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ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy, MD, Secretary Arastoo Vossough, MD, PhD, Treasurer and Laurence Eckel, MD, Treasurer May 24, 2016

ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

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Page 1: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ASPNR INTERESTING CASE SESSION ASNR 54th Annual Meeting

May 23, 2016

Erin Simon Schwartz, MD, President

Susan Palasis, MD, Vice-President

Ashok Panigrahy, MD, Secretary

Arastoo Vossough, MD, PhD, Treasurer

and

Laurence Eckel, MD, Treasurer

May 24, 2016

Page 2: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

IN WHAT YEAR WAS THE ASPNR FOUNDED?

A.1992

B. 1993

C.1994

D.1995

E. 1996

Page 3: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

IN WHAT YEAR WAS THE ASPNR FOUNDED?

A.1992

B. 1993

C.1994

D.1995

E. 1996

Page 4: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ESS CASE 1

11 year old girl “progressively obtunded and vomiting”

Page 5: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Subdural hygromas

B. Subarachnoid hemorrhage

C. Anemia

D. Iatrogenic effect

WHAT IS THE DIAGNOSIS?

Page 6: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Subdural hygromas

B. Subarachnoid hemorrhage

C. Anemia

D. Iatrogenic effect

WHAT IS THE DIAGNOSIS?

Page 7: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Additional history we found in the medical record:

Patient with seizures and rapidly declining mental status after local anesthetic (mepivicaine) injected for dental procedure

Page 8: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

INTRAVASCULAR LIPID ADMINISTRATION

• Lipid infusions increasingly being used to treat local

anesthetic systemic toxicity (LAST)

• Possible antidotal effect of intravenous lipid emulsion

on action of lipophilic drugs, including local

anesthetics, first discovered in 1962

• First case reports of success in human in 2006 • Controversial, however, as efficacy and safety not clearly proven

Lipid Rescue - Efficacy and Safety Still Unproven. Höjer J, Jacobsen D, Neuvonen PJ, Rosenberg PH. Basic Clin Pharmacol Toxicol. 2016 May 2. doi: 10.1111/bcpt.12607. [Epub] PMID: 27136445

Page 9: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

SP CASE 1

• 7 month old previously healthy full term male

• Found unresponsive in daycare

• Caregiver stated that the baby choked while taking the bottle

Page 10: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Non C+ CT

Page 11: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

3D Volume rendering

+ Retinal hemorrhages

Negative skeletal survey

Page 12: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

T2 FSE T2 FSE FS

Page 13: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

TOF MRA TOF MRV

Page 14: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Abusive head trauma

B. Vitamin K deficiency

C. Osler-Weber-Rendu syndrome

D. Menkes disease

WHAT IS THE DIAGNOSIS?

Page 15: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Abusive head trauma

B. Vitamin K deficiency

C. Osler-Weber-Rendu syndrome

D. Menkes disease

WHAT IS THE DIAGNOSIS?

Page 16: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

OSLER-WEBER-RENDU SYNDROME

• Osler–Weber–Rendu syndrome (Hereditary hemorrhagic telangiectasia (HHT))

• Autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and organs

Page 17: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

OSLER-WEBER-RENDU SYNDROME

Digital Subtraction Angiography

Page 18: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ABUSIVE HEAD TRAUMA SUBDURALS

T2 FSE

• Mixed intensity SDH

• Hematohygromas

Page 19: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

VITAMIN K DEFICIENCY

• Early (within 24 hrs), Classic (1-7 days of life), and Late (2-24 weeks)

• Neonates and infants at risk because Vit K transfer through the placenta is limited

T2 T1

Page 20: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

MENKES DISEASE

• X-linked recessive disorder of transmembrane copper (Cu) transport

• Rapid CNS degeneration

• Elastin collagen formation • Fragile, tortuous vessels

• Predisposition to ischemia

• Rapid brain atrophy leads to SDH

Page 21: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHO WAS THE FIRST ASPNR PRESIDENT?

A.Thomas P Naidich

B. Robert A Zimmerman

C.James A Brunberg

D.A James Barkovich

E. Patrick D Barnes

Page 22: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHO WAS THE FIRST ASPNR PRESIDENT?

