Case 181 Answers

Basic Human Neuroanatomy

A Clinically Oriented Atlas

Case 181 Answers

1. Considering the patient’s history and examination, how precisely can the neurologic lesion be localized in this case?

Not precisely. Multifocal process.

2. This patient’s signs and symptoms constitute a classical neurologic syndrome. What is the name of that syndrome? This syndrome is one of a few other syndromes, all of which are included in a larger group of syndromes. What is the name of that group of syndromes?

Progressive supranuclear palsy (PSP). Parkinsons plus syndromes.

3. Indicate the level of the neurologic lesion in this case as precisely as possible and the structures involved by the pathologic process. If precise localization is not possible, list as many levels or structures as you can that might produce the patient’s clinical signs and symptoms.

Neuronal loss and gliosis (with neurofibrillary tangles and tau deposition [a 4-repeat tauopathy]) involving the cerebral cortex; the PAG, substantia nigra, red nuclei, pretectal area, and superior colliculi of the midbrain; the globus pallidus; subthalamic nuclei; and dentate nuclei of the cerebellum.

4. This patient’s sudden outbursts of laughter are characteristic of a classic neurologic symptom complex. What is the name of that symptom complex? What does it usually signify? In this patient, what other examination findings are indicative of this symptom complex?

Pseudobulbar palsy. Bilateral frontal lobe or UMN lesions. Positive glabellar tap, snout, left palmomental, and grasp reflexes.

5. In general, what type of pathologic process do you think is involved in this case?

Degenerative. A 4-repeat tauopathy. Multifocal in nature (as mentioned above).

6. What diagnostic procedure(s) would you undertake at this point?

References:
1. Steele JC, Richardson JC, Olszewski J. Arch Neurol 1964;10:333-359.
2. Hauw J-J, Daniel SE, Dickson D, et al. Neurology 1994;44:2015-2019.
3. Williams DR, Lees AJ, Wherrett JR, Steele JC. Neurology 2008;70:566-573.
4. Dickson DW, Ahmed Z, Algom AA, et al. Curr Opin Neurol 2010;23:394-400.
5. Gardner RC, Boxer AL, Trujillo A, et al. Ann Neurol 2013;73:603-616.

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