Clinical, pathological and radiology behind ataxia to diagnose

1. APPROACH TO ATAXIA
PRESENTER:- Dr VISHAL SINGH
DR RML HOSPITAL AND ABVIMS

2. Introduction
• Ataxia stands for lack of order.
• Used to describe disturbance in coordination
necessary to perform willed motor activites.

3. Types of Ataxia
1. Frontal ataxia/apraxia:
Difficulty in walking due to frontal lobe lesions; associated
with headaches and/or convulsions.
2. Paretic or limb ataxia:
Focal limb weakness in children leading to difficulty in
walking.
3. Truncal ataxia:
Cerebellar/systemic causes of ataxia.
4. Sensory ataxia:
Sensory system abnormality leading to difficulty in walking,
characterized by a high-steppage gait and diurnal variation
with worsening in the dark.

4. • A detailed history and meticulous exami-
nation is paramount for clinical localization of
the disease.
• The diagnosis of ataxia can be especially
challenging in the pediatric population.

5. HISTORY
causes of sudden onset of ataxia
• A preceding history of trauma, concussion,
posterior circulation traumatic thrombo-
embolism or dissection
• A history of toxin ingestion might aid in deciding
the necessary toxin screening to be performed.
• a history of infection suggests the presence of
post-infectious cerebellar ataxia or acute
labyrinthitis.

6. AGE OF ONSET
• An infant with jerky eye movements may be
suffering from opsoclonus- myoclonus
syndrome and may need a detailed evaluation
to rule out the presence of a neuroblastoma.
• Hypotonia, truncal ataxia, and titubation in
infants may be suggestive of a cerebellar
mass, and require neuroimaging.

7. Time of Onset
• Can be acute, subacute, and chronic.
• Acute ataxic disorder starts within 72 hours of
presentation.
• Subacute ataxia is usually noticed in the
presence of a progressive intracranial etiology.
• Chronic ataxia is rare in children.

8. Acute <72 hrs Subacute Chronic
Neuroblastoma
(opsoclonus myoclonus
syndrome)-jerky eye
movements
Intracranial tumors,
abscess-associated
headache, or presence of
fever
Cerebellar malformations,
degenerative diseases,
metabolic diseases-a
detailed elective workup.
including neuroimaging,
CSF and blood tests, and
sometimes, genetic
counselling especially in
cases of autosomal
dominant disorders may be
required

9. • Any diurnal variation in ataxia, esp. its worsening
in the dark, is highly suggestive of sensory ataxia
due to impaired sensory input.
• Inborn errors of metabolism, hereditary ataxias,
and cerebellar malformations manifest as
episodic ataxia.
• A positive family history may also be present in
such conditions
• CSF studies can be performed prior to
neuroimaging if the findings strongly suggest
presence of peripheral neuropathy.

10. Ataxia with Vomiting
• In toddlers and adolescents, acute causes of ataxia with vomiting
are more common.
• Acute causes of ataxia are as follows:
1. Toxin ingestion: Based on history, a specific toxin screening needs
to be performed and treated accordingly.
2. Guillain-Barré syndrome: A history of progressive weakness,
previous infection and a detailed neurological examination may
clinch this diagno- sis. CSF studies might aid in diagnosis.
3. Acute labyrinthitis: History of severe vertigo, infection, hearing
loss and nystagmus with a normal neuroim- aging makes one
suspect this condi- tion. A positive response to vestibular
rehabilitation exercises is also a diag- nostic indicator.

11. 4. Intracranial lesion:
Usually presents with a history of early morning
headache and projectile vomiting with or without
neurological deficit.
Fever is suggestive of an infective pathology like an
abscess.
H/O ear infection, contact with a patient having
tuberculosis, or cyanotic heart disease must be
obtained.
Asymmetric pupils, drowsiness, convulsions, and focal
neurological deficits demand an urgent neurosurgical
attention.

12. 5. Post-infectious cerebellar ataxia (acute cerebellitis):
H/o viral infection is suggestive; otherwise it is a diagnosis of exclusion. CSF
studies might help.
6. Stroke, thromboembolism:
Present as sudden onset of weakness (paretic ataxia). Ataxia can be an
initial presentation of the moyamoya disease. CT scan of the brain and
diffusion-weighted MR sequences are generally necessary.
7. Concussion, vascular dissection: History of trauma is usually present.
8. Basilar migraine:
Generally benign, it is a diagnosis of exclusion based on the location of
headache and episodic nature of symptoms.
9. The most common tumors presenting with ataxia and vomiting in children
are pilocytic astrocytoma, medullo- blastoma, and ependymoma

13. Associated Symptoms
• Patients with frontal ataxia or apraxia associated
with headache or convulsions are likely to have
frontal lobe lesions,
whereas those with sensory ataxia have a high
step- page, stomping gait.
• Patients with cerebellar lesions tend to sway
toward the same side of the lesion.
• Projectile vomiting along with early morning
headaches is s/o an intracranial mass lesion,
whereas h/o fever indicates an infective etiology.

14. Clinical Examination
• Detailed neurological examination is challenging
in the pediatric population.
• It is important to observe how a child reaches out
for toys, drinks from a glass, stands, walks, and
changes postures.
• Opsoclonus, papilledema, and signs suggestive of
neuropathy should be sought.
• Involvement of the cerebellar peduncle would
result in brain stem signs such as focal limb
weakness and cranial nerve palsies.
• Romberg's test is positive in sensory ataxia.

15. Gait and Station Assessment
• Gait and station assessment, using tandem.
walking, test equilibratory coordination,
• Coordination tests are abnormal with eyes
closed in sensory ataxia.

16. Test used for assessment of
nonequilibratory coordination.
Test Inference
Heel knee shin test Coordination in lower limb muscles
Finger – nose – finger test Tremors and upper limb eye coordination
can be tested.
Dysmetria ( past pointing ) can be tested.
Rapid alternating movements Agonist-antagonist coordination and
dysdiadochokinesia can be tested
Holmes rebound test and impaired chek Agonist-antagonist coordination and past
pointing can be tested.

17. Cerebellar Signs
• Cerebellar signs include;-
dysarthria,
asthenia,
pendular reflexes,
impaired coordination tests,
titubation, and
truncal ataxia..

19. INVESTIGATIONS : Neuroimaging
• necessary in almost every case, the only
exception being in a patient with a definite
history of toxin ingestion.
• Relevant neuroimaging is required in patients
who need urgent neurosurgical intervention.
Though MRI is IOC for most of these
conditions, a CT scan is preferred in an
emergency situation because it is quicker and
readily available.

20. PILOCYTIC ASTROCYTOMA

21. MEDULLOBLASTOMA

23. DIFFUSE PONTINE GLIOMA

24. PONTINE GLIOMA

25. TUBERCULOMA

26. Other Neurosurgical Causes of Ataxia
with Vomiting
1. Epidermoid/dermoid cysts.
2. Supratentorial ependymoma.
3. Supratentorial primitive neuroecto- dermal tumor (PNET).
4. Pineal gland lesions.
5. Brain stem glioma.
6. benign intracranial hypertension.
7. Phenytoin toxicity.
8. Hypothalamic glioma.
9. Pyogenic abscess.
10. Arteriovenous malformations.
11. Vein of Galen malformation.
12. Subdural empyema.
13. Child abuse leading to bilateral sub-dural hemorrhage.
14. Opportunistic infections in immuno-compromised situations.

28. THANK YOU