A.Thomas P Naidich

B. Robert A Zimmerman

C.James A Brunberg

D.A James Barkovich

E. Patrick D Barnes

Page 23: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

AP CASE 1

Page 24: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,
Page 25: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

SECOND SIBLING

Page 26: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,
Page 27: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE MOST LIKELY DIAGNOSIS?

A. TORCH Infection

B. Zika Infection

C. Aicardi-Goutieres Syndrome

D. Chorioamnionitis

Page 28: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE MOST LIKELY DIAGNOSIS?

A. TORCH Infection

B. Zika Infection

C. Aicardi-Goutieres Syndrome

D. Chorioamnionitis

Page 29: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,
Page 30: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

AUTOSOMAL RECESSIVE PSEUDO-TORCH SYNDROME

• Phenotypic overlap with AGS (Aicardi-Goutieres syndrome)

• Failure to determine causative intrauterine infectious agent

• CSF leukocytosis with elevated interferon-alpha, neuropterins

• Hepatic transaminases and thrombocytopenia

Page 31: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Child with right leg weakness

AV CASE 1

Page 32: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE DIAGNOSIS?

A. RF213 mutation (inherited moyamoya)

B. COL3A1 mutation

C. ACTA2 mutation

D. PHACES (unknown mutation)

Page 33: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE DIAGNOSIS?

A. RF213 mutation (inherited moyamoya)

B. COL3A1 mutation

C. ACTA2 mutation

D. PHACES (unknown mutation)

Page 34: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ACTA2 MUTATION

Multisystem smooth muscle dysfunction Straight and narrow intracranial vessels – occlusions and ischemia

No moyamoya collaterals Small corkscrew distal vessels with tiny aneurysms

Page 35: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

HX of gastric dysmotility and congenital mydriasis. Several weeks of intermittent R hand numbness, progressively worsening slurred speech

and expressive aphasia

Courtesy of Michelle Silvera

Page 36: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

PHACES Posterior fossa malformations

Segmental hemangiomas

Arterial anomalies (narrowing, occlusions,

primitive connections, dilatation,

collaterals, anomalous course, later

aneurysms, etc.)

Cardiac defects

Eye abnormalities

Sternal or ventral defects

Page 37: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHO WAS THE FIRST FEMALE ASPNR PRESIDENT?

A.Tina Young Poussaint

B. Jill V Hunter

C.Nancy K Rollins

D.Patricia Davis

E. Mary K Edwards-Brown

Page 38: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHO WAS THE FIRST FEMALE ASPNR PRESIDENT?

A.Tina Young Poussaint

B. Jill V Hunter

C.Nancy K Rollins

D.Patricia Davis

E. Mary K Edwards-Brown

Page 39: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

12 year old obese female, headache for one week, nausea/vomiting for one day

ESS CASE 2

Page 40: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Pseudotumor cerebri

B. Normal MRI

C. Acute infarction

D. Venous Sinus Thrombosis

WHAT IS THE DIAGNOSIS?

Page 41: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Pseudotumor cerebri

B. Normal MRI

C. Acute infarction

D. Venous Sinus Thrombosis

WHAT IS THE DIAGNOSIS?

Page 42: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

PSEUDOTUMOR CEREBRI

• Lumbar puncture:

• Opening pressure: >60cm H20

• Headaches improved

• Discharged on: Methylprednisone taper, Acetazolamide, Gabapentin, Metoclopramide prn

Page 43: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

EXCEPT…

• One week after discharge, patient abruptly worsened

• 10/10 occipital headache, blurry vision, confusion, weakness, emesis, enuresis, encopresis, fall and seizure

• Sluggish 3mm pupils, bilateral papilledema, right CN VI palsy, hyporeflexia, unsteady gait, psychomotor slowing, and delayed, slurred speech

• Lumbar puncture:

• Opening pressure: >36cm H20

• Repeat 5 days later, opening pressure: 48cm H20

• All viral, toxicology, and rheumatological laboratory tests normal, aside from mild anemia

Raised papules on extremities

Page 44: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Progressive deterioration with bulbar signs, urinary retention, acutely decreased responsiveness

40lb weight loss since onset of illness (two months)

Page 45: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Over one week, mental status substantially declined

Minimally responsive to sternal rub, only able to

communicate by blinking, progressed to complete

unresponsiveness

Page 46: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Inborn error of metabolism

B. Vasculitis

C. Pseudotumor cerebri

D. Toxic exposure

WHAT IS THE DIAGNOSIS?

