Basal Ganglia module

1. Basal Ganglia module (next slide)

2. Basal Ganglia menu Basal Ganglia Overview of Basal Ganglia structure Basal Ganglia circuitry (the Striato-Pallido-Thalamic loop) Modulation of inputs to the Basal Ganglia Modulation of outputs from the Basal Ganglia Summary of extrapyramidal circuitry Clinical signs related to the Basal Ganglia Effects of drugs on the Nervous System Select a blue button to move to that topic (previous slide) (next slide) (select this button to return to this slide)

3. Basal Ganglia Overview of Basal Ganglia structure Basal Ganglia circuitry (the Striato-Pallido-Thalamic loop) Modulation of inputs to the Basal Ganglia Modulation of outputs from the Basal Ganglia Summary of extrapyramidal circuitry Clinical signs related to the Basal Ganglia Effects of drugs on the Nervous System (next)

4. Overview of the Basal Ganglia overview Extrapyramidal Motor System The basal ganglia perform two functions: • Initiate movements • Regulate stereotypic movements Select a button to view that example #1 #2a #2b

5. Initiate movements Play Movie The basal ganglia govern the initiation changes in motor behavior - meaning both starting AND stopping of motor actions. Sipping from a cup requires proper initiation or stopping of multiple, sequential actions. (return…)

6. Stereotypic movements #1 Play Movie Stereotyped movements, like other habits, often are context dependent. The cerebellum governs the movement itself, but the initiation of the motor pattern in context is governed by the basal ganglia. The basal ganglia have been implicated in the expression of obsessive-compulsive disorders. (return…)

7. Stereotypic movements #2 Play Movie Not all stereotyped behaviors are rhythmic, and the motor pattern can be difficult to halt once it has begun. Have you ever tried to stifle a yawn? (return…)

8. Basal ganglia structures This cartoon represents a horizontal slice through the brain at the level of the thalamus. It is a midline view from above, with anterior at the top of the screen and posterior at the bottom of the screen.

11. Basal Ganglia menu

12. Basal Ganglia Overview of Basal Ganglia structure Basal Ganglia circuitry (the Striato-Pallido-Thalamic loop) Modulation of inputs to the Basal Ganglia Modulation of outputs from the Basal Ganglia Summary of extrapyramidal circuitry Clinical signs related to the Basal Ganglia Effects of drugs on the Nervous System (next)

13. Neural circuits of the Basal Ganglia circuitry The basal ganglia form an internal motor circuit … Cortex Caudate Putamen

14. The cortex receives motor planning information, then passes that information to the caudate & putamen, which govern timing of events

15. The information then is passed to the globus pallidus …

16. ... which helps govern movement magnitude, and then passes this basal ganglia output to thalamus nuclei

17. The thalamus combines information from the basal ganglia and the cerebellum, then sends it to the motor cortex Basal Ganglia menu

18. Basal Ganglia Overview of Basal Ganglia structure Basal Ganglia circuitry (the Striato-Pallido-Thalamic loop) Modulation of inputs to the Basal Ganglia Modulation of outputs from the Basal Ganglia Summary of extrapyramidal circuitry Clinical signs related to the Basal Ganglia Effects of drugs on the Nervous System (next)

19. Modulation of input to the Basal Ganglia The caudate & putamen receive input from the cortex, and … Input modulation

20. … from the thalamus.

21. The substantia nigra also modulates input to the basal ganglia.

22. Reciprocal connections with the caudate & putamen allow exitatory inputs from the substantia nigra to modulate the amount and type of output sent to the globus pallidus. Dopamine is the neurotransmitter used by these substantia nigra pathways.

23. When the substantia nigra isn’t working properly, input to the basal ganglia isn’t modulated properly, and the globus pallidus receive progressvely less information. Without this information, the initiation of movement (i.e., timing) message is less effective and the person’s movements progressively become slower (i.e., bradykinesia).

