yet incurable but management and treatment strategies mainly focus on symptomatic relief and eradication of causative factors; however certain evidence is present to suggest that early diagnosis and treatment can reverse the calcification process leading to complete recovery of mental functions. Families with a known history of Fahr's disease should be counseled prior to conception so that the birth of affected babies can be prevented. This review was written with the aim to remark on the current substantial evidence surrounding this disease.
... It has various manifestations such as movement abnormalities (in the form of tremors, chorea, and Parkinsonism), dementia, cerebellar dysfunctions, and neuro-psychiatric presentations like personality changes, behavioral abnormalities, psychosis, depression, and worsening of intellectual functioning [1,2,[4][5][6][7][8]. ...
Article
Full-text available
Fahr’s syndrome is a rare neurological disorder characterized by bilateral basal ganglia calcification. Calcification may also involve other brain areas like dentate nuclei of the cerebellum, thalamus, cerebral cortex, hippocampus, and subcortical white matter. Many cases of Fahr’s syndrome present with movement disorders, but may also present with dementia, psychiatric manifestations, and language difficulties. Fahr’s syndrome generally occurs secondary to metabolic abnormality mainly hypoparathyroidism. Fahr’s disease is another variant that is characterized by idiopathic bilateral calcification of basal ganglia in absence of any evident etiology. The present case report presented a rare case of Fahr’s syndrome secondary to hypoparathyroidism presenting with pre-senile dementia with behavioral abnormalities.
... De modo semelhante ao observado na literatura encontramos, em nossos casos, média de idade elevada, sugerindo que com o avanço da idade ocorreria maior quantidade da calcificação 1,2,8,9 . Existem relatos de manifestações clínicas diversas em pacientes com CNB, tais como: distúrbio do movimento, da linguagem, demência, crises epilépticas sinais cere b e l a res, sinais piramidais, quadros psiquiátricos e distúrbios de equilíbrio 2 , 3 , 4 , 8 , 1 2 , 1 4 -1 7 , não havendo clara associação entre esses sintomas ou sinais e a CNB 2,[8][9][10][11][12]14 . ...
Article
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Analisamos os aspectos clínicos de 25 pacientes consecutivos que apresentaram calcificação nos núcleos da base na tomografia computadorizada (TC) de crânio. Esta ocorreu em 0,68% de todos os exames realizados no período. Vinte e três pacientes apresentavam condições clínicas diversas, a saber: cefaléia em 7 casos, acidente vascular cerebral em 5, síndrome extrapiramidal em 2, processo expansivo cerebral em 2, epilepsia, retardo do desenvolvimento neuropsicomotor, demência e trauma de crânio em um caso cada ou outras condições neurológicas em 3. Não havia sintomas neurológicos em 2 casos. Em 15 pacientes (60,0%) havia, além da calcificação dos núcleos da base, outras alterações na TC. Correlação clínica foi observada apenas com as outras alterações da TC e não com a calcificação dos núcleos da base, corroborando a hipótese de que esta possa ser um achado incidental.
... Forty percent of patients with basal ganglia calcifications present with psychiatric symptoms at the beginning of the disease. Among these; cognitive and psychotic disorders are most prominent (7,9,14). Our first case also manifested severe irritability lasting for a long time with the addition of amnesia at the latter terms of his disease. ...
Article
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Fahr's syndrome is a rare clinical entity that presents mainly with extrapyramidal signs and accompanied with metabolic, biochemical, neuroradiological and neuro-psychiatric situations at the same time. Bilateral intracranial calcifications are usually encountered as an incidental radiological finding. In this study, we discuss two cases of Fahr's syndrome in the light of the literature; one of them idiopathic and the other one occurring secondary to hypoparathyroidism. Clinically, one of the cases presented with seizures and the other one with imbalance of the coordination system. Cranial computerized scans of both patients revealed intracranial diffuse bilateral calcifications in thalamus, basal ganglia and cerebellum. Fahr's syndrome, although encountered rarely, should also be taken into account in the differential diagnosis of cases with abnormal intracranial calcifications along with other familial, congenital and metabolic diseases and syndromes.©2007, Fırat Üniversitesi, Tıp Fakültesi Key words: Fahr's syndrome, hypoparathyroidism, epilepsy, bilateral intracranial calcifications ÖZET Fahr's Sendromu: İki Olgunun Raporları Fahr sendromu esas olarak ekstrapiramidal bulguların ve aynı zaman dametabolik,biyokimyasal, nöroradyolijik ve nöropsikiyatrik durumların birlikte prezente oldugu nadir bir klınik antitedir. Genellikle bilateralin trakraniyal kalsifikasyonlar insidental radyolijik bulgudur. Bu calışmada, biz iki fahr sendromlu olguyu literatur ışıgında tartışdık.Olgulardan birisi idiyopatik ve digeri hipoparatiroidizme sekonder oluşan olgu idi. Klinik olarak olgulardan biri nöbet ile ve digeri denge bozuklugu ile prezente oldu. Heriki hastanin kranial bilgisayarlı tomografisinde talamus, bazal ganglionlar ve serebellumda intrakranyaldiffuz bilateral kalsifikasyonlar mevcut idi. Fahr sendromu nadir karsılaşılan bir antite olmasına ragmen, anormal intrakraniyal kalsifikasyonlu vakaların ayırıcı tanısında diger familyal, konjenital ve metabolik hastaliklar ve sendromlar ile birlikte göz önünde bulundurulmalıdır. ©2007, Fırat Üniversitesi, Tıp Fakültesi Anahtar kelimeler: Fahr's sendromu, hipoparatiroidizm, epilepsi, bilateral intrakranial kalsifikasyonlar.
