General anesthesia for patient with Fahr's syndrome

Abstract

Rationale:Fahr's syndrome (FS) is a rare neurological and psychiatric disorder characterized by bilateral brain calcifications when a secondary cause of the calcification is found.Patient concerns:A 53-year-old female patient diagnosed with FS for laminectomy because of ossification of posterior longitudinal ligament. She had a history of generalized tonic-clonic (GTC) seizure and medication with anticonvulsant drugs. She had gait disturbance, spasticity, bradykinesia, and diffuse calcifications in the basal ganglia, thalamus, cerebellum, and cerebral hemispheres on the brain computed tomography. On the preoperative examination, the serum and ionized calcium (Ca) were decreased to 3.7 and 2.22 mg/dL. Hypomagnesemia is combined with hypocalcemia. Furthermore, the level of parathyroid hormone was decreased to 2.18 pg/mL.Diagnoses:Due to the radiologic findings and laboratory test results, she was diagnosed with FS with primary hypoparathyroidism.Interventions:The Ca and magnesium (Mg) had been corrected before surgery but the electrolytes revealed low level at the intraoperative period. The 300 mg of calcium chloride was administered at 2 times and 1200 mg of it were infused for 1 hour during intraoperative periods. In addition, total 4 g of Mg sulfate intravenously administered. We used rocuronium as a neuromuscular block and checked neuromuscular function by train-of-four monitoring.Outcomes:Residual neuromuscular blockade was reversed with pyridostigmine and her muscle power completely recovered. The patient was extubated successfully and no unpredictable events occurred. On the day following transfer, serum electrolytes remained low, and although Ca was continuously supplied, serum Ca did not recover to a normal level. The patient was medicated with anticonvulsant drugs but experienced GTC seizure 2 weeks after surgery.Lessons:We presume that the pathophysiology of FS was related to primary hypoparathyroidism and hypomagnesemia. FS raises concerns associated with neuromuscular problems, spasticity, and seizure, and concerns of hypotension, heart failure, cardiac arrhythmia, and cerebrovascular attack during perioperative periods, among anesthesiologists because of hypocalcemia and vessel calcification. During the perioperative period, Ca levels should be closely monitored, and titrated Ca replacement therapy is recommended. The simultaneous correction of hypomagnesemia is of considerable importance when correcting hypocalcemia.

Adrenocorticotropic hormone combined with magnesium sulfate therapy for infantile epileptic spasms syndrome: a real-world study

“…Previous animal experiments have shown that low magnesium or magnesium-free perfusate can directly stimulate the hippocampal slices and olfactory bulb slices of rats, leading to spontaneous epileptic discharges [7][8][9]. Some studies have indicated that epileptic patients have significantly lower levels of magnesium in their plasma and serum compared to control groups [10,36]. Furthermore, MgSO 4 infusion has been successfully used to help one drug-resistant epilepsy patient with normal MRI performance and two patients with Alpers syndrome caused by mtRNA polymerase mutations to improve their status epilepticus [12,13].…”

Delayed awakening from general anesthesia revealing Fahr syndrome: Case report and literature review

“…Sometimes FS associates with cerebrovascular diseases and myopathies. 9,10 Before the advent of CT scan, the diagnosis of FS was based on X-rays of the skull and autopsy. Today, brain CT is the investigation of choice for the detection of intracerebral calcifications.…”

“…The clinical signs that can be encountered are convulsions, dementia, syncope, vertigo, dysarthria, headache, orthostatic hypotension, and neuropsychiatric disorders such as hallucinations, delusions, anxiety, mania, and depression. Sometimes FS associates with cerebrovascular diseases and myopathies 9,10 …”