Abstract
The chapter begins with discussion of anatomy and functions of the brainstem (mesencephalon, pons, and medulla) and cerebellum. No other part of the central nervous system is packed with so many critical axon tracts and nuclei. Main brainstem functions are as follows: to provide transit and processing nuclei for ascending and descending pathways that convey signals to and from the cerebellum, cerebrum, and spinal cord; to integrate functions such as consciousness, muscle tone, posture, and normal autonomic functions; and to house cranial nerves 3–10. The cerebellum occupies only 10 % of the brain volume but contains more neurons than the entire rest of the brain. Lesions in the brainstem often manifest as cerebellar, somatosensory and motor symptoms plus cranial nerve dysfunction. Damage to the cerebellum often results in errors in smooth motor movements. The chapter ends with a discussion of central pontine myelinolysis, lateral medullary syndrome or Wallenberg’s syndrome, and spinocerebellar atrophies with attention to their pathophysiology, major clinical features, major laboratory findings, and principles of management and prognosis.
A 44-year-old woman with a history of alcoholism is brought to the emergency room by her husband. He reports that over the past 2 days, she has been confused and sleepy. Her examination showed jaundice and lethargy. On laboratory examination, her serum sodium was found to be 124 mmol/L. Correction of her serum sodium commenced but her mental status worsened requiring admission to the intensive care unit. She developed spastic quadriparesis and had only limited ability to move her eyes up and down. MRI of the brain revealed hyperintensity in the pons suggestive of central pontine myelinolysis. She was supported with mechanical ventilation and nutrition. Despite this support, she passed away within a month of her initial presentation.
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Recommended Reading
Kandel ER, Schwartz JH, Jessell JM. Principles of neural science, 5th edn. New York: McGraw-Hill Professional; 2012. (Good review of brainstem and cerebellar anatomy and physiology)
Querol-Pascual M. Clinical approach to brainstem lesions. Semin Ultrasound CT MRI. 2010;31:220–9. (Excellent brief review of brainstem locations for common brainstem clinical signs and symptoms)
Hurley RA, Filley CM, Taber KH. Central pontine myelinolysis: a metabolic disorder of myelin. J Neuropsychiatry Clin Neurosci. 2011;23(4):369–74. (Good review of the clinical manifestations and pathophysiology of CPM)
Kameda W, et al. Lateral and medial medullary infarction: a comparative analysis of 214 patients. Stroke. 2004;35:694–9. Comprehensive review of the features of medial vs. lateral medullary infarction with good anatomy-clinical correlations)
Durr A. Autosomal dominant cerebellar ataxias: polyglutamine expansions and beyond. Lancet Neurol. 2010;9:885–94. (Excellent review of clinical, pathological and genetic features of major SCAs)
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This video shows a 67 year-old woman with Multiple System Atrophy-Type Cerebellar
Segment 1: Cranial Nerve Exam
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Saccadic intrusions into fixation
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Dysmetric and slow saccades
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Impaired vestibulo-ocular reflex
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Scanning dysarthria
Segment 2: Cerebellar Exam
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Dysmetria on finger-to-nose
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Overshooting target on finger chase
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Dysmetria on heel-to-shin
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Limb rebound
Segment 3: Gait Exam
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Ataxic gait requiring use of walker
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Davis, L., Pirio Richardson, S. (2015). Disorders of the Brainstem and Cerebellum. In: Fundamentals of Neurologic Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-2359-5_8
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DOI: https://doi.org/10.1007/978-1-4939-2359-5_8
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Online ISBN: 978-1-4939-2359-5
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