A 62 year old man sudden developed an ataxic gait, and he tended to fall to the right. His right arm and leg became clumsy and he became hoarse. When he was asked to stand with his feet together, he tended to fall to the right, whether his eyes were open or closed. The right eyelid drooped, and the right pupil was distinctly smaller than the left. He did not sweat on the right side of his face. Pain and temperature sensations were decreased on the right side of the face and over the left side of the body and left limbs. A) where in the central nervous system was the lesion? What was the cause of the somatic sensory deficits? B) what was the cause of the autonomic changed in the right eye and face? C) what was the mother disorder affecting gait? Why did he become hoarse?

OneClass: A 62 year old man sudden developed an ataxic gait, and he tended to fall to the right. His ...

Mr G, a 65 year old right handed male, was found sprawled on the floor by his wife. When he woke, he appeared slightly confused and said something about everything looking blurry but couldnât explain it well. He vaguely complained of a headache and a strange numbness in his right arm but his wife couldnât make much sense of what he was trying to say. Mrs G said her husband was talking rapidly, but his words did not make any sense. She described it as if he was speaking meaningless words and irrelevant phrases. She was quite upset because it appeared that Mr G didnât understand anything said to him and yet he seemed completely unaware of his condition. Mrs G noted that he was dragging his right arm and immediately called for an ambulance.
Mr G has been a chronic smoker for the last 40 years and has a history of type 2 diabetes.
Physical examination:
Vital signs: Temperature 38 ËC. Pulse=80. Blood pressure=150/100mmHg
BMI=32
Head: Minor abrasions and mild bruising
Heart: Regular rate, no murmurs
Lungs: Clear
Abdomen: Soft, normal bowel sound
Neurologic Examination:
Mental status: Alert. Mildly agitated. Speech fluent, but with meaningless words and phrases. Unable to repeat words correctly. Spontaneous speech filled with made up words and jargon. Minor impairment in reading and writing.
Cranial nerves: Pupils equal, round and reactive to light. Extraocular movements were intact. Visual field testing showed difficulty in seeing objects on patientâs right side. Facial sensation intact to light touch and pinprick. Smile was symmetrical on both sides. No dysarthria.
BMSC12007
Motor: Slightly increased tone in the right arm. Normal tone on the left side. Moved all four limbs, but was unable to steadily hold right arm over head.
Reflexes: Mildly increased reflexes in the right arm.
Coordination: Normal finger-to-nose and heel-to-shin test.
Gait: Patient took small steps hesitatingly, was able to walk without support.
Sensory: Temperature and light touch sensation intact on both sides. Decreased pinprick sensation on the right side. Graphesthesia and stereognosis normal in the left hand, absent in the right hand.

(Given the signs and symptoms above it strong suggest transient ischemic attack (TIA))

TASK: Discuss a most likely diagnosis for Mr Gâs condition (TIA) by discussing risk factors and underlying pathophysiology. What can be other possible differential diagnosis? Support your answer with reasoning.

To investigate Neilâs symptoms further, doctors referred him to a neurologist, Dr. Bonasera, that specialized in motor disorders. Dr. Bonasera noted that Neil exhibited diminished right dorsalis pedis and posterior tibial pulses. His cognitive tests were normal. Tests of the cranial nerves were normal, with the exception of occasional slurred speech. His motor tests revealed generally slowed movements. Neil was experiencing pain and stiffness in the shoulder joints, which could explain his restricted range motion. Dr. Bonasera tested the motor strength of his finger extensors (4+/5), hip flexors (4/5), and various muscle groups in his arms and legs (5/5). There were tests for dysmetria (i.e., lack of coordination) and dexterity, which were normal yet slowed. Neil did have difficulty lifting his legs for some of the examinations, and he walked with a shuffling gate, often keeping both feet close together for a narrow base of support. He took very small steps and often stooped over while walking. When placing one foot in front of the other in a tandem walking stance, he would fall to either side and was unable to regain his balance. Sensory examination was normal, deep tendon reflexes were normal, and the Babinski sign was absent bilaterally. There were no Parkinsons-like symptoms, increased motor tone, or muscle fasciculations observed in testing.