... The criteria include bilateral calcification of the basal ganglia with neuropsychiatric and/or extrapyramidal features associated with normal calcium and phosphate metabolism [9] . These calcifications which are usually idiopathic occur most commonly at the basal ganglia, but other structures may also be affected [10] . Patients exhibit progressive neurological symptoms such as seizures, rigidity, and dementia with classical bilateral basal ganglia calcification shown on CT imaging. ...
Article
Full-text available
Fahr disease is a movement disorder characterized by calcium deposition in different parts of brain. It could be familial or secondary to a number of metabolic or infective causes. The age of presentation is bimodal and is according to the etiology. Here we present a case of 21 year old Pakistani female. She presented with sudden left sided weakness and dysphasia. On examination there was generalized rigidity of both upper and lower limbs. Power was unilaterally decreased on left side and her speech was dysrthric. After detailed history, examination and investigations she was diagnosed as a case of Fahr syndrome secondary to Systemic lupus erythematous Fahr syndrome has very heterogeneous clinical presentations and etiologies. Therefore, it's very imporatant to diagnose it early so that it can be properly treated.
... The criteria include bilateral calcification of the basal ganglia with neuropsychiatric and/or extrapyramidal features associated with normal calcium and phosphate metabolism [7]. These calcifications which are usually idiopathic occur most commonly at the basal ganglia, but other structures may also be affected [8]. Patients exhibit progressive neurological symptoms such as seizures, rigidity, and dementia with classical bilateral basal ganglia calcification shown on CT imaging. ...
Article
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Key Clinical Message While rare, Fahr's disease should be considered as a differential diagnosis for seizures, movement disorders, or cognitive impairment in tropical settings. Classically, bilateral calcification of the basal ganglia is seen on CT. Endemic infections, metabolic, and toxic causes should be excluded. Treatment using Levodopa is often beneficial.
... A sintomatologia é independente da localização cerebral ou do volume dos depósitos, mas maiores volumes de calcificação parecem estar associados a maior incidência de sintomatologia extrapiramidal. Há uma alta incidência de sintomas afectivos orgânicos como manifestação inicial de doença de Fahr -21% segundo um estudo com 62 doentes realizado na Alemanha (Taxer 1986 O quadro tinha tido início há cerca seis meses. A doente teria ficado progressivamente com mais energia, boa disposição e maior capacidade de que a habitual para realizar as tarefas domésticas. ...
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Resumo: A doença de Fahr é uma doença neuropsi-quiátrica rara caracterizada por calcificação dos gânglios basais, podendo-se manifestar por alterações do movimento, demência e doenças afectivas. O diagnóstico é feito através de métodos imagiológicos (TAC ou RMN CE). É apresentado um caso clínico de doença de Fahr, em que os primeiros sintomas de doença foram alterações do humor. Abstract: Fahr's disease is a rare neuropsychiatric disorder with calcification of the basal nucleus. Its symptoms include movement disorders, dementia and affective disorders. The diagnosis is made with brain image particulary CAT or MRI. The authors descri-be a clinical case of Fahr's disease, which presented initially with affective symptoms.
... De modo semelhante ao observado na literatura encontramos, em nossos casos, média de idade elevada, sugerindo que com o avanço da idade ocorreria maior quantidade da calcificação 1,2,8,9 . Existem relatos de manifestações clínicas diversas em pacientes com CNB, tais como: distúrbio do movimento, da linguagem, demência, crises epilépticas sinais cere b e l a res, sinais piramidais, quadros psiquiátricos e distúrbios de equilíbrio 2 , 3 , 4 , 8 , 1 2 , 1 4 -1 7 , não havendo clara associação entre esses sintomas ou sinais e a CNB 2,[8][9][10][11][12]14 . ...
