Basal Nuclei

1. Basal Nuclei A 55 yr old male is brought in to the doctor by his wife. She says that he has been irritable for the past three months and is acting strange. She also claims and he supports that in the past two weeks he has been fidgety with his hands and now he is demonstrating some strange facial tics. The historical exam yields little information as this patient was an orphan and has no parental or family history. The physical exam showed normal reflexes and sensation, normal strength and no ataxia. There appeared to be mild hypotonia, but this was subjective. The one constant finding was an apparent restlessness during the exam and constant movements of the face. He was given a simple test of memory and attention where he performed below average, and according to his wife way below what she knew he could do. During the test he was irritable and had one outburst over a seemingly trivial event. Select the true statement concerning this patient. • he is in the early stages of Parkinson’s disease and his restless hands are the early signs of a resting tremor • In the advanced stage of this disease he will have degeneration in the caudate nucleus • he probably has a loss of dopamine producing cells in the substantia nigra • his condition will improve with the administration of L-Dopa • all are true

2. Basal Nuclei

3. Basal Nuclei Dornella is a 60 year old female who presents to the office with difficulty using her right arm.Ms. Dornella states that the difficulty in using her right arm has occurred over the past 2 months. She is right-handed. The clumsiness and intermittent tremor have become progressively worse. No other symptoms are present. To direct questioning she says the right shoulder aches, “it may be bursitis”, and that she has noted saliva on her pillow in the mornings. Her children feel she is slower and looks depressed. She denies any visual disturbances, vertigo, palpitations, sensory symptoms or unconsciousness. She denies headaches and there is no history of trauma. Her lab results are normal, her physical exam demonstrates some hypertonia, and a paucity of movements. She is slow to start, but has no retropulsion when pulled from behind. Select the correct statement concerning this patient. • she probably has degeneration in her striatum. • her decrease in movements can be improved by the administration of Ach • she is in the early stages of Huntington’s disease. • her condition could be improved by the administration of L-Dopa • all are correct

5. Dopamine and Ach ..nergic Effect

6. Parkinson’s Disease PD Disease of mesostriatal dopaminergic system normal Muhammad Ali in Alanta Olympic

7. Parkinson’s Disease - Paralysis Agitans Substantia Nigra, Pars Compacta (SNc) DOPAminergic Neuron Clinical Feature (1) Slowness of Movement - Difficulty in Initiation and Cessation of Movement

8. Parkinson’s Disease Paralysis Agitans Clinical Feature (2) Resting Tremor Parkinsonian Posture Rigidity-Cogwheel Rigidity Masked Face

9. Basal Nuclei A vascular occlusion of a region of the brain damaged the overwhelming numbers of fibers that supply the basal ganglia. Which of the following regions constitute the receiving area of the overwhelming majority of fibers that supply the basal ganglia? • Paleostriatum • Neostriatum • Subthalamic nucleus • Substantia nigra • Claustrum

10. Basal Nuclei cont… A 43-year-old male who began to display marked involuntary movements at times of rest was seen by a neurologist, who concluded that he was suffering from Huntington’s disease. Which of the following neurotransmitters was lost or reduced in this individual? • Dopamine in the neostriatum • Substance P in the substantia nigra • ACh and GABA in intrastriatal and cortical neurons • Serotonin in the neostriatum • Histamine in subthalamic nucleus

11. Sam is a 62-year-old man, previously healthy, who was brought to a neurologist by his daughter because of increasing difficulty in walking. His daughter noticed that for the past year, he had difficulty getting out of a chair and took a lot of time to begin to walk. When he did walk, he walked with a slow, shuffling gait. In addition, she had noticed some changes in his face, and that he had been drooling excessively. His signature on checks became progressively smaller from the beginning of his name to the end, and he had developed a new tremor. She brought him in to make sure this wasn’t just “aging.” The neurologist examined Sam and noticed immediately that Sam’s facial expression was masklike, with few eye blinks. When asked to write a sentence, the letters became progressively smaller toward the end of the sentence

12. His speech was soft and monotonous, and he had a slow, resting pill-rolling tremor in both of his hands. He had very little spontaneous movement, and his arms, legs, and trunk were stiff. When the neurologist tried to flex his arm, he felt many catches, similar to a cogwheel. There was no weakness, sensory problems, or abnormalities in his reflexes. When asked to walk, Sam took many tries to rise from his chair. When he finally stood up, his posture was stooped and flexed. His gait was slow, his feet shuffled when he walked, and his arms didn’t swing with his steps. The neurologist told Sam’s daughter that she was correct that this wasn’t aging and explained to her all of the details about a new medication that Sam needed to take.

13. Damage to which of the following structures causes Sam’s problem with movement? • Substantia gelatinosa • Substantia nigra, pars reticularis • Substantia nigra, pars compacta • Caudate nucleus • Thalamus

14. SYDENHAM’S CHOREA Clinical Feature - Complication of Rheumatic Fever - Fine, disorganized , and random movements of extremities, face and tongue - Accompanied by Muscular Hypotonia - Typical exaggeration of associated movements during voluntary activity - Usually recovers spontaneously in 1 to 4 months Principal Pathologic Lesion: Corpus Striatum

15. HUNTINGTON’S CHOREA Clinical Feature - Predominantly autosomal dominantly inherited chronic fatal disease (Gene: chromosome 4) - Insidious onset: Usually 30-50 - Choreic movements in onset - Frequently associated with emotional disturbances - Ultimately, grotesque gait and sever dysarthria, progressive dementia ensues. Principal Pathologic Lesion: Corpus Striatum (esp. caudate nucleus) and Cerebral Cortex

16. HEMIBALLISM Clinical Feature - Usually results from CVA (Cerebrovascular Accident) involving subthalamic nucleus - sudden onset - Violent, writhing, involuntary movements of wide excursion confined to one half of the body - The movements are continuous and often exhausting but cease during sleep - Sometimes fatal due to exhaustion - Could be controlled by phenothiazines and stereotaxic surgery Lesion: Subthalamic Nucleus

17. Basal Nuclei • A vascular occlusion of a region of the brain damaged the overwhelming numbers of fibers that supply the basal ganglia. Which of the following regions constitute the receiving area of the overwhelming majority of fibers that supply the basal ganglia? • Paleostriatum • Neostriatum • Subthalamic nucleus • Substantia nigra • Claustrum

18. Basal Nuclei cont… • A 43-year-old male who began to display marked involuntary movements at times of rest was seen by a neurologist, who concluded that he was suffering from Huntington’s disease. Which of the following neurotransmitters was lost or reduced in this individual? • Dopamine in the neostriatum • Substance P in the substantia nigra • ACh and GABA in intrastriatal and cortical neurons • Serotonin in the neostriatum • Histamine in subthalamic nucleus

19. A 45 year-old basket ball player noticed the abrupt onset of involuntary movements on his left side. His left arm would make large, violent, flailing or ballistic movements that interfered with his game. The most likely site of the damage was the: • left substantia nigra • left globus pallidus • right globus pallidus • left subthalamic nucleus • right subthalamic nucleus

20. A 63 year-old patient is seen in a clinic with the following symptoms: a resting tremor in the Hand, a slow shuffling gait, difficulty arising from a chair when seated. These symptoms could be caused by a lesion in the: • Subthalamic nucleus • cerebellar hemisphere • Corticospinal tract • Substantia nigra