Just one day later, another neurologist noted brisk reflexes and bilateral Babinski signs. Doctors performed magnetic resonance imaging (MRI) of the brain to reveal an infarct in the right caudate nucleus and an infarct in the right cerebellum. Doctors prescribed intense physical therapy. Neil saw some improvements for articulation, but he continued to struggle with the speed of speech and began to experience weakness of the tongue on his left side. CT angiograms revealed an aneurysm of the infrarenal aorta extending into the right common iliac artery. A spinal MRI revealed degenerative disease of the cervical spine (C4-C5) with a mild central canal stenosis. No such degeneration was present in the thoracic or lumbar spine. However, Neil did have mild impingement of the right L3-L5 nerve roots. A lumbar puncture revealed normal CSF pressures, cell counts, and composition. Neil continued physical therapy and experienced significant improvements in speech, walking, and balance. He also commented that his muscle soreness and fatigue had improved.

Neil continued his physical therapy while recovering at home. However, his symptoms worsened over the following weeks. Neil grew progressively weaker, occasionally falling. He required assistance for walking, and he was experiencing some numbness in both hands. He was admitted to a hospital, where he underwent a neurological examination. Cognitive and cranial nerve tests were normal. There were no fasciculations of the neck or tongue muscles. He was able to rotate and extend the neck with normal strength, however neck flexion seemed to be compromised. Neil exhibited widespread fasciculations in his arms and legs, and the muscles of these limbs had mildly atrophied. Motor tests were conducted for the arms and legs (4/5), and wrist extensors and intrinsic muscles of the hand (3+/ 5). Neil exhibited hyperactive deep-tendon reflexes and positive Babinski signs bilaterally. Sensory examinations were unchanged.

Based on the compounding symptoms and rapid progression of the disease, doctors suspected that Neil was suffering from amyotrophic lateral sclerosis (ALS), a disease that results in the destruction of upper and lower motor neurons. To confirm this diagnosis, neurologists conducted a series of tests., including nerve conduction tests and needle EMG. Nerve conduction tests of the motor and sensory nerves revealed normal conduction velocities with low amplitude compound motor action potentials, and normal sensory nerve potentials. There were abundant fasciculations and characteristics that suggested remodeling of motor units following denervation. Furthermore, EMG suggested net motor unit loss. There was no evidence of demyelination. Based on these results, doctors confirmed the diagnosis of ALS. For the following weeks, Neil experienced physical therapy aimed to help him preserve his strength and energy. He soon began to experience difficulty swallowing, and his cough progressed. Soon thereafter he developed pneumonia, was placed on a feeding tube, and ultimately died just 15 weeks after his initial hospitalization.

5. Electromyography involves recording electrical activity from muscle fibers. Justify Neil's low amplitude compound motor action potentials given his diagnosis of ALS. Be sure to describe how a muscle fiber initiates and propagates action potentials, and state how that process differs in the case of ALS.

6. Motor unit remodeling includes a reduction in the number of motor units, atrophy of muscle fibers, and an increased number of fibers per motor unit. Describe how motor unit remodeling may be indicative of ALS. Be sure to describe the typical structure and function of a motor unit, a typical distribution of motor unit (and fiber types) within a muscle, and how motor unit remodeling could contribute to Neil's weakness.

7. Based on the etiology of the disease, justify the finding of hyperactive deep-tendon reflexes and positive Babinski signs bilaterally in a patient suffering from ALS. Be sure to describe a typical reflex, and describe how descending inhibition may modulate a reflex response.

8. Justify why Neil's motor tests progressively worsened, whereas sensory, and cognitive tests remained unchanged. Be sure to describe the central and peripheral nervous system structures required for perception versus movement, and identify where deficits may be found in a patient with ALS.

