Cases for Clinical Neuroanatomy

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Cases for Clinical Neuroanatomy

• The Cerebellum

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Case 1 J.N.

• 11 year old, previously healthy girl from London
• 6 week history of headache, fatigue
• 2 week history of clumsiness and vomiting with
  headache
• Weight loss not quantified, but clothing fitting
  unusually loosely
• Seen by locum for GP in Byron re worsening HA
  and vomiting
• Clinical pickup of papilledema prompted urgent
  referral to Peds ER and CT scan
• Other focal findings of Left dysmetria, past
  pointing and tremor with unsteady gait and
  positive Rhomberg sign

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• Seen in ER October 18, 2007
• Findings of significance as noted
• CT head performed and Pediatric Neurosurgery
  contacted

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• Admitted to Childrens from the ER
• Initiated treatment with Decadron
• MRI head obtained
• Decided against placement of EVD up front, but an
  EVD was kept at the bedside in the event of acute
  deterioration (due to sudden complete obstructive
  hydrocephalus)
• Brought to OR on October 25 for suboccipital
  craniectomy and gross total removal of posterior
  fossa tumor and insertion of right frontal EVD
• Frozen section and final pathology
  medulloblastoma

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• Postop MRI showed gross total removal of tumor
  and resolution of hydrocephalus with external
  drainage (note no blood in ventricles)
• No new neurological deficits postop

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Case 2 T.C.

• 28 month old female from Guelph, Ontario
• Presented with a 2 week history of unwellness
• recurrent, intermittent vomiting associated with
  occasional headache
• Seen x3 at GP, walk-in clinic and hospital prior
  to admission to Guelph General Hospital October
  7, 2007 for persistent vomiting, lethargy and
  dehydration
• Mother says symptoms were initially attributed to
  a viral illness

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• Vomiting typically occurred immediately
  post-eating and in the mornings on waking, but
  not daily
• Non-bilious, no blood in emesis, contains just
  the food the child had eaten no abdominal pain,
  no diarrhea
• Afebrile, with no ill-contacts no history of
  recent travel
• no ear pain/pharyngitis
• No changes noted in vision, behaviour, speech,
  gait
• Weight loss of 6 lbs over 2 weeks

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• Past Medical History
• Born at term following a normal pregnancy
• Vaginal delivery no complications
• Normal milestone acquisition
• No serious illnesses requiring investigation/hospi
  talization
• No prior surgery
• No medication, no allergies
• Family history of migraines- mother

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• Presents to Guelph ER with vomiting October 7
• Temp 37.5, HR 106, RR 20, O2 sat room air- 100
• Hemodynamically stable cap refill lt 2 seconds
• Irritable during exam but alert and in no
  distress
• Weight 11.4 kg
• HEENT all WNL- eye exam felt to be WNL
  (fundoscopy not documented) neck supple TMs
  normal sl. enlarged tonsils, no nodes
• CVS all WNL
• Resp- good air entry bilaterally, no abnormal
  sounds, mild nasal discharge
• Abdomen- entirely WNL- no tenderness,
  organomegaly, normal bowel sounds

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• Admitted to pediatric ward
• IV started, bloodwork (CBC, lytes, gluc, urea,
  creat) done- all normal
• Started on amoxil 300 mg tid po for presumed
  respiratory infection or sinusitis prn Tylenol
• Some thought was given to arranging CT on October
  8

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• October 9
• Nurses called by mother at 0900 hrs
• Child found unresponsive, not breathing
• Mother reports that overnight, mother slept
  beside the child and she grunted during sleep and
  tended several times to extend her arms and
  stiffen her legs- mentioned to ward nurses, who
  checked her, found her breathing and sleeping
  peacefully
• Found by MD shortly after, in nurses arms,
  cyanotic, apneic
• HR 80/min unresponsive to voice, extending arms
  intermittently
• Bag and mask ventilation initiated

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• emergency endotracheal intubation, performed
  without need for either an induction agent or
  muscle relaxation
• Full ventilatory support required- no respiratory
  efforts
• Phenobarb 60 mg given
• 0955 hrs Pupils noted to be non-reactive and
  asymmetric (RgtL) no movement of the extremities,
  no tonic-clonic activity hyperventilation was
  initiated

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• Note was made during transfer of some spontaneous
  movements and she made some respiratory efforts
  en route to London
• On arrival to PCCU 1440 hrs
• Intubated, ventilated
• Extremities cool to touch
• HR 120/min, RR 12 (no breaths over vent), BP
  98/63
• Sats 99 on 0.25 FiO2 Temp 32.9

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• Neurological exam at arrival
• Pupils 5mm bilat, non-reactive
• Bilateral papilledema
• Neck supple
• Absent corneal, nasal tickle, gag, cough reflexes
  (including with deep tracheal suctioning)
• No movement of extremities to pain
• Absent deep tendon reflexes
• Flaccid tone in all extremities
• GCS 3(T)