Page 47: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Inborn error of metabolism

B. Vasculitis

C. Pseudotumor cerebri

D. Toxic exposure

p-Dichlorobenzene: 2.8mcg/mL (normal = 0, reporting limit 0.020)

2,5-Dichlorophenol (DCB metabolite): 200mg/L (normal = 0, reporting limit 5.0)

WHAT IS THE DIAGNOSIS?

Page 48: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

LEUKOENCEPHALOPATHY SECONDARY TO

PARADICHLOROBENZENE TOXICITY

• Paradichlorobenzene in many forms of mothballs

• Patient kept many boxes in her poorly ventilated room for >3 years, “reminded her of her grandmother”

• Excessive inhalation or ingestion can cause a progressive leukoencephalopathy with ichthyotic rash and anemia, reports of intracranial hypertension

• Toxin stored & released from fat, acute worsening during catabolic stress (patient decompensated during stress & when NPO)

Twin Girls with Neurocutaneous Symptoms Caused by Mothball Intoxication.

http://www.nejm.org/doi/full/10.1056/NEJMc060329

Hernandez SH, Wiener SW, Smith SW. Case files of the New York City poison center:

paradichlorobenzene-induced leukoencephalopathy. J Med Toxicol. 2010

Jun;6(2):217-29

Page 49: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

• 3 yr old female with intermittent vomiting for 2.5 weeks

• Headache

• No fever

• Diagnosed with abusive head trauma at 5mos of age

T2 at 5mos

SP CASE 2

Page 50: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

3 years later

Ax Non C+ CT Cor Non C+ CT

Page 51: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Ax T2 FSE

Ax T1 C+

Cor T1 C+

Page 52: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

DWI ADC

Page 53: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Post-traumatic encephalomalacia

B. Intracranial epidermoid cysts

C. Malignant myxoid glial tumors

D. Abscesses

WHAT IS THE DIAGNOSIS?

Page 54: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Post-traumatic encephalomalacia

B. Intracranial epidermoid cysts

C. Malignant myxoid glial tumors

D. Abscesses

WHAT IS THE DIAGNOSIS?

Page 55: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

MALIGNANT MYXOID GLIAL TUMORS

• Very rare

• Meningeal glial rests

• Low grade and high grade histology

Page 56: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

BRAIN ABSCESS

T2 DWI ADC T1 C+

Page 57: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

INTRACRANIAL EPIDERMOID CYST

T1 C+ DWI ADC

Page 58: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE NAME OF THE ASPNR BEST PAPER

PRESENTATION AWARD?

A. The Really Great Paper Award

B. The Golden Brain Award

C. The Derek Harwood-Nash Award

D. The ASPNR Gold Medal Award

Page 59: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE NAME OF THE ASPNR BEST PAPER

PRESENTATION AWARD?

A. The Really Great Paper Award

B. The Golden Brain Award

C. The Derek Harwood-Nash Award

D. The ASPNR Gold Medal Award

Derek Harwood-Nash was one of the fathers of

pediatric neuroradiology and a former President of

the ASNR. In addition to his many tremendous

contributions to our field, he was instrumental in

establishing the ABR CAQ in Neuroradiology. The

ASPNR is proud to honor his memory with this award.

Page 60: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

AP CASE 2

Ophthalmoplegia (non-painful)

Page 61: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

INTRAOPERATIVE

ULTRASOUND

Page 62: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,
Page 63: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE MOST LIKELY DIAGNOSIS?

A. Meningioma

B. Hemangiopericytoma

C. Hemangioendothelioma

D. Schwannoma

Page 64: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE MOST LIKELY DIAGNOSIS?

A. Meningioma

B. Hemangiopericytoma

C. Hemangioendothelioma

D. Schwannoma

Page 65: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

HEMANGIOENDOTHELIOMA (VASCULAR TUMORS)

• Differentiation from hemangioma based on infiltrative growth pattern and predominant Kaposi sarcoma-like content (fascicles of spindle cells)

• Tumor can also have fibrogenic thrombi in the capillaries, hemorrhage, areas of lymphangiomatosis

Page 66: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

5 year old with progressive mental decline,

swallowing problems, and weakness

AV CASE 2

Page 67: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

MRS, TE=135ms

Page 68: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,
Page 69: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE DIAGNOSIS?

