CEREBELLUM & ITS
CONNECTIONS (applied)
Dr. Md. Saiduzzaman (Munna)
MD Resident (Neurology, Phase B)
Department of Neurology
MMCH.
contents
 Examination
 Ataxia
 Cerebellar disorders
 Case scenarios
Examination
 Speech
 Nystagmus
 Finger nose test
 Dysdiadocokinesia
 Rebound phenomenon
 Muscle tone
 Knee jerk
 Stance and gait.
Ataxia
It is a group of disorders that affect co-ordination,
balance and speech and may result from a disorder
of the cerebellum and/or its connections, vestibular
or sensory disorders.
Symptoms in Patients with Ataxia
 Patients with cerebellar and sensory ataxia often
present with abnormalities of gait.
 The initial symptoms may be a sense of insecurity
while walking, especially when performing acts
that require a bit more skill, such as turning or
balancing on a narrow ledge. Even before gait
becomes abnormal, patients may note problems
with specialized skills such as bicycling or
climbing.
 Sensation is more like being on a boat.
Symptoms…
 Limb Ataxia
Clumsiness with activities such as writing, picking up objects,
and buttoning. Movements tend to become slower.
These symptoms are one-sided with lateralized lesions of the
cerebellum.
 Truncal Ataxia
Midline cerebellar lesions cause truncal ataxia. Patients may
experience head tremor and truncal instability leading to
oscillatory movements of the head and trunk while sitting or
standing (titubation). They may need back support while
sitting.
Symptoms…
 Dysarthria and Bulbar Symptoms
Ataxic diseases of cerebellar origin result in
slurred speech and abnormalities of pitch and
volume control (scanning speech).
 Dysphagia can result from incoordination of
swallowing muscles, and patients report
strangling and choking. Ineffectiveness of cough
may also be a symptom.
Symptoms…..
 Visual Symptoms
Patients may experience blurriness or a sense of
environmental movements as a result of cerebellar ocular
oscillations associated with cerebellar disease.
 Symptoms in Sensory Ataxia
Patients with a sensory basis for ataxia usually do not
experience dysarthria or visual symptoms. They may report
other symptoms of sensory pathway disease such as
paresthesias and numbness.
Midline…
 Lesion in cerebellar vermis.
 Causes “truncal ataxia” where the affected person is unable to
sit on their bed without steadying themselves.
 Often affects eye movements; there may be nystagmus, ocular
dysmetria and poor pursuit.
Differences between cerebellar and
sensory ataxia
Cerebellar ataxia Sensory ataxia
Sensory symptoms absent Present
Other cerebellar signs present Absent
Objective sensory loss absent Present
Broad based, staggering gait High stepping gait
Romberg’s sign absent Present
Vertigo may be present Absent
MRI is the investigation of choice NCS, biochemical parameters and
infection screen.
Neuro-imaging abnormalities pointing to
diagnosis of ataxia
Cerebellar disorders
 Mass in the cerebellum/ posterior fossa
Gliomas, meningiomas,Medulloblastoma
abscess
 Abnormal craniovertebral junction
Arnold-Chiari malformation,
 Infarcts, vascular malformations
Ischemic lesions, Haematoma, AVM
Cerebellar disorders……
 Signal density change in the cerebellum
MS, acute cerebellitis
 Cerebellar atrophy
- SCAs
- idiopathic cortical cerebellar atrophy
- drugs and toxin,
- Vitamin E deficiency
- Autoimmune ataxias
- Dandy-Walker syndrome
Cerebellar abscess
Arnold-Chiari malformation
Cerebellar infarction
Cerebellar haematoma
Multiple sclerosis
Acute cerebellitis
Dandy-Walker malformation
Case scenario 1
• A 51 years old diabetic, hypertensive male
with sudden onset difficulty walking and
tendency to fall to left side 03 days
• Intention tremor on left side
Case scenario 2
A 40 yrs old Diabetic male presented
to an ENT specialist with
complaints of
▪vertigo– for 15 days.
▪ discharge from left ear—
for3 months.
O/E : ▪ Pt is pyretic.
▪ scanning speech.
▪cerebellar ataxia
Infections affecting cerebellum
•Bacterial- cerebellar abscess,
tuberculoma
•Viral – acute- varicella.
chronic- HIV.
•Parasite- Toxoplasma, F. malaria,
•Prions- CJD, Kuru.
Case scenario 3
A 16 year old boy with
Progressive Gait ataxia for
02 years
Bilaterally symmetrical
intention tremor
Ankle reflex lost
Plantar bilateral extensor
Case scenario 4
• A 30 years old lady
• Recurrent ataxia and Recurrent visual
blurring for 03 episodes in the last 02
years
• INO
• Bilateral plantar
extensor
Case scenario 5
A 35 yrs old epileptic male pt.
on AED for 5 yrs with
complaint:
- Difficulty in walking – 5
months.
- Dysarthria for - 4 months.
O/E :
- Mildly anaemic,
- Scanning speech +
- truncal ataxia +
Investigation: PBF----
pancytopenia.