Article
Twenty-five patients presenting basal ganglia calcification were assessed. This finding comprised 0.68% of all skull CT scan carried out during the period. Two patients were neurologically asymptomatic and 23 presented a variety neurological disorders--headache (7 patients), stroke (5 patients), extrapyramidal syndromes (2 patients), tumor (2 patients), epilepsy (1 patient), mental retardation (1 patient), dementia (1 patient), cranial trauma (1 patient), other neurological conditions (3 patients)--or were asymptomatic from the neurological point of view (2 patients). Findings in the CT scan other than the basal ganglia calcification were observed in 15 (60%) patients. There was a clinical-CT scan correlation in these cases but not in those in which the basal ganglia calcification was an isolated finding. This study highlights the fact that basal ganglia calcification is often a nonspecific finding on CT scan and that it may not be possible to establish a clinical-pathological correlation between them.
... Forty percent of patients with basal ganglia calcifications presents with psychiatric symptoms at the beginning of the disease. Among these; cognitive and psychotic disorders are most prominent [14][15][16]. Our first patient during the previous few years demonstrated remarkable impairments in memory functions and daily activities, including difficulties Eur Res J 2016 ...
Article
Full-text available
Fahr’s disease is a rare degenerative neurological disorder characterized by the presence of abnormal calcium deposition and associated cell loss in the areas of the brain that control movement, including the basal ganglia and cerebral cortex. Clinical findings associated with Fahr's disease include parkinsonism, dystonia, chorea, ataxia and psychiatric symptoms. Fahr's disease may result from metabolic disorders, especially parathyroid disorders. We report our experience with 4 patients admitted to our emergency department with complaints such as convulsions, hand spasms, loss of consciousness, and weakness. Computed tomography of all patients showed calcification in the bilateral basal ganglia. The purpose of this paper is to draw attention to a rare disorder involved in the etiology of elderly patients admitted to the emergency department for seizure and/or unconsciousness.
... Neurological symptoms include Parkinson's disease-like movement disorder, vertigo, epilepsy, syncope, cerebellar ataxia, and dementia [4][5]. Psychiatric symptoms include paranoid ideation, delusions, low mood, lack of interest, and auditory and visual hallucinations [3,[6][7]. ...
Article
Full-text available
Fahr's syndrome is a rare disease entity which presents with multiple neurological symptoms like movement disorder or cognitive impairment. We describe a case of a young male patient who presented with symptoms mimicking schizophrenia. He failed to improve despite medical management. He developed an episode of seizure which prompted us to make a computed tomography (CT) scan of the brain, revealing bilateral calcification of basal ganglia, despite normal serum calcium and parathyroid hormone (PTH) levels. This case experience explains the need to rule out all pathological causes of hallucinations before making a diagnosis of schizophrenia.
... The prevalence of the neurological symptomatology in Fahr's syndrome ranges from one third to one half of patients [3,4]. Generally, the location and extent of lesions have an effect on the manifestation; in particular in patients with dementia or patients with extrapyramidal symptoms, more extensive lesions cause more severe symptomatology [5]. On the genetic basis, FIBGC or Fahr's disease is most commonly transmitted as an autosomal dominant trait, however, also transmitted as an autosomal recessive trait, or occurs sporadically. ...
... Fahr's syndrome is characterized by basal ganglia calcification with clinical manifestations in the form of neuropsychiatric disorders, neurological symptoms, and [1,2] cognitive symptoms. Basal ganglia calcification [BGC] can be idiopathic or secondary to genetic, metabolic, and endocrinological disorders. ...
Article
Full-text available
Calcification of basal ganglia or Fahr's disease is a rare disease characterized by bilateral and symmetrical intracranial deposition of calcium mainly in cerebral basal ganglia. Basal ganglia calcification secondary to endocrinological cause is known as Fahr's syndrome. Motor and neuropsychiatric symptoms are prominent features. We report a case presented with hemiplegia without any psychiatric symptoms. Radiological findings were suggestive of bilateral calcification of cerebellar hemispheres and basal ganglia. Parathyroid hormone levels were low with no significant findings in other investigations along with negative family history. Based on the radiological and biochemical findings the case was diagnosed as fahr's syndrome.
