Amit Kandel

Reversible Diffusion Weighted Imaging Changes in Propionic Acidemia

Propionic acidemia is an inborn error of metabolism with neurologic manifestations. We describe a 3-year-old boy with propionic acidemia presenting with a metabolic crisis including headache, vomiting, and altered mental status with metabolic acidosis. Electroencephalography showed focal slowing in right temporal region. Magnetic resonance imaging (MRI) of the brain showed restricted diffusion with apparent diffusion coefficient correlate in the right parietooccipital region. Correction of metabolic acidosis led to clinical improvement and normalization of MRI diffusion weighted imaging/apparent diffusion coefficient changes. This article suggests that restricted diffusion resulting from metabolic crises in propionic acidemia may be reversible in some cases.

Whole brain CT perfusion deficits using 320-detector-row CT scanner in TIA patients are associated with ABCD2 score

Background: Transient ischemic attacks (TIA) are cerebral ischemic events without infarction. The uses of CT perfusion (CTP) techniques such as cerebral blood volume (CBV), time to peak (TTP), mean transit time (MTT) and cerebral blood flow (CBF) provide real time data about ischemia. It has been shown that CTP changes occur in less sensitive CTP scanners in patients with TIA. Larger detector row CTP (whole brain perfusion studies) may show that CTP abnormalities are more prevalent than previously noted. It is also unclear if these changes are associated with TIA severity. Objective: To demonstrate that TIA patients are associated with perfusion deficits using whole brain 320-detector-row CT perfusion, and to determine an association between ABCD2 score and perfusion deficit using whole brain perfusion. Methods: We retrospectively reviewed all TIA patients for CTP deficits from 2008–2010. Perfusion imaging was reviewed at admission; and it was determined if a perfusion deficit was present along with vascular territory involved. Results: Of 364 TIA patients, 62 patients had CTP deficits. The largest group of patients had MCA territory involved with 48 of 62 patients (77.42%). The most common perfusion abnormality was increased TTP with 46 patients (74.19%). The ABCD2 score was reviewed in association with perfusion deficit. Increased age >60, severe hypertension (>180/100 mmHg), patients with speech abnormalities, and duration of symptoms >10 min were associated with a perfusion deficit but history of diabetes or minimal/moderate hypertension (140/90–179/99 mmHg) was not. There was no association between motor deficit and perfusion abnormality. Conclusion: Perfusion deficits are found in TIA patients using whole brain CTP and associated with components of the ABCD2 score.

Visual diagnosis: 6-month-old boy with strange body movements.

1. Osman Farooq, MD* 2. Ayesha Kirmani, MBBS† 3. Nitin Agarwal, MD* 4. Hema Purushotham, MD* 5. Amit Kandel, MD‡ 1. *Department of Pediatric Neurology, University of Buffalo, The State University of New York, Buffalo, NY. 2. †Liaqat University of Medical and Health Sciences, Jamshoro, Pakistan. 3. ‡Department of Neurology, University of Buffalo, The State University of New York, Buffalo, NY. * Abbreviations: ACTH: : adrenocorticotropin hormone ECMO: : extracorporeal membrane oxygenation ED: : emergency department EEG: : electroencephalography A 6-month-old boy with a history of seizures presents to the emergency department (ED) with new “strange body movements.” His mother first noticed this behavior 2 weeks ago, when his arms and legs would be held in a flexed position, lasting 1 to 2 seconds at a time (Figure 1). She said that these did not look like his typical seizures, and he remained alert and responsive during the episodes. After first witnessing these episodes 2 weeks ago, she did not notice them again until 1 week before presentation, at which time they began occurring every other day, increasing in frequency to 3 times per day, at times with full body flexing. Figure 1. Click here to view the video. On physical examination, his vital signs are within normal limits. He is smiling and playful. On motor examination, spontaneous movements of all extremities are seen but are slightly decreased on the left compared with the right. Bilateral fisting is noted, with increased tone in the left upper and lower extremities. Axial tone is mildly decreased for age. His history was notable for a cardiorespiratory arrest at 1 month of age secondary to respiratory syncytial virus bronchiolitis. At that time, he was resuscitated for 30 minutes, then given extracorporeal membrane oxygenation (ECMO). While receiving ECMO, he was noted to have episodes of left eyelid and arm twitching along with lip smacking. Electroencephalography (EEG) revealed status epilepticus. His seizures were pharmacologically controlled with phenobarbital and fosphenytoin. The patient was weaned off ECMO after 7 days. Subsequent EEGs revealed nonspecific abnormalities but no seizure activity. Magnetic resonance imaging, performed 12 days after the …