Fahr’s Disease: A Differential to Be Considered for Various Neuropsychiatric Presentations

Pourshahid, Seyedmohammad; Salloum, Mohammad; Elfishawi, Mohanad; Barakat, Mohamed; Basith, M. A.

doi:10.7759/cureus.2304

Cited by 8 publications

(15 citation statements)

References 5 publications

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“…[ 7 ] About 40% of patients with BG calcification show neuropsychiatric manifestations. [ 7 ] Cortico-subcortical disconnection due to BG calcification involves frontostriatal and limbic pathways[ 7 17 18 ] resulting in psychosis, mood, personality, and cognition disorders. In our study, we found that depression (23.1%) was the most common neuropsychiatric manifestation in FD followed by anxiety disorder (15.4%) and psychosis (7.7%).…”

Section: Discussionmentioning

confidence: 99%

Clinical and outcome analysis in head injury patients with fahr's disease

et al. 2021

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Context: Fahr's disease (FD) is a rare neurodegenerative disorder. Head injury in patients with FD is an uncommon occurrence. Aim: The aim is to evaluate clinical and outcome characteristics in traumatic head injury patients with FD. Settings and Design: Retrospective cohort study. Materials and Methods: This retrospective cohort study includes 13 patients of FD presenting as head injury in neurosurgical emergency between September 2018 and February 2021. Each patient was evaluated in terms of demographic profile, Glasgow coma scale (GCS) at admission, severity of head injury, type of head injury, preexisting clinical features of FD, radiological findings, Glasgow outcome score (GOS), family history of FD, and biochemical abnormalities. Patients were also evaluated for dichotomized outcome (Good recovery: GOS 5–4 versus Poor recovery: GOS 1–3) and gender differences in FD presentation. Statistical Analysis Used: Fisher's exact test and unpaired t -test were used. P < 0.05 was considered statistically significant. Results: Neurological symptoms (69.2%), neuropsychiatric manifestations (46.1%) and extrapyramidal features (38.5%) were preexisting in these patients. Seizure (61.5%) was the most common neurological manifestation. Depression (23.1%) and anxiety disorder (15.4%) were common psychiatric disorders seen. Akathisia (23.1%) followed by tremor (15.4%) were predominant extrapyramidal presentations. On dichotomized outcome analysis, preexisting neurological, neuropsychiatric, and extrapyramidal manifestations due to FD were not associated significantly with outcome following head injury. GCS at admission, severity of head injury and pupillary changes were significantly associated with outcome ( P < 0.05). Neuropsychiatric features ( P = 0.0210) were significantly more in females suffering from FD. Conclusions: Neurological features in FD predominate over neuropsychiatric and extrapyramidal symptoms. FD does not affect outcome following head injury.

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Section: Discussionmentioning

confidence: 99%

Clinical and outcome analysis in head injury patients with fahr's disease

et al. 2021

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“…There are some suggestions that intracerebral calcium deposition is secondary to local disturbance of the bloodbrain barrier or neuronal calcium metabolism disorder. 29 An important argument in favor of vascular theory was given by studies using cerebral flow scintigraphy that provided evidence of altered blood flow to the calcification sites. 30 Furthermore, inflammatory conditions, such as meningitis or encephalitis, are considered in pathogenesis of Fahr's syndrome.…”

Section: Discussionmentioning

confidence: 99%

Fahr’s disease: familial idiopathic basal ganglia calcification with and without extrapyramidal disorders

Castagna¹,

Ruberto²,

Cerra³

et al. 2020

Geriatr Care

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Fahr’s disease (FD), also known as familial idiopathic basal ganglia calcification, is a neurodegenerative disease affecting cerebral micro vessels, mainly in the basal ganglia. It mostly presents with movement disorders, dementia and behavioral abnormalities. It is considered hereditary with an autosomal dominant transmission. Fahr’s disease is often underestimated and under diagnosed. We reported the clinical differences found in two patients with Fahr’s Disease. In particular, we described a case of Fahr’s disease with behavioral alteration with extrapyramidal movement disorders, and a rare case of Fahr’s disease with cognitive and behavioral alterations in absence of extrapyramidal movement disorders.

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“…Baseline biochemical investigations are indicated to rule out other possible diagnoses (Table 4) [7-8].…”

Section: Discussionmentioning

confidence: 99%

Bilateral Basal Ganglia Calcification: Fahr's Disease

Ooi

Hussain

et al. 2019

Cureus

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Fahr’s disease/syndrome is a condition defined as bilateral striato-pallido-dentate calcinosis, a neurodegenerative disease with radiological findings of symmetrical and bilateral idiopathic calcifications of the cerebellum, periventricular white matter, and basal ganglia. Clinical correlation with radiological and a calcium metabolism panel is crucial in differentiating between Fahr’s disease and Fahr’s syndrome. We describe a case that presented with the clinical feature of a cerebrovascular accident and had an incidental radiological finding of Fahr syndrome. The clinical features, laboratory investigations, and clinical management of Fahr's disease/syndrome will be discussed in detail in the article.

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Fahr’s Disease: A Differential to Be Considered for Various Neuropsychiatric Presentations

Cited by 8 publications

References 5 publications

Clinical and outcome analysis in head injury patients with fahr's disease

Clinical and outcome analysis in head injury patients with fahr's disease

Fahr’s disease: familial idiopathic basal ganglia calcification with and without extrapyramidal disorders

Bilateral Basal Ganglia Calcification: Fahr's Disease

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