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2/13/2015 > Disclosures Bad Behavior, or Something More? > None Case Presentations UCSF DEPARTMENT OF NEUROLOGY Division of Epilepsy Nilika Shah Singhal, MD February 2015 3 Case 1: continued Case 1: 10 year old boy with spells


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2/13/2015 1

Nilika Shah Singhal, MD

February 2015

UCSF

DEPARTMENT OF NEUROLOGY Division of Epilepsy

Bad Behavior,

  • r Something More?

Case Presentations

> Disclosures

> None

3 2

Case 1: 10 year old boy with spells

  • One month of spells

– Head shaking + head/eye version + behavioral arrests limb shaking

  • Diagnosed with PNES

– Negative MRI, EEG – Failure of OXC to control limb shaking events

  • Gait Disturbance

– Diagnosed with conversion disorder by neurology

  • Mood Disturbance

– Withdrawn, anxious, dysphoric – Diagnosed with generalized anxiety disorder by psychiatry

2

Case 1: continued

  • Examination: vital signs within normal limits
  • Alert, oriented. Blunted affect. Tearful, asking “please help.”
  • Face symmetric, EOMI, no drooling. Dysarthria.
  • Normal muscle bulk throughout. Normal tone in arms, Paratonia

in both legs. Left pronator drift.

  • Tendon reflexes 2+/symmetric in arms; 3+ left>right patella, 5

beats clonus left ankle, 2 beats clonus right ankle

  • No ataxia, dysmetria
  • Sensation intact to light touch
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Case 1: continued

  • Several typical spells seen during the

evaluation: feeling of “ about to have one”

  • Hypermotor legs
  • Erratic clonic jerking + tonic stiffening of left leg

with left arm stiffening and right arm flexion.

  • Duration <30 seconds, no impaired awareness

during the episode, no post-ictal state.

2

Case 1: continued

2

Case 1: Q1: How would you characterize this event with bilateral motor symptoms but preserved awareness?

  • A. Psychogenic non-epileptic seizure
  • B. Supplementary Motor Area seizure
  • C. Movement Disorder (dyskinesia)
  • D. Behavioral disturbance

P s y c h

  • g

e n i c n

  • n
  • e

p i l e p t i . . . S u p p l e m e n t a r y M

  • t
  • r

A . . . M

  • v

e m e n t D i s

  • r

d e r ( d y s . . . B e h a v i

  • r

a l d i s t u r b a n c e

9% 4% 13% 74%

2

Case 1: continued

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2

Case 1: Q2: What would you predict would be the next diagnostic step?

  • A. Another MRI
  • B. Blood tests to search for vasculitis
  • C. Psychiatry referral for a frustrated

patient and neurologist

  • D. Lumbar puncture to search for

inflammation

A n

  • t

h e r M R I B l

  • d

t e s t s t

  • s

e a r c h f

  • r

. . . P s y c h i a t r y r e f e r r a l f

  • r

a f . . . L u m b a r p u n c t u r e t

  • s

e a r . .

53% 28% 1% 18%

2

Case 1: Diagnosis: Anti-NMDAr Encephalitis

  • Psychiatric episodes can occur at presentation, in

isolation, in children

– Delusional thinking, mood disturbances, aggression

  • A wide variety of movement disorders are seen in

children

– Patients commonly present with more than one movement disorder – Gait impairment, chorea, stereotypic movements, ataxia, dystonia, limb monorhythmia, athetosis, tremor, blepharospasm > Next case

3 2

Case 2: 5 year old boy with episodes of anxiety

  • Two weeks of spells

– Sudden-onset crying, seeming anxious and fearful; makes eye contact with grandmother and reaches out for her – He may ‘thrash about’ seem generally tremulous. – Self-resolving in <30 seconds. – Afterwards, he resumes what he was doing. – Patient seems scared in even trying to describe his experience

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2

Case 2: continued

  • Occurred from wakefulness initially; started to occur

from sleep

  • Three hospital visits

– Toxicology screen, head CT, EEG obtained

  • Diagnosed with psychosis; placed on a psychiatric

hold – Received Risperdal, Benadryl, Valium

2

Case 2: Q1: What do you think is the next most useful diagnostic test to perform?

  • A. Brain MRI
  • B. Lumbar Puncture
  • C. Extended video EEG monitoring
  • D. Serum tests for metabolic diseases

B r a i n M R I L u m b a r P u n c t u r e E x t e n d e d v i d e

  • E

E G m

  • n

. . . S e r u m t e s t s f

  • r

m e t a b

  • l

i c . . .

16% 5% 73% 7%

2

Case 2

3

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Case 2: Further work-up

2

Case 2: Features of cingulate gyrus seizures

  • Hypermotor
  • Ictal Vocalization
  • Infrequent generalization
  • Presence of emotionality: fear, laughter
  • Presence of personality changes

2

Case 2: Q2: What are advantages of epilepsy surgery compared to medical management of medically-refractory focal cortical dysplasia?

  • A. Improved life expectancy
  • B. Seizure-free rates higher than medical

management

  • C. Improved IQ
  • D. Answers 1 and 2
  • E. All of the above

I m p r

  • v

e d l i f e e x p e c t a n c y S e i z u r e

  • f

r e e r a t e s h i g h e r . . . I m p r

  • v

e d I Q A n s w e r s 1 a n d 2 A l l

  • f

t h e a b

  • v

e

0% 10% 68% 21% 0%

3

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2

Referring and Provider consultation, Access Center: Phone: (855)-PBC-UCSF

> Thank you!

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