neurology case of the week n.
Download
Skip this Video
Loading SlideShow in 5 Seconds..
Neurology Case of the Week PowerPoint Presentation
Download Presentation
Neurology Case of the Week

Loading in 2 Seconds...

play fullscreen
1 / 35

Neurology Case of the Week - PowerPoint PPT Presentation


  • 112 Views
  • Uploaded on

Neurology Case of the Week. Become a member of Movember …Grow a Stache ! Ladies are welcome to join . Hassanain Toma , MD Neurology PGY-4 Movember 2 nd ,2012. Chief Complaint. Altered mental status: Lethargic -> obtunded. HPI. Young boy: Admission - 11 days:

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'Neurology Case of the Week' - ranit


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
neurology case of the week

Neurology Case of the Week

Become a member of Movember…Grow a Stache! Ladies are welcome to join 

HassanainToma, MDNeurology PGY-4Movember 2nd,2012

chief complaint
Chief Complaint

Altered mental status: Lethargic -> obtunded

slide3
HPI

Young boy:

Admission - 11 days:

Fever of 104 -> diagnosed with a sore throat and placed on antibiotics

Admission - 8 days:

Diarrhea after -> stopped abx -> diarrhea continued.

coughing, nasal congestion, and rhinorrhea

Admission - 7 days

Vomiting x 4 days, decreased PO intake.

Admission -1 day

crying episodes.

Admission:

Abdominal pain, screaming due to pain

He was transferred here for further evaluation his abdominal pain.

Next day became increasingly lethargic, and was intubated for airway protection.

slide4

GROWTH/DEVELOPMENT:

  • Growth delay and mild development delay.
  • Able to walk, speaks.
  • Attends kindergarten.

PAST MEDICAL/SURGICAL/BIRTH HISTORY:

  • Eczema
  • Hypothyroidism
  • milk allergy (has since grown out of this per mom)
  • ADHD.

PAST SURGICAL

  • No surgeries.

MEDICATIONS:

  • Levothyroxine
  • Antacid
  • strattera

Adverse Reactions/Allergies:

  • Milk Products(Rashes)

FAMILY HISTORY:

  • Asthma, HTN.

SOCIAL HISTORY:

  • Parents live separately. Preston spends time at each parent's house.
  • Dad smokes outside the home.

IMMUNIZATIONS:

  • Up to date per mom.
physical exam
Physical Exam

General: Intubated, appears of stated age. No spontaneous movement.

Head/Neck: Microcephalic. No neck masses.

Eyes: PERRL. Erythema of conjunctiva of left eye.

ENT: TM's pearly and nonbulging bilaterally. No erythema or exudate of oropharynx. Dry lips.

Chest: CTAB, no wheezing.

CV: RRR, no murmurs, rubs, or gallops.

Abdomen: abdomen is soft. Non distended. +BS

Lymph: No cervical LAD.

Skin: No rashes seen on visible skin.

Mental State: Obtunded, not responsive to stimuli.

CN II: PERRL slow reacting.

CN III & IV: Positve dolls.

CN V: Grimaces pain. Corneal reflex preserved in both eyes.

CN VI: Unable to access extra ocular movements intact bilaterally.

CN VII: Symmetrical face.

CN VIII: Unable to assess hearing.

CN IX & X: Gag present.

Motor: The tone is hypertonic with rigidity.

Sensory: withdraws to pain.

Reflexes: 3 diffusely. Upgoing toes.

Coordination: could not be tested.

Gait: could not be tested.