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• Neurosurgical intervention
• Placement of right frontal EVD with extremely
  high opening pressure documented (30cm H20)
• No improvement neurologically with relief of ICP
• Discussion with family
• Poor prognosis (likely not survivable) with
  evidence of completed central herniation
• Warm to normothermia, withdraw all sedating drugs
  and proceed with brainstem death determinations
  x2
• Family requested consideration for organ donation

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Bestak, M. Epidemiology of Brain Tumors, in
Tumors of the Pediatric Central Nervous System,
Thieme, 2001
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Posterior Fossa Tumors

• Majority of pediatric brain tumors are located in
  the posterior cranial fossa (70)- this location
  is life-threatening due to anatomical
  configuration
• Main tumor histologies here medulloblastoma,
  astrocytoma (often pilocytic), ependymoma
• Medullo 20 of pediatric brain tumors, 30 of
  posterior fossa tumors

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Case 3 J.V.

• 5 year old boy, previously healthy
• Putting up a cork-board with his father in his
  bedroom, behind a dresser
• Father forgot the level, went out of the bedroom
  to get it, leaving J.V. there, momentarily
• On return, he found the drill pulled off the
  dresser, on the floor and unplugged from the
  wall-outlet
• The tip of the bit was broken off, but he could
  not locate it

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• Over the next half of the day, J.V. complained of
  headache, vomited and mom noticed his right arm
  was clumsy
• Uses right arm and hand to feed himself, and was
  unable to direct food into his mouth with a fork,
  spoon or hold his cup without spilling it
• Was able to walk without difficulty, had no
  problems speaking, swallowing food, no problems
  with his vision
• Mom looked at his head and found a scab behind
  his ear- fresh brought to ER

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• On Examination awake, alert, oriented to his
  name, location, day of the week
• No cranial nerve anomalies (I- XII) and no
  fundoscopic abnormalities full visual fields
• Full strength, normal tone in arms and legs,
  except slightly diminished tone in right arm
• Normal sensation to light touch and pinprick
  throughout
• Symmetrical DTRs, toes downgoing, no clonus
• Gait normal
• Right hand dysdiadochokinesia and past-pointing
• Small scab behind the right ear

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Case 4 P.C.

• 42 year old right handed salesman at a clothing
  store
• Following intercourse, 2300 hrs, wife noted his
  speech to be slurry
• Patient complained of acute onset of HA, neck
  pain and stiffness, vertigo
• Noted by his wife to have a left facial droop,
  clumsy left arm and hand she called 911
• Paramedics noted left pupil anisocoria, BP
  172/112, left facial droop and clumsy left arm
  with dysarthria

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• On examination in ER
• Awake, oriented X 3 but dysarthric
• HR 75 BP 205/120
• left pupil 4mm and right 3mm
• Left-beating nystagmus at rest
• Unable to fully gaze to the right
• Left facial droop
• Power full in all extremities no pronator drift
• Dysmetria on the left and some difficulty with
  heel-shin testing bilaterally, but worse on the
  left

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• Previous history of hypertension on Altace for 6
  months prior to presentation
• Non-smoker, non-drinker
• No drug allergies
• No prior surgery

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• Initially did well but over the course of about
  12 hours, he became drowsy and progressed from
  obeying commands (GSC Motor scale 6) to
  localizing pain (GSC Motor Scale 5) to abnormal
  flexor posturing (GSC Motor Scale 3)
• CT scan repeated

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• An External Ventricular Drain was inserted (EVD)
  to help control ICP
• Despite this, he deteriored to extensor posturing
  (GSC motor score of 2)
• CT repeated again

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• Due to declining level of consciousness despite
  external drainage and CT showing expanding
  hematoma in cerebellum, brought to surgery to
  decompress the posterior fossa

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• Diagnosis Hypertensive cerebellar hemorrhage
• Intraparenchymal hemorrhage 10 of strokes
• Twice as common as SAH (15 cases per 100,000/yr)
• Main cause of death is cerebral herniation (occur
  mainly in the first 48 hours after a bleed and
  GCSlt7 at presentation)
• Incidence increases significantly gt55 years
• Onset is usually during activity (rarely during
  sleep)
• Common sites 50 basal ganglia, 15 thalamus
  15 pons 10 cerebellum 10 cerebral white
  matter

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Case 4 S.C.A.