To investigate Neilâs symptoms further, doctors referred him to a neurologist, Dr. Bonasera, that specialized in motor disorders. Dr. Bonasera noted that Neil exhibited diminished right dorsalis pedis and posterior tibial pulses. His cognitive tests were normal. Tests of the cranial nerves were normal, with the exception of occasional slurred speech. His motor tests revealed generally slowed movements. Neil was experiencing pain and stiffness in the shoulder joints, which could explain his restricted range motion. Dr. Bonasera tested the motor strength of his finger extensors (4+/5), hip flexors (4/5), and various muscle groups in his arms and legs (5/5). There were tests for dysmetria (i.e., lack of coordination) and dexterity, which were normal yet slowed. Neil did have difficulty lifting his legs for some of the examinations, and he walked with a shuffling gate, often keeping both feet close together for a narrow base of support. He took very small steps and often stooped over while walking. When placing one foot in front of the other in a tandem walking stance, he would fall to either side and was unable to regain his balance. Sensory examination was normal, deep tendon reflexes were normal, and the Babinski sign was absent bilaterally. There were no Parkinsons-like symptoms, increased motor tone, or muscle fasciculations observed in testing.

Just one day later, another neurologist noted brisk reflexes and bilateral Babinski signs. Doctors performed magnetic resonance imaging (MRI) of the brain to reveal an infarct in the right caudate nucleus and an infarct in the right cerebellum. Doctors prescribed intense physical therapy. Neil saw some improvements for articulation, but he continued to struggle with the speed of speech and began to experience weakness of the tongue on his left side. CT angiograms revealed an aneurysm of the infrarenal aorta extending into the right common iliac artery. A spinal MRI revealed degenerative disease of the cervical spine (C4-C5) with a mild central canal stenosis. No such degeneration was present in the thoracic or lumbar spine. However, Neil did have mild impingement of the right L3-L5 nerve roots. A lumbar puncture revealed normal CSF pressures, cell counts, and composition. Neil continued physical therapy and experienced significant improvements in speech, walking, and balance. He also commented that his muscle soreness and fatigue had improved.

Neil continued his physical therapy while recovering at home. However, his symptoms worsened over the following weeks. Neil grew progressively weaker, occasionally falling. He required assistance for walking, and he was experiencing some numbness in both hands. He was admitted to a hospital, where he underwent a neurological examination. Cognitive and cranial nerve tests were normal. There were no fasciculations of the neck or tongue muscles. He was able to rotate and extend the neck with normal strength, however neck flexion seemed to be compromised. Neil exhibited widespread fasciculations in his arms and legs, and the muscles of these limbs had mildly atrophied. Motor tests were conducted for the arms and legs (4/5), and wrist extensors and intrinsic muscles of the hand (3+/ 5). Neil exhibited hyperactive deep-tendon reflexes and positive Babinski signs bilaterally. Sensory examinations were unchanged.

Based on the compounding symptoms and rapid progression of the disease, doctors suspected that Neil was suffering from amyotrophic lateral sclerosis (ALS), a disease that results in the destruction of upper and lower motor neurons. To confirm this diagnosis, neurologists conducted a series of tests., including nerve conduction tests and needle EMG. Nerve conduction tests of the motor and sensory nerves revealed normal conduction velocities with low amplitude compound motor action potentials, and normal sensory nerve potentials. There were abundant fasciculations and characteristics that suggested remodeling of motor units following denervation. Furthermore, EMG suggested net motor unit loss. There was no evidence of demyelination. Based on these results, doctors confirmed the diagnosis of ALS. For the following weeks, Neil experienced physical therapy aimed to help him preserve his strength and energy. He soon began to experience difficulty swallowing, and his cough progressed. Soon thereafter he developed pneumonia, was placed on a feeding tube, and ultimately died just 15 weeks after his initial hospitalization.

7. Based on the etiology of the disease, justify the finding of hyperactive deep-tendon reflexes and positive Babinski signs bilaterally in a patient suffering from ALS. Be sure to describe a typical reflex, and describe how descending inhibition may modulate a reflex response.

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