slide6
Labs

CSF

Clarity CLEAR

Color COLORLESS

RBC 0

WBC 2

Glucose 79

Protein 41

HEMATOLOGY

WBC 9.46

HGB 12.4

HCT 35.2%

Platelet 182

% Band 32.9 %

URINALYSIS/FECES

Color Ur STRAW

Clarity Ur CLEAR

Specific Gravity Ur 1.030

pH Ur 6.0

Glucose Ur NEGATIVE

Ketones Ur 2+ A

Protein Ur NEGATIVE

Blood Ur NEGATIVE

Bili Ur NEGATIVE

Urobilinogen Ur NORMAL

Nitrite Ur NEGATIVE

Leukocytes Ur NEGATIVE

WBC Ur 1-4

RBC Ur 1-4

Bacteria Ur NONE

Renal Epithelial Cells Ur FEW

Casts Ur NONE

Crystals Ur NONE

CHEMISTRY

Sodium 137

Potassium 4.0

Chloride 101

Carbon Dioxide 22

Anion Gap 14

Calcium 9.3

Glucose 93

BUN 10

Creatinine 0.31

C Reactive Prot 2.3 H

Protein Total 6.5

Alb 3.6

Bili, Total 0.6

Bili, Direct 0.0

Bili, Indirect 0.3

AST 146 H

ALT 138 H

AP 143

Amylase 70

Lipase 308 H

Sed Rate 34H

ENDOCRINOLOGY

TSH 3.02

T4 Free 1.5

slide7

What??

Where??

slide14
Labs

INF DIS/ANTIGEN/MOLECULAR

Adenovirus PCR Quant Plasma Not Dete

Adenovirus PCR Quant CSF Not Dete

West Nile PCR CSF Negative

West Nile PCR Blood Negative

EBV PCR Quant CSF Not Dete

VZV PCR Quant CSF Not Dete

SEROLOGY/INF DISEASE

E Equine EncephIgG CSF <1:10

E Equine EncephIgM CSF <1:10

CalifEncephIgG CSF <1:10

CalifEncephIgM CSF <1:10

St. Louis EncephIgG CSF <1:10

St. Louis EncephIgM CSF <1:10

W Equine EncephIgG CSF <1:10

W Equine EncephIgM CSF <1:10

West Nile Virus IgG CSF Negative

West Nile Virus IgM CSF Negative

Bart henselaeIgG <1:128

Bart henselaeIgM <1:20

Bart quintanaIgG <1:128

Bart quintanaIgM <1:20

Calif (LaCross) IgG <1:10

Calif (LaCross) IgM <1:10

E Equine EncephIgG <1:10

E Equine EncephIgM <1:10

St Louis EncephIgG <1:10

St Louis EncephIgM <1:10

W Equine EncephIgG <1:10

W Equine EncephIgM <1:10

MycoplasmaAbIgG 0.08

MycoplasmaAbIgGInterp Negative

MycoplasmaAbIgM 0.12

MycoplasmaAbIgMInterp Negative

  • MOLECULAR INF DISEASE

CMV PCR Quant NEG

Enterovirus RT-PCR NEG

Epstein Barr Virus PCR NEG

Herpes Simplex Virus PCR NEG

Respiratory Viral Panel PCR

Influenza A (subtypes H1, 2009 H1, H3)

Influenza B

Respiratory Syncytial Virus (RSV)

Adenovirus POS

Human Metapneumovirus

Parainfluenza 1,2,3,4

Rhinovirus/Enterovirus

Bordetellapertussis

Chlamydophila pneumonia

Mycoplasma pneumonia

Coronavirus (HKU1, NL63, OC43 and 229E)

BIOCHEMICAL GENETICS

Phosphoserine 7

Taurine 78

Phosphoethanolamine 0

Aspartic Acid 21

HydroxyProline 0

Threonine 304 H

Serine 132

Asparagine 73

Glutamic Acid 58

Glutamine 609

Sarcosine 0

Proline 153

Glycine 339

Alanine 464

Citrulline 9

Alpha Amino Butyric Acid 23

Valine 245

Cystine 57 H

Methionine 42

Cystathionine 0

Isoleucine 70

Leucine 152

Tyrosine 68

Phenylalanine 87

B-Alanine 0

Homocystine 0

Ornithine 80

Lysine 277

Histidine 74

Arginine 146 H

background
Background
  • Establishment as a new disease in 1995
  • Higher incidence in East Asian countries
  • Handful of cases in Caucasians
  • M=F
  • Peak at 6-18 months old, but can occur in up to 11yo
    • < 5yo 81.8%.
    • Mortality rate 31.8
    • Neurological sequelae(27.7%)
    • coagulopathy, hepatic dysfunction, and computed tomographic abnormalities

had a poor prognosis.