A. Leigh syndrome

B. LBSL

C. Listeria with rhombencephalitis

D. Late-onset Alexander disease

Page 70: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE DIAGNOSIS?

A. Leigh syndrome

B. LBSL

C. Listeria with rhombencephalitis

D. Late-onset Alexander disease

Page 71: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

• Childhood or adolescent onset and slow disease course. Patients develop insidious pyramidal, cerebellar, and dorsal column dysfunction

• Recessive mutations in DARS2, which encodes mitochondrial aspartyl-transfer ribonucleic acid synthetase

MS van der Knaap et al. A new leukoencephalopathy with brainstem and spinal cord

involvement and high lactate. Ann Neurol. 2003

LEUKOENCEPHALOPATHY WITH BRAINSTEM AND SPINAL CORD

INVOLVEMENT AND HIGH LACTATE (LBSL)

Page 72: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Steenweg et al. Neuropediatrics, October, 2012

Revised 2012 MRI criteria

Major criteria

Cerebral white matter (relative sparing of the subcortical white matter)

Dorsal columns and lateral corticospinal tracts of the spinal cord

Pyramids at the level of the medulla or decussation of the medial lemniscus

Minor criteria

Splenium of the corpus callosum

Posterior limb of the internal capsule

Superior and inferior cerebellar peduncles

Intraparenchymal part of the trigeminal nerve

Mesencephalic trigeminal tracts

Anterior spinocerebellar tracts in the medulla oblongata

Cerebellar white matter

LEUKOENCEPHALOPATHY WITH BRAINSTEM AND SPINAL CORD

INVOLVEMENT AND HIGH LACTATE (LBSL)

Page 73: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

LEIGH SYNDROME

SURF1 Mutation

Subacute necrotizing

encephalomyelopathy

Page 74: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

LISTERIA INFECTION AND RHOMBENCEPHALITIS

Listeria monocytogenes – gram+ bacillus

May lead to abscess

Page 75: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ALEXANDER DISEASE

- GFAP Mutation

- Often large head

- Initially anterior white matter involvement

- Late onset disease often has brainstem

findings

Page 76: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHO WAS THE FIRST WINNER OF THE DEREK HARWOOD-

NASH AWARD IN PEDIATRIC NEURORADIOLOGY?

A. A James Barkovich, MD

B. Aria Tzika, PhD

C. Charles Fitz, MD

D. William Ball, MD

Page 77: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHO WAS THE FIRST WINNER OF THE DEREK HARWOOD-

NASH AWARD IN PEDIATRIC NEURORADIOLOGY?

A. A James Barkovich, MD

B. Aria Tzika, PhD

C. Charles Fitz, MD

D. William Ball, MD

Derek Harwood-Nash Outstanding Pediatric Neuroradiology Paper:

Tzika AA, Barnes PD (mentor), Tarbell NJ, Nelson SJ, Scott RM.

“Multivoxel proton spectroscopy of childhood brain tumors",

presentation at ASNR 33rd Annual Meeting, Chicago, IL. 1995

Page 78: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ESS CASE 3

Page 79: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Hunter Syndrome (MPS II)

B. Achondroplasia

C. Osteopetrosis

D. Morquio Syndrome (MPS IV)

WHAT IS THE DIAGNOSIS?

Page 80: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Hunter Syndrome (MPS II)

B. Achondroplasia

C. Osteopetrosis

D. Morquio Syndrome (MPS IV)

WHAT IS THE DIAGNOSIS?

