Pearls & Oy-sters: clues to the diagnosis of adult-onset acid maltase deficiency.

Pearls and oy-sters: ictal syncope in a patient with temporal lobe epilepsy.

PEARLS & OY-STERS: ICTAL SYNCOPE IN A PATIENT WITH TEMPORAL LOBE EPILEPSY Nitin K. Sethi, New York: Varade et al. reported ictal syncope in a patient with temporal lobe epilepsy (TLE). The MRI revealed a right temporal-occipital cavernous angioma and the seizure showed onset in the right posterior temporal electrode (T6) on EEG, all compatible with a diagnosis of symptomatic localization-relate...

Pearls & Oy-sters: The importance of atypical features and tracking progression in patients misdiagnosed with ALS.

• Atypical features that should alert neurologists to the possibility of alternative diagnoses include symmetric findings, disease duration greater than 2 years at presentation, young age at onset (less than 50), and pain. • While timely diagnosis of amyotrophic lateral sclerosis (ALS) is important, it is equally important that clinicians avoid premature closure by ruling out disease mimics, an...

Adult-onset acid maltase deficiency. Case report of an adult with severe respiratory difficulty.

Pompe's disease (acid maltase deficiency) classically affects infants and children, with a few sporadic cases occurring in adults. An adult patient initially have progressive muscular weakness, exertional dyspnea, diaphragmatic paralysis, and objective evidence of restrictive respiratory disease. Muscle biopsy established the diagnosis of acid maltase deficiency. The patient's brother had died ...

Pearls & Oy-sters: cough headache secondary to Chiari malformation type I.

Pearls & oy-sters: cerebral venous air embolism after central catheter removal: too much air can kill.