Idiopathic Basal Ganglia Calcification: Fahr’s Syndrome, a Rare Disorder

Thillaigovindan, Ranjani; Arumugam, Eswaran; Rai, Rathika; Prabhu, R; Kesavan, R

doi:10.7759/cureus.5895

Cited by 3 publications

(3 citation statements)

References 9 publications

(9 reference statements)

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“…Fahr’s disease progresses with worsening neurological dysfunction, bilateral basal ganglia calcification, and systemic or mitochondrial conditions (including endocrinopathies) [ 14 ]. The affected patients usually present with a family history of autosomal dominant inheritance.…”

Section: Discussionmentioning

confidence: 99%

The Impact of COVID-19 Infection on a Neurologically Compromised Male With Fahr’s Disease Presenting With Acute Delirium and Aspiration Pneumonia: A Case Report

Bhangal¹,

Sandhu²,

Umar³

et al. 2022

Cureus

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Fahr’s disease or idiopathic basal ganglia calcification is a rare, sporadic, genetically dominant, and inherited neurological condition that manifests with dysphagia and Parkinson’s disease. The computed tomography (CT) scan is the method of choice to diagnose basal ganglia calcifications seen in Fahr’s disease. This case report elaborates on the emergency management of a 58-year-old male patient with acute respiratory distress, acute delirium, schizophrenia, Fahr’s syndrome, and history of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) (coronavirus disease 2019 or COVID-19) infection. The patient’s chest X-ray, laboratory workup, and vital signs were suggestive of aspiration pneumonia-induced sepsis and acute hypoxemic respiratory failure. Post-admission antibiotic management reduced sepsis complications without improving the altered mental status. A comprehensive clinical assessment suggested the attribution of Fahr’s disease to the patient’s aspiration pneumonia and other clinical complications. In addition, COVID-19 infection, sepsis-induced inflammatory processes, and pre-existing neurological compromise possibly deteriorated the patient’s neurological outcomes, overall prognosis, and recovery.

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Section: Discussionmentioning

confidence: 99%

The Impact of COVID-19 Infection on a Neurologically Compromised Male With Fahr’s Disease Presenting With Acute Delirium and Aspiration Pneumonia: A Case Report

Bhangal¹,

Sandhu²,

Umar³

et al. 2022

Cureus

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“…The diagnostic criteria for Fahr's syndrome include bilateral striatopallidodentate calcification on neuroimaging, progressive cognitive dysfunction, and movement disorders without biochemical, infectious, toxic, and traumatic causes [4]. A computed tomography scan remains the best imaging modality for Fahr's disease [5].…”

Section: Introductionmentioning

confidence: 99%

Fahr’s Syndrome: A Rare Case Presentation

Wazir,

Ali,

Mufti

et al. 2023

Cureus

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Idiopathic basal ganglia calcification (IBGC), also known as Fahr's disease, is a rare neurological disorder characterized by metabolic, biochemical, neuroradiological, and neuropsychiatric alterations resulting from symmetrical and bilateral intracranial calcifications. In most cases, an autosomal dominant pattern of inheritance and genetic heterogeneity is observed. Neuropsychiatric symptoms with movement disorders account for 55% of the manifestations of this disease.In this report, we present the case of a 42-year-old Pakistani male who presented to the emergency department with a sudden onset of generalized tonic muscle contractions. His medical history revealed progressive cognitive impairment, and he had a history of taking oral calcium supplements. Initial laboratory investigations showed hypocalcemia with normal magnesium and phosphate levels, while his parathyroid hormone levels were low. The interictal electroencephalogram was normal, and CT imaging of the brain without contrast revealed bilateral symmetrical dense calcifications in the basal ganglia, thalami, periventricular area, corona radiata, centrum semiovale, and dentate nucleus of the cerebellum, suggestive of Fahr disease. Intravenous calcium gluconate was administered in the emergency department, leading to an improvement in the patient's symptoms. The diagnosis of IBGC with relevant symptoms was confirmed through laboratory values and characteristic features observed in the CT examination.

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“…2 One such pathological condition is Fahr's disease, a condition characterized by bilateral intracranial calcifications, particularly in parts of the brain that control movement (including the basal ganglia). 3 It is a rare condition that generally presents with extrapyramidal symptoms, cerebellar symptoms, problems with speech, dementia, etc. 4 Here, we describe a case of Fahr's syndrome secondary to hypoparathyroidism, mimicking meningoencephalitis with neuropsychiatric manifestations.…”

Section: Introductionmentioning

confidence: 99%

Secondary Fahr’s syndrome mimicking meningoencephalitis

Vamshikrishnapatel,

Kumar,

Biswas

et al. 2023

Eur J Clin Exp Med

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Introduction and aim. Fahr’s disease is a rare neurological disorder characterized by bilateral basal ganglia calcifications. The aim of this report is to highlight a case of Fahr’s disease with atypical features such as neck rigidity and peripheral nerve involvement. Description of the case. Here, we present the case of a South Asian patient with secondary Fahr’s disease whose clinical presentation mimicked that of meningoencephalitis. The patient had neurological and neuropsychiatric symptoms along with abnormal body movements. She also had restricted neck mobility. Computed tomography of the head revealed bilateral dense calcifications in the basal ganglia suggestive of Fahr’s syndrome. Investigations revealed severe hypocalcemia, hyperphosphatemia, and low parathyroid hormone levels, which led to the identification of hypoparathyroidism as the underlying cause. The presence of pre-existing epilepsy and neck rigidity made the diagnosis difficult. The relatively rapid development of symptoms along with the presence of peripheral nerve involvement made this case even more unique. Calcium levels were corrected, and there was a marked symptomatic improvement. Conclusion. Neck rigidity and restricted neck mobility may be present in cases of Fahr’s syndrome due to calcifications of the nuchal ligament or other spinal ligaments and thus must be differentiated from meningoencephalitis. Although the symptoms of Fahr’s syndrome are generally limited to the central nervous system, there may be involvement of the peripheral nerves as well.

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Idiopathic Basal Ganglia Calcification: Fahr’s Syndrome, a Rare Disorder

Cited by 3 publications

References 9 publications

The Impact of COVID-19 Infection on a Neurologically Compromised Male With Fahr’s Disease Presenting With Acute Delirium and Aspiration Pneumonia: A Case Report

The Impact of COVID-19 Infection on a Neurologically Compromised Male With Fahr’s Disease Presenting With Acute Delirium and Aspiration Pneumonia: A Case Report

Fahr’s Syndrome: A Rare Case Presentation

Secondary Fahr’s syndrome mimicking meningoencephalitis

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