Young Male With Seizure Disorder and Intracranial Calcification: A Case of Fahr's Syndrome.

Jithesh G,Mohammed Ajmal,Monika Pathania,Rifika Bansal,Paras Gupta

doi:10.7759/cureus.22189

Abstract

Fahr’s syndrome is a neurodegenerative disorder characterized by abnormal deposition of calcium in the brain, especially in basal ganglia. The term Fahr’s disease is used when primary familial brain calcification is present, and the term Fahr’s syndrome is used for secondary causes. Our patient is a 35-year-old male who presented to our hospital with complaints of two episodes of generalized tonic-clonic seizures. He had a history of recurrent episodes of seizures since the age of 15 and they all were generalized tonic-clonic seizures. He did not have a family history of epilepsy. Lab investigations showed a normal hemogram, and liver and renal function were within normal limits. Serum electrolyte levels showed hypocalcemia, but other electrolyte levels were normal. He had low parathyroid hormone levels and normal levels of vitamin D. Brain imaging studies with non-contrast CT and a contrast-enhanced MRI showed bilaterally symmetrical dense calcifications. The etiology in our patient was the primary hypoparathyroidism and was treated accordingly. He reported symptomatic improvement with treatment and had no episodes of seizures after the commencement of the treatment. So, in cases of Fahr’s syndrome, treatable etiologies must be ruled out as they can delay the progression of the disease.

Highlights

Fahr’s syndrome is a neurodegenerative disorder that is characterized by abnormal deposition of calcium in the brain, especially in basal ganglia
We are reporting a case of Fahr’s syndrome secondary to hypoparathyroidism in a patient presenting with seizures
The possibility for intracerebral calcification secondary to TORCH (Toxoplasmosis, Other, Rubella, Cytomegalovirus (CMV), and Herpes) infections was ruled out with the age of presentation, uneventful antenatal history, and absence of a history of any developmental delay. He did not have any dermatological manifestations, and lactate levels were normal in his cerebrospinal fluid, ruling out the possibility of Mitochondrial Encephalomyopathy Lactic acidosis Stroke-like episodes (MELAS), and other secondary causes of Fahr’s syndrome

Summary

Introduction

Fahr’s syndrome is a neurodegenerative disorder that is characterized by abnormal deposition of calcium in the brain, especially in basal ganglia. The possibility for intracerebral calcification secondary to TORCH (Toxoplasmosis, Other (syphilis, varicella-zoster, parvovirus B19), Rubella, Cytomegalovirus (CMV), and Herpes) infections was ruled out with the age of presentation, uneventful antenatal history, and absence of a history of any developmental delay He did not have any dermatological manifestations, and lactate levels were normal in his cerebrospinal fluid, ruling out the possibility of Mitochondrial Encephalomyopathy Lactic acidosis Stroke-like episodes (MELAS), and other secondary causes of Fahr’s syndrome. He was administered a loading dose of levetiracetam (1 g) intravenously and maintained on oral levetiracetam (500 mg) twice daily. T1 hyperintensities bilateral caudate nuclei, thalami, subthalamic nuclei, and substantia nigra

Discussion

Conclusions

Disclosures