• Fahr’s syndrome
• Ralstonia
• calcium
• hypoparathyroidism
• seizures

• Adult
• Middle Aged
• Humans
• Basal Ganglia Diseases/diagnosis
• Basal Ganglia Diseases/diagnostic imaging
• Calcinosis/etiology
• Calcinosis/complications
• Neurodegenerative Diseases/complications
• Neurodegenerative Diseases/diagnosis
• Hypoparathyroidism/complications
• Hypoparathyroidism/diagnosis

• Fahr’s syndrome
• basal ganglia
• hypocalcemia
• hypoparathyroidism
• seizures

Fahr’s syndrome is a rare neurodegenerative disorder characterized by bilateral calcifications of the basal ganglia and cerebral cortex. These deposits are made of calcium and phosphorus and are thought to be due to abnormalities in calcium-phosphate homeostasis. The clinical manifestation includes extrapyramidal symptoms (e.g., spastic paralysis), generalized or partial seizures, cognitive impairment, and neuropsychiatric symptoms. Here, we discuss a case of a young female with a medical history of systemic lupus erythematosus (SLE) and lupus nephritis who developed generalized seizures and was found to have extensive bilateral calcifications in the basal ganglia and cerebral cortex. Her laboratory workup showed disorganized calcium-phosphorus homeostasis.

• Fahr´s syndrome
• cognitive impairment
• hypoparathyroidism
• seizure

Patients with chronic idiopathic hypoparathyroidism may develop neurological complications, including calcification of the basal ganglia and other areas of the brain. In Fahr's syndrome, intracranial calcification is associated with an underlying disorder such as hypo or hyperparathyroidism. We report the case of a 37-year-old gentleman, with a history of bilateral cataract surgery and seizures, who presented with a new episode of seizure and was found to have severe hypocalcemia and bilateral symmetric intracranial calcification due to previously diagnosed primary hypoparathyroidism. He had symptoms and signs mimicking ankylosing spondylitis (AS), but with negative radiological and serological findings, not fitting into the diagnosis of axial spondyloarthropathies (SpA), as per standard criteria. Patients with long-standing idiopathic hypoparathyroidism can have severe calcification of soft tissues and bones, including vertebrae and paravertebral soft tissues, causing inflammatory back pain and stiffness. It is vital to report such cases as their occurrence is rare, and physicians should be aware of the possibility while evaluating patients with inflammatory back pain. Treatment in these cases is directed towards hypocalcemia and underlying primary pathology rather than spondyloarthropathy.

• fahr’s syndrome
• hypocalcemia
• hypoparathyroidism
• spondyloarthropathies

The basal ganglia and thalami are paired deep grey matter structures with extensive metabolic activity that renders them susceptible to injury by various diseases. Most pathological processes lead to bilateral lesions, which may be symmetric or asymmetric, frequently showing characteristic patterns on imaging studies. In this comprehensive pictorial review, the most common and/or typical genetic, acquired metabolic/toxic, infectious, inflammatory, vascular and neoplastic pathologies affecting the central grey matter are subdivided according to the preferential location of the lesions: in the basal ganglia, in the thalami or both. The characteristic imaging findings are described with emphasis on the differential diagnosis and clinical context.

• Basal ganglia
• Bilateral lesions
• CT
• MRI
• Symmetric lesions
• Thalamus

• Amnesia
• Basal ganglia
• Hypoparathyroidism

Hypoparathyroidism with basal ganglia calcification is clinically rare. Here, we report a case of Fahr’s syndrome due to hypoparathyroidism and review the literature in terms of etiology, clinical manifestation, diagnosis, and treatment.

A 62-year-old man experienced repeated twitching of both hands in recent 10 years. On July 28, 2017, the patient was admitted to our hospital due to slow response and speech difficulties. On medical examinations, he had a positive Chvostek sign, while no Albright’s hereditary osteodystrophy signs or history of neck surgery or radiation, and his family members had no similar medical history. Laboratory examinations revealed hypocalcemia, hyperphosphatemia, and low parathyroid hormone (PTH) levels. Computed tomography revealed basal ganglia calcification. Based on these investigations, a diagnosis of Fahr’s syndrome due to hypoparathyroidism was suggested. After receiving intravenous calcium gluconate to relieve symptoms, the patient continued to take oral calcium carbonate and calcitriol for treatment.

The possibility of hypoparathyroidism should be considered in patients with chronic hypocalcemia, recurrent tetany, and even neuropsychiatric symptoms. Hypoparathyroidism is a common cause of basal ganglia calcification. Therefore, it is recommended that blood calcium, phosphorus, and PTH levels should be measured in all individuals with basal ganglia calcification to exclude hypoparathyroidism.

• Hypoparathyroidism
• Hypocalcemia
• Fahr’s syndrome
• Case report