acute presentation
Acute Presentation
  • Convulsions are 1st sign of brain dysfunction
    • 0.5-3 days after onset of antecedent infections
histology encephalopathy
Histology-> encephaloPATHY
  • Necrosis (due to severe edema) in the thalami, tegmentum, and dentate nuclei
  • Florid petechial hemorrhage around small parenchymal vessels
  • Patchy cerebral white matter lesions of ANE are not hemorrhagic
  • Absence of inflammatory cells in brain parenchyma is characteristic,
    • Differentiates ANE from acute disseminated encephalomyelitis & acute hemorrhagic encephalitis
pathogenesis
Pathogenesis

1- Viral invasion of central nervous system

  • Controversial- via peripheral nerves?
  • positive viral RNA in CSF and brain but lack of inflammation in brain tissue of fatal cases
  • Not dependent on infectious agents.
  • Vascular endothelial cells, astrocytes and neurons -> apoptosis
  • Viral invasion likely a result not a cause of disease

2- Predisposition

  • Mutations in the gene Ran-binding 2 (RANBP2) associated with familial or recurrent viral ANE.
    • Autosomal-dominant ANE due to missense mutations in RANBP2
  • Hepatic and/or renal dysfunction

3- cytokine storm

  • proinflammatory cytokines
    • interleukin (IL)-6, IL-1b, tumor necrosis factor (TNF)-a, soluble TNF receptor
    • IL-6 level was correlated with worse prognosis
    • IL-6 and TNF-a -> apoptosis & injury of vascular endothelium, glial cells, and neurons,
      • -> vascular lesions and breakdown of the blood–brain barrier (BBB)
      • -> induce brain edema and damage, CNS disorders, and/or systemic symptoms
  • Other cytokines/chemokines
    • CXCL8/IL-8, CCL2/MCP-1, and CXCL10/IP-10
investigations bloods
Investigations - Bloods

Various abnormal findings

Elevation of serum aminotransferases and lactic dehydrogenase indicates liver dysfunction

Elevation of creatinekinase, urea nitrogen and amylase indicates concomitant involvement of the muscles, kidneys and pancreas respectively.

investigations imaging
Investigations - Imaging
  • Bilaterally symmetric lesions of the thalami
    • ± lateral putamin & external capsule, tegmentum, cerebellar nuclei
  • The lesions are often necrotic and hemorrhagic.
  • Diffusion-weighted imaging (DWI) -> cytotoxic edema.
slide26
Axial T2-weighted image showing bilaterally symmetric hyperintensity in the thalami. Note the target appearance of the lesions.

Axial T2-weighted image showing bilaterally symmetric hyperintensity in the dorsal pons. Coronal FLAIR images showing bilaterally symmetric hyperintensity in the thalami and dorsal columns.

treatment
Treatment

Prognosis

Early intervention improves outcome!

Often grave

back to our patient
BACK TO OUR PATIENT
  • Methylprednisolone 30mg q24hrs.
  • Mannitolat a 0.5g/kg q6 hour
    • Serum osm goal ~ 320.
  • 3% hypertonic saline
    • Na goal high 140 and low 150 range.
  • NO HYPOTHERMIA PROTOCOL
  • Patient died on Hospital day 8 (diffuse cerebral edema)
    • Autopsy: Diffuse brain edema, simplified broad gyri and friable brain parenchyma consistent with multifocal bilateral hemorrhagic and ischemic strokes (pending examination after fixation).
references
References

1: Neilson DE. The interplay of infection and genetics in acute necrotizing encephalopathy. CurrOpinPediatr. 2010 Dec;22(6):751-7. Review. PubMed PMID:

21610332.

2: Wang GF, Li W, Li K. Acute encephalopathy and encephalitis caused by influenza virus infection. CurrOpin Neurol. 2010 Jun;23(3):305-11. Review. PubMed PMID: 20455276.

3: Mizuguchi M, Yamanouchi H, Ichiyama T, Shiomi M. Acute encephalopathy associated with influenza and other viral infections. ActaNeurolScandSuppl. 2007;186:45-56. Review. PubMed PMID: 17784537.

4: Mastroyianni SD, Gionnis D, Voudris K, Skardoutsou A, Mizuguchi M. Acute necrotizing encephalopathy of childhood in non-Asian patients: report of three cases and literature review. J Child Neurol. 2006 Oct;21(10):872-9. Review.

PubMed PMID: 17005104.

5: Mizuguchi M. Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev. 1997 Mar;19(2):81-92. Review. PubMed PMID: 9105653.