24. Parkinson’s disease is related to a deterioration of the substantia nigra and globus pallidus, and is characterized by resting tremors and bradykinesia. Basal Ganglia menu

25. Basal Ganglia Overview of Basal Ganglia structure Basal Ganglia circuitry (the Striato-Pallido-Thalamic loop) Modulation of inputs to the Basal Ganglia Modulation of outputs from the Basal Ganglia Summary of extrapyramidal circuitry Clinical signs related to the Basal Ganglia Effects of drugs on the Nervous System (next)

26. Modulation of output from the Basal Ganglia Output modulation - part 1 1) The putamen provides processed information to the globus pallidus.

27. - part 1 In addition to modulating input to the basal ganglia, the substantia nigra also modulates the output.

28. - part 1 The substantia nigra, in turn, has many connections.

29. - part 2 2) The subthalamus plays a role in modulating output from the basal ganglia

30. - part 2 Deterioration of the subthalamus results in the ballisms, or explosive movements occurring periodically, that characterize Huntington’s disease. Basal Ganglia menu

31. Basal Ganglia Overview of Basal Ganglia structure Basal Ganglia circuitry (the Striato-Pallido-Thalamic loop) Modulation of inputs to the Basal Ganglia Modulation of outputs from the Basal Ganglia Summary of extrapyramidal circuitry Clinical signs related to the Basal Ganglia Effects of drugs on the Nervous System (next)

32. Summary Basal Ganglia menu

33. Basal Ganglia Overview of Basal Ganglia structure Basal Ganglia circuitry (the Striato-Pallido-Thalamic loop) Modulation of inputs to the Basal Ganglia Modulation of outputs from the Basal Ganglia Summary of extrapyramidal circuitry Clinical signs related to the Basal Ganglia Effects of drugs on the Nervous System (next)

34. Clinical signs related to Basal Ganglia dysfunction Basal Ganglia clinical signs Parkinson’s disease resting tremor . . .. . . . . . . poverty of movement . . .. . . bradykinesia . . . . . . . . . . . stooped posture . .. . . . . . . cogwheel rigidity . .. . . . . . . masked face . . . .. . . . . . . Chorea . . . . . . . . . . . Choreaform movements . . Tics . . . . . . . . . . . . . Ballisms . . . . . . . . . . . #1 #2 Select a blue button to view the video #3 #4 #5 #6 #7 #8 #9 #10a #10b (YouTube’s website also can be a good source of example videos…) Basal Ganglia menu

35. Resting tremor An hallmark feature of Parkinson’s disease is the resting tremor - involuntary, oscillating movements at rest, often of the hands or head and neck. It is not a resting tremor if it occurs during execution of a movement! Play Movie Clinical signs menu

36. Poverty of movement Limited or no movement - often described by the person as becoming “stuck” in position - also is characteristic of Parkinson’s disease. Remember that the basal ganglia are responsible for initiating a change in movement patterns. Here, the individual is attempting to rise from a chair - a complex motor sequence where timing of the component movements is critical. Play Movie Clinical signs menu

37. Bradykinesia “Slowed movement” is another feature of Parkinson’s disease, and is particularly noticeable with walking - a motor behavior where the center of gravity shifts forward, and placing each foot forward alternately is what keeps you from falling. Slowed movements allows the center of gravity to shift too far forward, too rapidly. Play Movie Clinical signs menu

38. Stooped posture A biomechanical feature of many disorders, but characteristic of Parkinson’s disease when seen in combination with other symptoms. Here, note the resting tremor, bradykinesia, and - despite the poor quality image - a masked facial expression. Play Movie Clinical signs menu

39. Cogwheel rigidity An animation of the concept, meant to illustrate the lack of smooth movements at the joint. The feeling is similar to that of a cogwheel: “click - stop - click - stop...” Play Movie Clinical signs menu

40. Masked facial expression “The Phantom of the Opera” Warner Brothers, 2004 Gerard Butler as the Phantom Conveying emotion through facial expression requires initiation of motor behavior. Without these behaviors, the person may have a “masked” facial expression. Be aware, however, that emotional capacity is undiminished even if the emotion can’t be expressed! Clinical signs menu