Page 81: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

23 Months

9 Years

Page 82: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

HUNTER SYNDROME MUCOPOLYSACCHARIDOSIS TYPE II

Imaging Findings:

Brain: Enlarged perivascular

spaces, delayed myelination,

white matter ↓T1/↑T2, volume

loss

Spine: Beaking of cervical

vertebrae, foramen magnum

and/or cervical stenosis,

cervical myelomalacia

Page 83: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

• Dysostosis with dwarfism, dental anomalies, corneal clouding, no direct CNS involvement (intellectually normal)

MORQUIO SYNDROME MUCOPOLYSACCHARIDOSIS TYPE IV

Spine Imaging Findings: Platyspondyly

with “ovoid” vertebral bodies, odontoid hypoplasia +/- cervical

subluxation, cervical stenosis and

myelomalacia

Page 84: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ACHONDROPLASIA

• Most frequent form of rhizomelic dwarfism

Imaging Findings: Caudal

narrowing of interpediculate

distance, notch-like sacroiliac

groove, exaggerated lumbar

lordosis, cervical stenosis

Fetal CT courtesy of Teresa Victoria

Page 85: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

OSTEOPETROSIS

Imaging Findings: Hyperdense bones

AD I - calvarium with sparing of spine

AD II - skull base, spine, pelvis

AR forms - more aggressive with

systemic manifestations

Page 86: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

• 7 mos old girl with decreased leg movement since 4 mos of age

• Difficulty with urination and bowel movements

Sag T2 Sag T1 Sag T1 C+ Cor T2

SP CASE 3

Page 87: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Ax T2

DWI ADC

SP CASE 3

Page 88: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Plexiform neurofibroma

B. Myxopapillary ependymoma

C. Differentiating neuroblastoma

D. Atypical teratoid rhabdoid tumor

WHAT IS THE DIAGNOSIS?

Page 89: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Plexiform neurofibroma

B. Myxopapillary ependymoma

C. Differentiating neuroblastoma

D. Atypical teratoid rhabdoid tumor

WHAT IS THE DIAGNOSIS?

Page 90: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

DIFFERENTIATING NEUROBLASTOMA

• Seven subtypes of neuroblastic tumors according to degree of neuroblastic maturation

MIBG

Page 91: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

MYXOPAPILLARY EPENDYMOMA

T1 T1 C+

Page 92: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ATYPICAL TERATOID RHABDOID

T2 FSE

T1 C+

3 weeks later

post excision

Page 93: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

PLEXIFORM NEUROFIBROMA

ADC

DWI

STIR

Page 94: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHO PLAYED NIKOLA TESLA IN THE MYSTERY THRILLER DRAMA “THE

PRESTIGE”?

A. Johnny Depp

B. Robert Downey Jr.

C. David Bowie

D. Justin Timberlake

Question courtesy of Susan Palasis

Page 95: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHO PLAYED NIKOLA TESLA IN THE MYSTERY THRILLER DRAMA “THE

PRESTIGE”?

A. Johnny Depp

B. Robert Downey Jr.

C. David Bowie

D. Justin Timberlake

pubs.acs.org selvedgeyard.com

Fetal CT courtesy of Teresa Victoria

Page 96: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Sagittal T2

Axial T2

AP CASE 3

Acute Flaccid Paralysis

Page 97: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE MOST LIKELY DIAGNOSIS?

A. Spinal Cord Infarct

B. Demyelination Lesion

C. Enterovirus myelitis

D. Metastatic disease

Page 98: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE MOST LIKELY DIAGNOSIS?

A. Spinal Cord Infarct

B. Demyelination Lesion

C. Enterovirus myelitis

D. Metastatic disease

Page 99: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ENTEROVIRUS D68 AND CNS DISEASE

2005 EV-D68 isolated from CSF in “a young adult” with flaccid paralysis1

2008

2014

2012 EV-D68 isolated from nasal swabs of two children w “polio-like” syndrome2

1Khetsuriani et al, MMWR, 2006 2Roux et al, Neurology. 2014;83(10):Supplement P3.335.

Page 100: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

0 2 4 6 810

12

14

16

18

20

22

24

26

28

30

32

33 - 73

0

2

4

6

Age (years)

Aff

ecte

d in

div

idu

als

Page 101: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

TAKE HOME POINTS

• Enterovirus myelopathy has fairly nonspecific imaging features • T2 prolongation in central gray, cervical

>thoracolumbar, variable nerve root enhancement

• Consistent clinical features help make diagnosis of Acute Flaccid Myelitis

• Motor recovery is usually incomplete

• Enterovirus species are the predominant AFM associate

Page 102: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

AV CASE 3

Page 103: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,
Page 104: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE DIAGNOSIS?

A. Fetal Teratoma

B. NF-1 with optic pathway glioma

C. Pallister-Hall syndrome

D. Orofaciodigital Syndrome

Page 105: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE DIAGNOSIS?

