Cerebellum Flashcards
What are the three major functional divisions of the cerebellum?
“Vestibulocerebellum
What deep cerebellar nucleus is the largest and involved in fine motor planning?
“Dentate nucleus”
What artery supplies the inferior aspect of the cerebellum and is commonly affected in lateral medullary syndrome?
“Posterior inferior cerebellar artery (PICA)”
What is the primary neurotransmitter released by Purkinje cells in the cerebellum?
“GABA”
What inherited ataxia presents with cardiomyopathy, scoliosis, and neuropathy?
“Friedreich ataxia”
What pediatric syndrome is associated with cerebellar atrophy, oculocutaneous telangiectasias, and immunodeficiency?
“Ataxia-Telangiectasia”
What genetic disorder is characterized by the ‘molar tooth sign’ on MRI and presents with ataxia and oculomotor apraxia?
“Joubert syndrome”
What condition presents with truncal ataxia, macrocephaly, and a hypoplastic cerebellar vermis on MRI?
“Dandy-Walker malformation”
What childhood brain tumor commonly arises in the midline cerebellum and presents with ataxia and increased ICP?
“Medulloblastoma”
What acquired cause of pediatric ataxia typically follows a viral infection and resolves spontaneously?
“Acute post-infectious cerebellitis”
What metabolic disorder presents with ataxia, dystonia, and low ceruloplasmin levels?
“Wilson disease”
What is the most common presentation of spinocerebellar ataxias (SCAs)?
“Progressive ataxia with dysarthria and coordination difficulties”
What examination finding distinguishes cerebellar ataxia from sensory ataxia?
“Cerebellar ataxia persists with eyes open or closed; sensory ataxia worsens with eyes closed (positive Romberg sign)”
What is the most sensitive imaging modality for cerebellar disorders?
“MRI Brain”
What genetic test should be ordered for a child with progressive ataxia and cardiomyopathy?
“Friedreich ataxia genetic testing (FXN gene analysis)”
What is the mainstay of treatment for Wilson disease?
“Copper chelators (penicillamine
What cerebellar disorder is associated with episodic ataxia triggered by stress or exertion?
“Episodic ataxia (EA1 or EA2)”
What is the term for the inability to perform rapid alternating movements?
“Dysdiadochokinesia”
What acquired condition is a common cause of cerebellar stroke in children?
“Moya Moya disease”
What condition is suggested by progressive ataxia, ophthalmoplegia, and high serum lactate?
“Mitochondrial disease (e.g.
What type of tremor is most commonly associated with cerebellar dysfunction?
“Intention tremor”
What is the first-line treatment for autoimmune cerebellitis?
“Corticosteroids (followed by IVIG or plasmapheresis if needed)”
What laboratory test helps distinguish Wilson disease from other causes of pediatric ataxia?
“Serum ceruloplasmin and 24-hour urinary copper”
What differentiates medulloblastoma from pilocytic astrocytoma on MRI?
“Medulloblastoma is midline and hyperdense; Pilocytic astrocytoma is cystic with an enhancing mural nodule”