41. Chorea Huntington’s disease and other choreas typically are disorders in modulation of the output from the basal ganglia. Ongoing, smoothly flowing movements are typical - but the constant movement consumes calories rapidly. The movements do not occur during sleep. A different form of chorea (now called Sydenham’s chorea) was once termed St. Vitus’ dance due to the frenzied limb movements and contorted facial expressions. This form of chorea can appear after rheumatic fever, but also may co-occur with obsessive-compulsive disorder - which also is associated with basal ganglia dysfunction. Play Movie Clinical signs menu

42. Choreaform movements Other intrusive movements may appear similar to those of Huntington’s chorea and Sydenham’s chorea, but do not arise from the same causes (genetic source or bacterial infection). These movements are termed “choreaform” and they can be significant barrier to function. This individual has been asked to touch her nose with each index. Unlike true choreas, her impairment primarily is unilateral (right side). Play Movie Clinical signs menu

43. Tics A tic is a sudden, repetitive, stereotyped, nonrhythmic, involuntary movement (motor tic) or sound (phonic tic) that involves discrete groups of muscles. Tics can be invisible to the observer - such as abdominal tensing or toe crunching. Tics must be distinguished from symptoms of other disorders, such as choreas, autism, seizures, or obsessive-compulsive disorder. Play Movie Clinical signs menu

44. Ballisms #1 Play Movie Ballisms are sudden, explosive, large-amplitude, flinging motions of the limbs. If confined to one limb or one side of the body, they are termed “hemiballisms.” This motor intrusion may cause postural instability and lead to falls and cause problems with everyday activities (such as eating, cooking, or dressing), but frequently also is a significant social barrier. Clinical signs menu

45. Ballisms #2 Play Movie Caused by a lesion of the contralateral subthalamic nucleus or its connections, ballisms may be less severe, more severe, or may progressively become worse over time. The severity may relate to the size or to the frequency of the ballism. This individual has been asked to touch his nose with his left index finger. Clinical signs menu

46. Basal Ganglia Overview of Basal Ganglia structure Basal Ganglia circuitry (the Striato-Pallido-Thalamic loop) Modulation of inputs to the Basal Ganglia Modulation of outputs from the Basal Ganglia Summary of extrapyramidal circuitry Clinical signs related to the Basal Ganglia Effects of drugs on the Nervous System (next)

47. Drug effects Effects of Drugs on the nervous system Click on a button to view the video Tardive dyskinesia - a side effect of many anti-psychotic drugs L-dopa therapy - side effects related to treatment of Parkinson’s disease Catatonic rigidity - side effect of Phenothiazine, an anti-psychotic medication video #1 Drug side effects may occur when a drug is introduced into a system that already is malfunctioning, or when the drug acts on brain regions other than just the intended target area. video #2 video #3 Basal Ganglia menu

48. Tardive dyskinesia Tardive dyskinesia Play Movie Tardive dyskinesia occurs as a result of long-term use of certain anti-psychotic medications, such as those used in the treatment of schizophrenia. Characterized by periodic involuntary movements (tongue & mouth movements in particular), tardive dyskinesia is addressed by halting the use of that particular drug - but the damage is done, and the presence of these intrusive movements cannot be reversed. Drug Effects menu

49. L-dopa therapy L-dopa Play Movie L-dopa is used to treat Parkinson’s disease. While very effective in the earlier stages of the disorder, symptoms continue to develop and larger doses of drug are required. Eventually the side effects if high doses of L-dopa will impair function and diminish quality of life. This is an example of a drug regime that allows the symptoms of a disorder to be managed - at least for a time - but is NOT a cure for the disorder. This example also shows three stages of drug action - prior to treatment (poverty of movement; resting tremor; stooped posture; shuffling gait), soon after administration (intrusive movements), and later on - during the therapeutic phase of the drug’s action. Drug Effects menu

50. Catatonic rigidity Play Movie Catatonic rigidity occurs as a side effect after long-term use of certain anti-seizure medications. Use of modern medications has limited the incidence of this iatrogenic disorder. Drug Effects menu