A. Fetal Teratoma

B. NF-1 with optic pathway glioma

C. Pallister-Hall syndrome

D. Orofaciodigital Syndrome

Page 106: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

PALLISTER-HALL SYNDROME

Hypothalamic hamartoma (5% of all cases)

Polydactyly - syndactyly

Anal atresia

Renal anomalies

Bifid epiglottis

Page 107: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

PALLISTER-HALL SYNDROME

Page 108: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

FETAL INTRACRANIAL

TERATOMA

Page 109: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT WAS THE FIRST NEUROIMAGING TECHNIQUE

DEVELOPED? A. Pneumoencephalography

B. Functional MRI

C. Human Circulation Balance

D. Cerebral angiography

Page 110: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT WAS THE FIRST NEUROIMAGING TECHNIQUE

DEVELOPED? A. Pneumoencephalography

B. Functional MRI

C. Human Circulation Balance

D. Cerebral angiography

Italian neuroscientist Angelo Mosso who

invented the 'human circulation balance',

which could non-invasively measure the

redistribution of blood during emotional

and intellectual activity.

Sandrone, S.et al. (2014). Weighing brain activity with the

balance: Angelo Mosso’s original manuscripts come to light.

Brain, 137(2), 621–633.

Page 111: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Former 26 week premature infant, now 5 months old, paraspinal lesion incidentally detected on

Chest CTA

ESS CASE 4

Page 112: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Sag T2 Sag T1

Cor STIR

Page 113: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Ax T2

Post Gad T1 Fat Sat

Page 114: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

I123 MIBG/CT Fusion

Page 115: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

I-123 MIBG SPECT/CT SCAN CAN BE NEGATIVE IN NEUROBLASTOMA

A. True

B. False

Page 116: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

I-123 MIBG SPECT/CT SCAN CAN BE NEGATIVE IN NEUROBLASTOMA

A. True

B. False

Page 117: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

I-123 MIBG SPECT/CT FOR NEUROBLASTOMA

Primary Tumor:

• Sensitivity 67-100%

• Specificity 68%

Metastases:

• Sensitivity 79-100%

• Specificity 33-89%

~10% False Negative Rate

Bleeker G, et al. 123I-MIBG scintigraphy and 18F-FDG-PET imaging for diagnosing neuroblastoma. Cochrane Database Syst Rev. 2015 Sep 29;9:CD009263

Page 118: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE DIAGNOSIS?

A. Neuroblastoma

B. Hematoma

C. Hemangioma

D. Neurenteric Cyst

Page 119: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE DIAGNOSIS?

A. Neuroblastoma

B. Hematoma

C. Hemangioma

D. Neurenteric Cyst

Page 120: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

INFANTILE CAPILLARY HEMANGIOMA

• Most common soft tissue tumor of infancy

• Incidence has steadily increased over past 35 years, correlating with decreasing gestational age at birth as well as birth weight

Anderson KR, et al. Increasing incidence of infantile hemangiomas (IH) over the past 35 years: Correlation with decreasing gestational age at

birth and birth weight. J Am Acad Dermatol. 2016 Jan;74(1):120-6

Page 121: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

GASTRULATION

From Carlson, Human Embryology and Developmental Biology, 2014

Page 122: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

NEURENTERIC CYST

Page 123: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

• 6yr old with increasing seizures

• Fever

Ax T2 Cor T2

SP CASE 4

Page 124: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Hypertrophic olivary degeneration (HOD)

B. Adrenomyeloneuropathy

C. Enterovirus rhombencephalitis

D. Neuromyelitis optica (NMO)

WHAT IS THE DIAGNOSIS?

Page 125: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

A. Hypertrophic olivary degeneration (HOD)

B. Adrenomyeloneuropathy

C. Enterovirus rhombencephalitis

D. Neuromyelitis optica (NMO)

WHAT IS THE DIAGNOSIS?