DRUGS & TOXIN AFFECTING
CEREBELLUM
 Alcohol
 Anticonvulsant drugs- Phenytoin
 Chemotherapy- 5-flurouracil , cytosine
arabinose
 Heavy metals-mercury , manganese , bismuth
 Chronic solvent abuse
Dandy Walker Malformation
Clinical presentation:
Infancy: symptoms and signs of hydrocephalus with prominent occiput.
Childhood: signs of cerebellar dysfunction with or without signs of
hydrocephalus.
Investigation: CT scan or MRI
Management: Cysto peritoneal shunt.
Arnold-Chiari malformation
 There is displacement of cerebellar tonsils and vermis through the
foramen magnum
 There is compression of spino-medullary junction
 Often associated with syringomyelia and hydrocephalus
CHIARI MALFORMATION
• Type I: No meningomyelocoele
• Type II: With meningomyeocoele (lumber)
• Type III: With occipitocervical meningomyelocoele
• Type IV: Only cerebellar hypoplasia
• Headache (raised ICP)
• Progressive ataxia
• Spastic quadriparesis
• Lower cranial nerve involvement
• Downbeat nystagmus
• Features of syringomyelia
Case scenario 6
A 10 year old boy presents with
vomiting, unsteady gait, slurred
speech, headache and diplopia
TUMOURS OF CEREBELLUM
Cerebellar tumors may be –
Primary-
Common in children
secondary-
Common in adults
Clinical features-
-ataxia
-brainstem dysfunction
-features of raised ICP.
Primary tumors of cerebellum
Medulloblastoma
Astrocytoma
Hemangioblastoma
Ependymoma
Oliogodendroglioma
Medulloblastoma, astrocytoma- common in
children
Hemangioblastoma- common in adults
Case scenario 7
• 50 years old male
patient
• Gait ataxia for 08
weeks
• Bilaterally
symmetrical
intention tremor
• Smoker for 20
years
• Clubbing
PARANEOPLASTIC CEREBELLAR
DEGENERATION
Common neoplasms associated-
SCLC, Ca Breast, Ca Ovary,
Hodgkin’s lymphoma
Immune associations-
Anti-Yo ab Ca breast
Anti Ri ab SCLC
Anti VGCC SCLC
Very few responds to therapy.
Metabolic disorders affecting
cerebellum
 Hypothyroidism
 Hypoxia
 Hypoglycemia
 Hyponatremia
 Deficiency disorders (vitamin B1, B12, E)
 Inborn errors of metabolism( lipid /amino acid
metabolism)
Cerebellum clinical

Cerebellum clinical

  • 1.
    CEREBELLUM & ITS CONNECTIONS(applied) Dr. Md. Saiduzzaman (Munna) MD Resident (Neurology, Phase B) Department of Neurology MMCH.
  • 2.
    contents  Examination  Ataxia Cerebellar disorders  Case scenarios
  • 3.
    Examination  Speech  Nystagmus Finger nose test  Dysdiadocokinesia  Rebound phenomenon  Muscle tone  Knee jerk  Stance and gait.
  • 4.
    Ataxia It is agroup of disorders that affect co-ordination, balance and speech and may result from a disorder of the cerebellum and/or its connections, vestibular or sensory disorders.
  • 7.
    Symptoms in Patientswith Ataxia  Patients with cerebellar and sensory ataxia often present with abnormalities of gait.  The initial symptoms may be a sense of insecurity while walking, especially when performing acts that require a bit more skill, such as turning or balancing on a narrow ledge. Even before gait becomes abnormal, patients may note problems with specialized skills such as bicycling or climbing.  Sensation is more like being on a boat.
  • 8.
    Symptoms…  Limb Ataxia Clumsinesswith activities such as writing, picking up objects, and buttoning. Movements tend to become slower. These symptoms are one-sided with lateralized lesions of the cerebellum.  Truncal Ataxia Midline cerebellar lesions cause truncal ataxia. Patients may experience head tremor and truncal instability leading to oscillatory movements of the head and trunk while sitting or standing (titubation). They may need back support while sitting.
  • 9.
    Symptoms…  Dysarthria andBulbar Symptoms Ataxic diseases of cerebellar origin result in slurred speech and abnormalities of pitch and volume control (scanning speech).  Dysphagia can result from incoordination of swallowing muscles, and patients report strangling and choking. Ineffectiveness of cough may also be a symptom.
  • 10.
    Symptoms…..  Visual Symptoms Patientsmay experience blurriness or a sense of environmental movements as a result of cerebellar ocular oscillations associated with cerebellar disease.  Symptoms in Sensory Ataxia Patients with a sensory basis for ataxia usually do not experience dysarthria or visual symptoms. They may report other symptoms of sensory pathway disease such as paresthesias and numbness.
  • 12.
    Midline…  Lesion incerebellar vermis.  Causes “truncal ataxia” where the affected person is unable to sit on their bed without steadying themselves.  Often affects eye movements; there may be nystagmus, ocular dysmetria and poor pursuit.
