Donuru Achala,  Kandula Vinay,  Averill Lauren

Final Pr. ID: Poster #: EDU-073

The purpose of educational exhibit is to summarize the radiological appearances of various conditions causing basal ganglia (BG) calcification in children. The pathogenesis for symmetric BG calcification is diverse and ranges from benign physiological calcifications to a variety of pathological disorders including metabolic, infectious and genetic diseases. We present a practical approach to further narrow the differential diagnosis based on associated musculoskeletal imaging findings in patients with BG calcification.
Parathyroid disorders are the most common causes of pathological BG calcification. In hyperparathyroidism, distinctive subperiosteal bone resorption can be seen in phalanges on hand radiographs. Tapering of the clavicles, brown tumors and salt-and-pepper appearance of the skull are also classic radiographic features. In the Albright hereditary osteodystrophy phenotype of pseudohypoparathyroidism, shortening of the fourth and fifth metacarpals as well as advanced bone age can aid in diagnosis. Tumoral calcinosis is another radiographically distinct disease that can cause BG calcification, characterized in the extremities by periarticular calcific deposits. Cockayne syndrome is a rare autosomal recessive (AR) disorder with 4 overlapping subtypes, all with BG calcification as well as diffuse skeletal abnormalities that become apparent in the toddler years. Carbonic anhydrase deficiency type 2 is another rare AR disorder with intracranial calcification including the BG, as well as osteopetrosis and pathologic fractures. Coat’s plus syndrome has characteristic rock-like intracranial calcifications paired with leukodystrophy and brain cysts. It manifests skeletally with osteopenia, delayed fracture healing, bowing of long bones, scoliosis, midface hypoplasia and femoral head avascular necrosis. Other common conditions such a Down syndrome and HIV, and rare disorders such Fahr's disease, malignant phenylketonuria, and Aicardi-Goutieres syndrome are in the differential diagnosis of BG calcifications. Read More

Authors:  Donuru Achala , Kandula Vinay , Averill Lauren

Keywords:  basal ganglia, calcification, musculoskeletal

Tierradentro-garcia Luis,  Martinez-correa Santiago,  Forero-millan Julian,  Martinez Mesha

Final Pr. ID: Poster #: CR-043

Background:

Pediatric acute-onset neuropsychiatric syndrome (PANS) is a rare neuroinflammatory entity that is often associated with infectious and non-infectious triggers. This condition was first associated with recent Streptococcal infections (PANDAS) in the late 1990s. PANS manifests with sudden obsessive-compulsive disorder and/or restricted food intake with additional neuropsychiatric abnormalities. Children with PANS demonstrate generalized increased median diffusivity in the brain, more prominently in the deep gray matter, including the thalami, basal ganglia, and amygdala. Although neuroimaging plays a supportive role in diagnosis, its use is limited by the lack of positive findings in cases with clinically diagnosed PANS.

Purpose:

We aim to showcase brain MRI findings in a child with a clinical diagnosis of PANS at different time points. Our goal is to build on the potential role of neuroimaging in this condition in the acute and subacute phases.

Case description:

We discuss the case of an 11-year-old boy who presented with fever and altered mental status two weeks after having an upper respiratory infection. The patient underwent a contrast-enhanced brain MRI, which showed increased T2W/FLAIR signal and swelling of the caudate nuclei and putamina bilaterally, with no abnormal enhancement or restricted diffusion; notably, there was mild increased diffusion in the basal ganglia, likely reflecting vasogenic edema. Five days later, a repeat brain MRI revealed mild, diffuse high T2W/FLAIR signal, which was overall decreased compared to the prior scan. Additionally, diffusion in the caudate relatively decreased compared to the putamina. Twelve days later, there was a continued decrease in the T2W/FLAIR signal in the putamina, caudate nuclei, and possibly the lateral portion of the globi pallidi.

Discussion points:

-To present common neuroimaging findings in patients with suspicion/diagnosis of PANS/PANDAS over time, including increased T2W/FLAIR signal and mild increased diffusion in the basal ganglia.
-To discuss the current roles/limitations of neuroimaging in the workup based on the existing literature.
-To illustrate the primary differential diagnoses that can mimic PANS/PANDAS neuroimaging findings in children, including autoimmune encephalitis, Sydenham chorea, mitochondrial disorders, and tic disorders.
-To hypothesize the role of glymphatic, CSF, and venous dynamics in neuroinflammation in PANS/PANDAS related to early vasogenic edema. Read More

Authors:  Tierradentro-garcia Luis , Martinez-correa Santiago , Forero-millan Julian , Martinez Mesha

Keywords:  Pediatric Neuroradiology, Basal Ganglia, Diffusion Weighted Imaging

Venkatakrishna Shyam Sunder,  Elsingergy Mohamed,  Worede Fikadu,  Andronikou Savvas

Final Pr. ID: Poster #: SCI-012

Perinatal Hypoxic Ischemic Injury (HII) has a higher prevalence in the developing world. One of the primary concepts for suggesting that an imaging pattern reflects a global insult to the brain is when the injury is noted to be bilateral and symmetric in distribution. In the context of HII in term neonates, this is either bilateral symmetric (a) peripheral/watershed injury (WS) or (b) bilateral symmetric Basal-ganglia-thalamus (BGT) pattern, often with the peri-Rolandic and hippocampal injury. Unilateral, asymmetric or unequal distribution of injury may therefore be misdiagnosed as perinatal arterial ischemic stroke. This has been previously reported in the typical distribution patterns, without a clear indication of the prevalence. We aimed to determine the prevalence of unilateral, asymmetric and unequal degree HII and to characterize the distribution on MRI in a large database of patients with Cerebral Palsy (CP) from South Africa. Read More

Authors:  Venkatakrishna Shyam Sunder , Elsingergy Mohamed , Worede Fikadu , Andronikou Savvas

Keywords:  Hypoxic Ischemic Injury (HII), Basal-ganglia-thalamus and Watershed, Unilateral, Asymmetric or Unequal distribution