... The criteria include bilateral calcification of the basal ganglia with neuropsychiatric and/or extrapyramidal features associated with normal calcium and phosphate metabolism [9] . These calcifications which are usually idiopathic occur most commonly at the basal ganglia, but other structures may also be affected [10] . Patients exhibit progressive neurological symptoms such as seizures, rigidity, and dementia with classical bilateral basal ganglia calcification shown on CT imaging. ...
Article
___________________________________________________________________________________________ Fahr's disease is a progressive and idiopathic basal ganglia calcification with normal metabolism of calcium and phosphor with motor and psychiatric signs and symptoms. Dementia, chorea athetosis, psychosis and depression due to Fahr's disease are frequently reported, but Fahr's disease with bipolar mood disorder manifestation is very rare and we found only 3 case in review of literature from 1955 to 2005.In this case report , a 21-year old girl is presented who was admitted to Sari-Zare psychiatric hospital for aggression, restlessness and insomnia. After mental status examination and Para clinical investigation, bipolar disorder due to Fahr's disease was detected. To date no specific treatment was found for this disease. This point is important that the patients with Fahr,s disease are sensitive to neuroleptic malignant syndrome.
Article
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Patients investigated at our institute during the last decade included 166 (1.2%) who showed uni- or bilateral basal ganglia calcification on computed tomography. We tested the significance of this neuroradiological observation by statistical comparison of these patients' clinical disorders with the findings in a random sample of 622 patients without basal ganglia calcification. The odds for the most common neurological disturbances were similar in patients with and without basal ganglia calcification. After adjustment for differences in age and brain atrophy there was no evidence of a significantly increased risk of dementia (odds ratio 1.1), cerebral infarction (1.4), epilepsy (0.9), vertigo (1.6), headache (1.8), or alcoholism (0.9), which represented the most common diagnoses. We conclude that basal ganglia calcification cannot be considered as a clinically relevant neuroradiological finding in the majority of cases and that it should not be used as an explanation for frequently observed neurological disturbances.
Article
Twenty-two patients aged 36-63 years were diagnosed as having Fahr's syndrome on the basis of the presence on CT of unexpected extensive calcification of the basal ganglia. Even when associated with calcification of other brain areas, the main diagnostic criterion remained basal ganglia calcification larger than 800 mm2. Normal values of parathormone, serum calcium and phosphorus excluded hypercalcaemia and hypoparathyroidism. Mitochondrial CNS disease was excluded clinically. MRI and repeated CT and neurological examination were performed in all of the patients. The patients were divided into two groups: neurologically asymptomatic (group 1) and neurologically symptomatic (group 2). T2-weighted sequences demonstrated hyperintense areas in all of the patients involving the white and the grey matter of the brain. In group 1 the hyperintense lesions were significantly smaller than in group 2. The neurological symptoms correlated better with the hyperintensities on T2-weighted MR images than with the calcification demonstrated on CT. Hyperintensities in T2-weighted MRI and the areas shown by CT to have calcification had different locations. In 15 patients with dementia, the white matter of the entire centrum semiovale was bilaterally hyperintense. In another 3 patients with hemiparesis, hyperintense areas in the internal capsule, contralateral to the side of hemiparesis, were demonstrated in the T2-weighted sequence. The hyperintense T2 signals may reflect a slowly progressive, metabolic or inflammatory process in the brain which subsequently calcifies and are probably responsible for the neurological deficit observed.
Article
Fahr's disease is characterized by basal ganglia calcification with clinical manifestations in the form of neuropsychiatric disorders, neurological symptoms, and cognitive symptoms. In this case series, we describe two cases of basal ganglia calcification, one of whom presented with psychotic symptoms and the other with mood symptoms, and discuss the literature with regard to psychiatric manifestations of basal ganglia calcification.
Article
H´á um tipo especifico de calcificação intracraniana que compromete simetricamente os gânglios da base e os núcleos denteados do cerebelo e é denominado calcificação simétrica dos gânglios da base (CSGB) ou doença de Fahr, no qual o principal método diagnóstico ES a tomografia computadorizada. Essa revisão mostra que pode estar associado a vários processos patológicos, como distúrbios do cálcio e fósforo e as manifestações clinicas não estabelecem estrita relação com a topografia das lesões, havendo destaque para as crises epilépticas e distúrbios psiquidtricos. Conclui-se, portanto, que a CSGB nem sempre é idiopática e o termo calcificação não é apropriado, também não constitui uma doença definida.
