FAHR’s ALERT. A Case Study Report
Speaker Credentials
MD
Format
Poster
Start Date
6-11-2020 11:00 AM
End Date
6-11-2020 11:15 AM
Abstract
FAHR’s ALERT A Case Study Report Author’s: David G. Cotto Velez, MD, Internal Medicine, Kendall Regional Medical Center; Jorge Hernandez Canciobello, MD, Internal Medicine, Kendall Regional Medical Center; Heidy L. Izquierdo, MD, Internal Medicine, Kendall Regional Medical Center; Jose D. Cordoba, MD, Internal Medicine, Kendall Regional Medical Center; Jose Gonzalez, MD; Jose G. Gascon, MD Introduction: FAHR’s Syndrome (FS) or Bilateral Striopallidodentate Calcinosis, is a rare disorder of unknown prevalence which is characterized by bilateral symmetric intracranial calcification (BLSICC) mostly in basal ganglia and globus pallidus. Cognitive impairment is a common presentation, followed by cerebellar impairment and speech disorder. We describe a middle age male arriving as stroke alert s/p generalized tonic-clonic seizure in the setting of BLSICC due to FS. Case presentation: A 58-year-old male with history of presumptive recurrent CVA with residual right sided hemiparesis and mild dysarthria, dementia, cognitive impairment, epilepsy on carbamazepine who presented as stroke alert, GCS 15, NIH 3 with worsening dysarthria, slow speech and right sided hemiparesis. Onset of symptoms associated with tonic-clonic seizure episode. Initial EKG NSR, brain CT scan showed a small hyperdense lesion in the occipital horn of the right lateral ventricle suggesting a small hemorrhage vs calcification; symmetrical calcification of the bilateral dentate nuclei, bilateral globus pallidus and bilateral medial cerebellum. Slurred speech improved. PTH noted slightly elevated, but calcium and phosphorus within normal values. Therapeutic Carbamazepine levels noted. Repeated imaging confirmed no bleeding. Patient was diagnosed with FS and reassured accordingly. Discussion: FS is a neurodegenerative clinical entity with Primary (sporadic or familial) or Secondary manifestations (endocrine disorders). The diagnosis of FS is essentially provided by the presence of BLSICC as seen in this patient with those neurological findings, who possibly was misdiagnosed in the past. The direction of this case is to always assess a correct diagnosis to educate properly and avoid overtreatment.
FAHR’s ALERT. A Case Study Report
FAHR’s ALERT A Case Study Report Author’s: David G. Cotto Velez, MD, Internal Medicine, Kendall Regional Medical Center; Jorge Hernandez Canciobello, MD, Internal Medicine, Kendall Regional Medical Center; Heidy L. Izquierdo, MD, Internal Medicine, Kendall Regional Medical Center; Jose D. Cordoba, MD, Internal Medicine, Kendall Regional Medical Center; Jose Gonzalez, MD; Jose G. Gascon, MD Introduction: FAHR’s Syndrome (FS) or Bilateral Striopallidodentate Calcinosis, is a rare disorder of unknown prevalence which is characterized by bilateral symmetric intracranial calcification (BLSICC) mostly in basal ganglia and globus pallidus. Cognitive impairment is a common presentation, followed by cerebellar impairment and speech disorder. We describe a middle age male arriving as stroke alert s/p generalized tonic-clonic seizure in the setting of BLSICC due to FS. Case presentation: A 58-year-old male with history of presumptive recurrent CVA with residual right sided hemiparesis and mild dysarthria, dementia, cognitive impairment, epilepsy on carbamazepine who presented as stroke alert, GCS 15, NIH 3 with worsening dysarthria, slow speech and right sided hemiparesis. Onset of symptoms associated with tonic-clonic seizure episode. Initial EKG NSR, brain CT scan showed a small hyperdense lesion in the occipital horn of the right lateral ventricle suggesting a small hemorrhage vs calcification; symmetrical calcification of the bilateral dentate nuclei, bilateral globus pallidus and bilateral medial cerebellum. Slurred speech improved. PTH noted slightly elevated, but calcium and phosphorus within normal values. Therapeutic Carbamazepine levels noted. Repeated imaging confirmed no bleeding. Patient was diagnosed with FS and reassured accordingly. Discussion: FS is a neurodegenerative clinical entity with Primary (sporadic or familial) or Secondary manifestations (endocrine disorders). The diagnosis of FS is essentially provided by the presence of BLSICC as seen in this patient with those neurological findings, who possibly was misdiagnosed in the past. The direction of this case is to always assess a correct diagnosis to educate properly and avoid overtreatment.