Fahr syndrome

Sharma, Rohit; Nuñez, Anthony

doi:10.53347/rid-63485

Cited by 3 publications

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“…The most common site of abnormal calcium deposits is the globus pallidus. Additionally, deposits have been observed in the globus pallidus dentate nuclei, thalami, and other deep cortical structures ( 2 , 4 – 6 ). Fahr’s disease can manifest at any age, but the most frequent onset age is between the 30s and 50s ( 2 , 6 , 7 ).…”

Section: Discussionmentioning

confidence: 99%

“…Additionally, deposits have been observed in the globus pallidus dentate nuclei, thalami, and other deep cortical structures ( 2 , 4 – 6 ). Fahr’s disease can manifest at any age, but the most frequent onset age is between the 30s and 50s ( 2 , 6 , 7 ). The main clinical presentations include neurological symptoms such as seizures, Parkinsonism, rigidity, ataxia, dysarthria, tremor, and headaches, as well as psychiatric symptoms such as mood disorders, psychotic symptoms, irritability, aggressiveness, lack of impulse control, regressive behaviors, behavioral disinhibition, obsessive-compulsive spectrum symptoms, and cognitive and psychomotor impairment.…”

Section: Discussionmentioning

confidence: 99%

“…Currently, there is no consensus on treating Fahr’s disease and its associated symptoms. The prognosis of Fahr’s disease is variable and unpredictable ( 2 ). Most of the literature primarily focuses on managing and controlling the symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…The clinical presentation of this disease varies and may include psychosis, cognitive impairment, seizures, headaches, and motor function deterioration ( 1 ). The optimal treatment for Fahr’s disease remains unclear ( 2 ). While antipsychotics can offer symptomatic management of psychosis, patients with Fahr’s disease are more susceptible to side effects such as extrapyramidal syndromes ( 3 ).…”

Section: Introductionmentioning

confidence: 99%

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Idiopathic young-onset Fahr’s disease with schizophrenia-like presentation: a case report

Li,

Hsieh,

Chen

et al. 2024

Front. Psychiatry

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This case report describes an exceptionally rare case in which a prior diagnosis of schizophrenia was later determined to be early-onset Fahr’s disease, linked to a genetic mutation in the SLC20A2 gene. Initially, the patient exhibited symptoms resembling schizophrenia, including aggression and hostility, and was highly susceptible to medication side effects such as restlessness and Parkinsonism. Despite maintaining independent activities of daily living, his neurological examinations revealed hidden weakness on the left side. Following adjustments to the medication regimen, stability was achieved with residual psychotic symptoms under treatment with Risperidone 1.5mg/day, Valproic acid 1500mg/day, and Quetiapine 37.5mg/day. This case underscores the importance of conducting comprehensive imaging studies at the time of initial psychiatric diagnosis, regardless of the apparent typicality of the presentation. Additionally, it emphasizes the need for patience and adherence to the “Start Low and Go Slow” approach in medication management to minimize the risk of exacerbating psychiatric symptoms and aggression.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Idiopathic young-onset Fahr’s disease with schizophrenia-like presentation: a case report

Li,

Hsieh,

Chen

et al. 2024

Front. Psychiatry

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“…Clinical manifestations can range from asymptomatic cases to more severe courses, even among individuals within the same family [10]. Severe forms may lead to progressive deterioration, encompassing psychosis, depression, dementia, gait disturbance, speech difficulties, basal ganglia movement disorders, and cerebellar signs [5,10,11].…”

Section: Discussionmentioning

confidence: 99%

Basal Ganglia Calcification: A Case Report of Two Siblings With Fahr's Disease

Magalhães,

Alves,

Paulino Ferreira

et al. 2024

Cureus

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Fahr's disease is a rare neurodegenerative disorder caused by bilateral and usually symmetrical intracranial calcifications. In most cases, it exhibits an autosomal dominant pattern of inheritance and genetic heterogeneity. Patients may present with movement disorders, cognitive impairment, and psychiatric disorders. Currently, there are no disease-modifying drugs, so the management is based on the treatment of the symptoms. We present two cases involving male siblings, both with psychiatric symptoms as the initial presentation of the disease. Brain computed tomography revealed bilateral calcifications in the basal ganglia for which no underlying cause was found. In both cases, remission of behavioural changes and psychiatric symptoms was achieved with psychotropic drugs.

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Adie syndrome

Sharma¹,

Gaillard²

2009

Radiopaedia.org

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Fahr syndrome

Cited by 3 publications

References 0 publications

Idiopathic young-onset Fahr’s disease with schizophrenia-like presentation: a case report

Idiopathic young-onset Fahr’s disease with schizophrenia-like presentation: a case report

Basal Ganglia Calcification: A Case Report of Two Siblings With Fahr's Disease

Adie syndrome

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