Article
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Bilateral striopallidodentate calcinosis, commonly known as Fahr's disease is a rare clinical entity present mainly with extrapyramidal signs and accompanied with metabolic, biochemical, neuroradiological and neuropsychiatric situations. It is characterised by the symmetrical and bilateral intracranial deposition of calcium associated with cell loss in the basal ganglia, cerebral cortex and cerebellum.In this study, we discussed two brothers' cases of Fahr's diseases who presented with different symptomatology. The first presented with walking difficulty, cramps and dysarthria and moderate memory impairment whereas the second with vertigo, ataxia, forgetfulness and headache. CT scans of both patients revealed intracranial diffuse bilateral calcifications in the basal ganglia and the cerebellum. The second patient revealed progressive cerebral atrophy but reduction in the calcification.Fahr's disease, although encountered rarely, should also be taken into account in the differential diagnosis of cases with abnormal intracranial calcifications along with other familial, congenital and metabolic diseases and syndromes.
Chapter
Basal ganglia calcifications are a frequent neuroimaging finding upon cerebral computerized tomography (CT) and may incidentally be identified in about 1 % of otherwise normal elderly subjects. The clinical picture of symptomatic basal ganglia calcifications includes neuropsychiatric abnormalities and movement disorders. Idiopathic cases, often referred to as Fahr’s disease, idiopathic basal ganglia calcification (IBGC) or bilateral striato-pallido-dentate calcinosis (BSPDC), are either familial, mostly with an autosomal dominant mode of inheritance, or, less commonly, sporadic. Secondary causes such as disorders of calcium metabolism, vascular malformations, phacomatoses, tumors, or parasitical infections of the central nervous system may also lead to intracranial mineralization deposits. Apart from the basal ganglia (striatum and pallidum), a variety of anatomical structures can be affected by calcification, including the dentate nucleus, thalamus, and subcortical white matter. Calcifications typically show a symmetric distribution and differ in intensity and localization among affected subjects. Cerebral CT is the diagnostic gold standard to verify intracerebral calcifications. In contrast, magnetic resonance imaging (MRI) appears to have a rather low sensitivity and specificity and often MRI results remain inconclusive. Recently, transcranial sonography was consistently shown to reveal symmetric hyperechogenic areas of the basal ganglia corresponding to mineralization in patients with BSPDC. However, systemic investigations on the diagnostic value are lacking. Functional radioligand imaging methods provide an opportunity to demonstrate changes in the nigrostriatal function, local brain perfusion, and glucose metabolism. This chapter aims to clarify the heterogeneous terminology of basal ganglia calcifications and addresses the etiology and diagnostic approaches. Assets and drawbacks of different diagnostic tools are discussed.
Chapter
Zahlreiche Arzneimittel können zu psychischen Störungen mit und ohne gleichzeitige neurologische Symptome führen. Abgesehen von Medikamenten, bei denen eine enzephalo- und/oder psychotrope Wirkung therapeutisch erwünscht ist (Psychopharmaka, Antikonvulsiva, Antiparkinsonmittel), können nach zahlreichen anderen Substanzen unerwünschte zentralnervöse Effekte mit psychopatho-logischen Syndromen auftreten. Risikofaktoren und Inzidenzraten für die das Zentralnervensystem betreffenden unerwünschten psychopathologischen Effekte sind nur bei wenigen Substanzen zureichend bekannt.
Chapter
In diesem Kapitel werden Erkrankungen, die häufig zu einer organisch bedingten psychischen Störung führen können, ausführlich dargestellt. Dabei wird auch darauf eingegangen, ob und inwieweit psychische Symptome Frühsymptome oder gar die Erstmanifestation dieser Erkrankungen sein können. Da bei einer Reihe der in Frage kommenden Störungen verschiedene OPS auftreten können und daher die Differenzialdiagnose erschwert ist, sind in einer tabellarischen Übersicht (Tabelle 5.1) die am häufigsten vorkommenden zusammengestellt.
Article
Fahr's disease is a rare neurodegenerative syndrome, characterized by massive symmetrical intracerebral calcifications of the basal ganglia, dentate nuclei of the cerebellum, and the adjacent parenchyma. Computerized tomography (CT) is considerably more sensitive to detect these intracranial calcifications than other imaging modalities. The clinical, CT scan, and 99(m)Tc-D,L-hexamethylpropylene amine oxime (99(m)Tc-HMPAO) brain perfusion single-photon emission computerized tomography (SPECT) findings in a 42-year-old woman with Fahr's disease are reported, and the clinical utility of 99(m)Tc-HMPAO SPECT findings in Fahr's disease is discussed in this article. In conclusion, 99(m)Tc-HMPAO brain perfusion SPECT seems to be useful in the clinical approach to Fahr's disease, and may provide more specific and clinically relevant information when compared with anatomical imaging.
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