• 50 year old right handed man with acute headache
  vomiting
• Staggering walk broad-based gait, drifting to
  the left
• Mental status was normal but cranial nerve exam
  showed nystagmus to the left on attempted
  horizontal gaze to the left
• All volitional eye movements were normal but the
  left pupil was smaller than the right though both
  responded normally to light
• Left palpebral fissure narrower than the right
• Sensation on face normal corneal reflexes normal
• Muscles of the lower face and mastication weaker
  on the right
• Intention tremor of the left arm and leg
• Two-point discrimination on the body was normal
  but pain and temperature sensation reduced in the
  right arm and legs

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• superior cerebellar artery syndrome
• ipsilateral cerebellar ataxia ( middle /-
  superior cerebellar peduncles ), nausea,
  vomiting, slurred speech, loss of pain and
  temperature over the opposite side of the body
• tremor of upper extremity, ipsilateral Horner
  syndrome and palatal myoclonus
• Clinically may be impossible to distinguish from
  a partial AICA or PICA territory stroke
• Diagnosis via MRI

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• Nystagmus- due to involvement of MLF
• Ipsilateral Horners syndrome- due to involvement
  of oculosympathetic fibers
• Ipsilateral ataxia- involvement of superior
  cerebellar peduncle
• Ipsilateral intention tremor- due to involvement
  of dentate and superior cerebellar peduncle
• Contralateral trunk/ext hypalgesia,
  thermoanesthesia- due to lateral spinothalamic
  tracts

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Case 5 E.H.

• 8 year old right handed boy
• Healthy, with normal birth and developmental
  history
• Presented to GP prior to Christmas with otitis
  media- placed on antibiotics
• Over the course of January, a 3 week history of
  headaches, nausea waking him from sleep, usually
  worse in the mornings
• Headaches mainly at the back of the head
  throbbing
• Progressive balance problem with difficulty
  walking
• Clumsiness of the right hand

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• Examination shows
• Awake, alert, oriented pale low-grade temp 38.1
• Neck supple
• Unable to perform tandem gait broad based gait
• Finger-to-nose dysmetria on the right right hand
  dysdiadochokinesia
• No cranial nerve palsies
• Full power 5/5 in arms and legs, normal tone
• Symmetrical reflexes Gr. 2 downgoing plantars

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• Labs
• Electrolytes all wnl
• WBC 13.0 neut- 7.6, lymphs- 3.0, mono- 0.6
• Hb 118
• Plts 722 (elevated)
• ESR 68 (elevated range 0-10)

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• Diagnosis otogenic cerebellar abcsess
• Organism Pseudomonas aeruginosa
• Brain abscess 1 of intracranial space-occupying
  lesions
• Micro-organisms may be introduced into the CNS
  via trauma, contiguuous pericranial infection,
  meningitis, hematogenous dissemination from
  distant infective focus
• Otogenic is the commonest source, with the
  temporal lobe or cerebellum as common locations

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• 4 stages of abscess formation
• Early cerebritis day 1-3
• Late cerebritis day 4-9
• Early encapsulation day 10-13
• Late capsule day 14 onwards
• Half of abscesses are polymicrobial (mixed
  aerobes and anaerobes)
• Medical treatment works well in the cerebritis
  stages
• Surgical treatment is needed in capsule stages,
  especially for lesions 3cm or more (poor
  antibiotic penetration)

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Case 6 N.D.

• 14 year old right handed boy Grade 9 student
• Complaining of one year of noticeable right eye
  inturning with progressive diplopia
• In retrospect, on school photos, right eye
  inturning has been present, but not really
  symptomatic, since grade 6.
• Associated with increasing headache, occipital or
  holocephalic
• jiggling eyes
• Problems with swallowing thin fluids-
  regurgitation,choking
• More need for asthma puffers this past year more
  coughing
• Never picked for teamstoo uncoordinated

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• On exam bright, alert, cooperative
• PERL 3mm no APD 20/25 without correction
• Obvious right eye inturning down-beating
  nystagmus bilaterally but EOM full no
  papilledema subjective diplopia worse on gaze to
  right
• Diminished gag refex
• Wide-based gait, unable to do tandem gait, unable
  to hop on one foot
• Past-pointing
• Decreased RAMs bilaterally

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Chiari Malformations

• May be congenital or acquired
• 4 types I thru IV
• Type I most common- low-lying cerebellar tonsils
  only
• Type II- accompanies spina bifida- multiple
  associated brain anomalies- congenital
• Type III- high cervical or occipital
  encephalocele
• Type IV- aplasia/hypoplasia of cerebellum

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• Symptoms brain stem, spinal cord, cerebellum
• Headache, neck pain
• Ataxia
• Nystagmus
• Cranial nerve dysfunction (especially lower)
• Hiccups
• Snoring, sleep apnea
• Dysarthria
• Numbness, weakness, wasting of hands or arms
• scoliosis

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Case 8 E.P.

• Newborn baby boy, born at 38 weeks gestation
• Delivered vaginally
• Found to have multiple lesions on his skin
• Hypotonic, poor reflexes
• Poor feeding and swallowing
• Some apnea, bradycardia spells

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Congenital Cerebellar Cysts

• Arachnoid cyst
• Dandy-Walker cyst or Dandy-Walker variant
• Mega cisterna magna
• Produce symptoms by compression of cerebellum /-
  brainstem
• Also associated with hydrocephalus (usually
  obstructive) and increased intracranial pressure
• Respond well to shunt insertion or open
  fenestration via craniotomy

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