Page 126: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

HOD SECONDARY TO MITOCHONDRIAL DISORDER

• Hypertophy and increased signal in the inferior olivary nucleus

• Palatal tremor

• Rare in children but can be seen following posterior fossa surgery

• Disruption of triangle of Guillain-Mollaret

Goyal M et al. AJNR 2000

Page 127: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ADRENOMYELONEUROPATHY (AMN)

Axial T2

• Form of adrenoleukodystrophy • Long chain fatty acids deposited damage myelin

• Symmetric signal abnormalities along corticospinal tracts

• Atrophy

Courtesy of TAGM Huisman and A Poretti

Page 128: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ENTEROVIRUS RHOMBENCEPHALITIS

Sag T1 Ax T1 Ax T2

• Fever, flaccidity

• Dorsal brainstem, dentate nuclei, cord gray matter

Courtesy of TAGM Huisman and A Poretti

Page 129: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

NEUROMYELITIS OPTICA (NMO)

FLAIR Ax T2 Sag T2

• 70% test positive for aquaporin 4

• Involve periventricular structures,

periaqueductal gray, medulla at

obex

• Optic neuritis

Page 130: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

IN WHAT YEAR WAS THE FIRST JOINT ASFNR-ASPNR

SYMPOSIUM?

A. 2000

B. 2004

C. 2008

D. 2012

E. 2016

Page 131: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

IN WHAT YEAR WAS THE FIRST JOINT ASFNR-ASPNR

SYMPOSIUM?

A. 2000

B. 2004

C. 2008

D. 2012

E. 2016

Page 132: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

AP CASE 4

ACUTE RIGHT HEMIPARESIS RECENT BIOPSY OF SKIN LESION

Thanks to Dr Andre Furtado

Page 133: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,
Page 134: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,
Page 135: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

ONE YEAR LATER

Page 136: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE MOST LIKELY DIAGNOSIS?

A.Diffuse Axonal Injury

B. Hereditary Hemorrhagic Telangiectasia (HHT)

C. Sturge-Weber

D. Parry-Romberg Syndrome

Page 137: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS THE MOST LIKELY DIAGNOSIS?

A.Diffuse Axonal Injury

B. Hereditary Hemorrhagic Telangiectasia (HHT)

C. Sturge-Weber

D. Parry-Romberg Syndrome

Page 138: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

AJNR 36:1355-61, Jul 2015

Page 139: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

PARRY-ROMBERG SYNDROME

• Rare, self-limiting progressive hemiatrophy of the face (but brain abnormalities can present first)

• Skin lesion = Scleroderma

• Intracranial findings: parenchymal calcifications, hemorrhage, white matter lesions, atrophy

• Etiology unknown (autoimmune?)

Page 140: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

Intermittent exotropia, headache, and

papilledema

T2 T1 Gad T1

AV CASE 4

Page 141: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

SWI Magnitude DWI ADC SWI Phase

T2

Page 142: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,
Page 143: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS YOUR DIAGNOSIS?

A. Choroid plexus carcinoma

B. Supratentorial PNET

C. Meningioma

D. Cavernous Malformation

Page 144: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT IS YOUR DIAGNOSIS?

A. Choroid plexus carcinoma

B. Supratentorial PNET

C. Meningioma

D. Cavernous Malformation

Page 145: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

GIANT CAVERNOUS MALFORMATION

(CAVERNOMA)

T2 T1 Gad T1

Page 146: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

INTRAVENTRICULAR MENINGIOMA

T2 T1 Post CT

Page 147: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

SUPRATENTORIAL PNET

T2 DWI T1 Post T2* GRE

Page 148: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

CHOROID PLEXUS CARCINOMA

T2 DWI

T1 Post FLAIR

Page 149: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT WAS YOUR FAVORITE SESSION AT THE 2016 ASNR

ANNUAL MEETING?

A. Session 2A

B. Lunch

C. Session 3D

D. The Annual Business Meeting

E. THE ASPNR INTERESTING CASE SESSION

Page 150: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

WHAT WAS YOUR FAVORITE SESSION AT THE 2016 ASNR

ANNUAL MEETING?

A. Session 2A

B. Lunch

C. Session 3D

D. The Annual Business Meeting

E. THE ASPNR INTERESTING CASE SESSION

Page 151: ASPNR INTERESTING CASE SESSION...ASPNR INTERESTING CASE SESSION ASNR 54 th Annual Meeting May 23, 2016 Erin Simon Schwartz, MD, President Susan Palasis, MD, Vice-President Ashok Panigrahy,

www.aspnr.org

Facebook Group:

ASPNR