  • 16.
    Differences between cerebellarand sensory ataxia Cerebellar ataxia Sensory ataxia Sensory symptoms absent Present Other cerebellar signs present Absent Objective sensory loss absent Present Broad based, staggering gait High stepping gait Romberg’s sign absent Present Vertigo may be present Absent MRI is the investigation of choice NCS, biochemical parameters and infection screen.
  • 17.
  • 18.
    Cerebellar disorders  Massin the cerebellum/ posterior fossa Gliomas, meningiomas,Medulloblastoma abscess  Abnormal craniovertebral junction Arnold-Chiari malformation,  Infarcts, vascular malformations Ischemic lesions, Haematoma, AVM
  • 19.
    Cerebellar disorders……  Signaldensity change in the cerebellum MS, acute cerebellitis  Cerebellar atrophy - SCAs - idiopathic cortical cerebellar atrophy - drugs and toxin, - Vitamin E deficiency - Autoimmune ataxias - Dandy-Walker syndrome
  • 21.
  • 22.
  • 23.
  • 24.
  • 25.
  • 26.
  • 27.
  • 28.
  • 29.
    • A 51years old diabetic, hypertensive male with sudden onset difficulty walking and tendency to fall to left side 03 days • Intention tremor on left side
  • 32.
  • 33.
    A 40 yrsold Diabetic male presented to an ENT specialist with complaints of ▪vertigo– for 15 days. ▪ discharge from left ear— for3 months. O/E : ▪ Pt is pyretic. ▪ scanning speech. ▪cerebellar ataxia
  • 34.
    Infections affecting cerebellum •Bacterial-cerebellar abscess, tuberculoma •Viral – acute- varicella. chronic- HIV. •Parasite- Toxoplasma, F. malaria, •Prions- CJD, Kuru.
  • 35.
  • 36.
    A 16 yearold boy with Progressive Gait ataxia for 02 years Bilaterally symmetrical intention tremor Ankle reflex lost Plantar bilateral extensor
  • 39.
  • 40.
    • A 30years old lady • Recurrent ataxia and Recurrent visual blurring for 03 episodes in the last 02 years
  • 41.
    • INO • Bilateralplantar extensor
  • 43.
  • 44.
    A 35 yrsold epileptic male pt. on AED for 5 yrs with complaint: - Difficulty in walking – 5 months. - Dysarthria for - 4 months. O/E : - Mildly anaemic, - Scanning speech + - truncal ataxia + Investigation: PBF---- pancytopenia.
  • 45.
    DRUGS & TOXINAFFECTING CEREBELLUM  Alcohol  Anticonvulsant drugs- Phenytoin  Chemotherapy- 5-flurouracil , cytosine arabinose  Heavy metals-mercury , manganese , bismuth  Chronic solvent abuse
  • 46.
    Dandy Walker Malformation Clinicalpresentation: Infancy: symptoms and signs of hydrocephalus with prominent occiput. Childhood: signs of cerebellar dysfunction with or without signs of hydrocephalus. Investigation: CT scan or MRI Management: Cysto peritoneal shunt.
  • 47.
    Arnold-Chiari malformation  Thereis displacement of cerebellar tonsils and vermis through the foramen magnum  There is compression of spino-medullary junction  Often associated with syringomyelia and hydrocephalus
  • 48.
    CHIARI MALFORMATION • TypeI: No meningomyelocoele • Type II: With meningomyeocoele (lumber) • Type III: With occipitocervical meningomyelocoele • Type IV: Only cerebellar hypoplasia • Headache (raised ICP) • Progressive ataxia • Spastic quadriparesis • Lower cranial nerve involvement • Downbeat nystagmus • Features of syringomyelia
  • 49.
  • 50.
    A 10 yearold boy presents with vomiting, unsteady gait, slurred speech, headache and diplopia
  • 52.
    TUMOURS OF CEREBELLUM Cerebellartumors may be – Primary- Common in children secondary- Common in adults Clinical features- -ataxia -brainstem dysfunction -features of raised ICP.
  • 53.
    Primary tumors ofcerebellum Medulloblastoma Astrocytoma Hemangioblastoma Ependymoma Oliogodendroglioma Medulloblastoma, astrocytoma- common in children Hemangioblastoma- common in adults
  • 54.
  • 55.
    • 50 yearsold male patient • Gait ataxia for 08 weeks • Bilaterally symmetrical intention tremor • Smoker for 20 years • Clubbing
  • 56.
    PARANEOPLASTIC CEREBELLAR DEGENERATION Common neoplasmsassociated- SCLC, Ca Breast, Ca Ovary, Hodgkin’s lymphoma Immune associations- Anti-Yo ab Ca breast Anti Ri ab SCLC Anti VGCC SCLC Very few responds to therapy.
  • 57.
    Metabolic disorders affecting cerebellum Hypothyroidism  Hypoxia  Hypoglycemia  Hyponatremia  Deficiency disorders (vitamin B1, B12, E)  Inborn errors of metabolism( lipid /amino acid metabolism)