Clinical Correlates Post-Midterm

Question 1

ALS Cause, Symptoms

Answer 1

• Progressive neuromuscular disease that destroys lower motor neurons, coticospinal/corticobulbar tracts and the primary motor area
• Symptoms:
  • Atrophy, weakness, fasciculations, spasms and cramping of affected muscles
  • loss of bulk in thenar, hypothenar, interossei, arm & shoulder
  • Reduction of cough reflex–> risk of aspiration pneumonia
  • Involvement of bulbar motor nuclei–> difficulty in swallowing, coughing, and speaking
  • Weakness, atrophy and fasciculations in tongue
  • Involvement of pharyngeal and laryngeal muscles
  • Death occurs from respiratory insufficiency and aspiration pneumonia

Question 2

Anterior Spinal Artery Occlusion Lesions of & Symptoms

Answer 2

• Lesions of + Anterior horn cell + Lateral and Anterior corticospinal tract + Anterolateral system - Symptoms: + Spastic parapesis + Bilateral extensor plantar response + Bilateral loss of pain and Temp below lesion + Retention of urine + Sexual functions impaired + PVT intact

Question 3

Lesion at Vestibulo Cerebelum

Answer 3

• Nystagmus
• Tilted Head
• Titubation (head nodding)
• Truncal ataxia (imbalance) w

Question 4

Lesion at Spino-cerebellum

Answer 4

• Distrubances affect posture and movement of limbs
• Ataxias of the limbs common (ipsilateral)
• Gait ataxia accompanied by lurching to the side of the lesion

Question 5

Lesion at Cerebro-Cerebellum

Answer 5

• Disturbances affect accuracy and timing of movement
  
  • Ataxia
  • Decomposition of movement
  • Dysarthria (slurred monotonous speech)
  • Dyssynergia (uncoordination of limbs)
    
    • Dysdiadokinesia (inability to eprform rapidly alternating movements)
    • Dysmetria (past-pointing)
  • Hypotonia
  • Intention tremor
  • Rebound phenomenon

Question 6

Cerebellar Lesions: Tumor

Answer 6

• Midline astrocytomas in children
• Motor signs
  
  • Impared tandem walking
  • Nystagmus
  • Truncal ataxia
• Other manifestation
  
  • Headache
  • Hydrocephalus

Question 7

Cerebellar Lesions: Cerebellar Stroke

Answer 7

• Vertebral arterial system
  
  • Unilateral occlcusive disease is common
• Motor signs:
  
  • Dysarthria
  • Dyssynergia (ipsilateral)
    
    • Dysmetria
    • Dysdiakinesia
  • Intention tremor
  • Limb ataxia
  • Rebound Phenomenon
  • Truncal ataxia
• Brainstem signs

Question 8

Cerebellar Lesions: Malnutrition

Answer 8

• Vit B1 deficiency linked to degenration of the anterior vermis and adjacent parts of the remaining anterior cerebellar lobe
  
  • Alcoholics at risk
• Cortical Purkinje cells degenerate, heralding reactive gliosis
• Motor signs (primarily of the legs and trunk)
  
  • dysmetria of legs (heel-shin test) and lurching gait
  • truncal ataxia and intentiontremor

Question 9

Louis-Bar Syndrome

Answer 9

• Autosomal recessive disorder widespread degenration of cerebellar Purkinje cells and compromised immune function (chromosome 11)
• Delayed development of motor skills accompanies increased vulnerability to infection
• Most obvious signs relate to walking, talking, facial

Question 10

Ventromedial Hypothalamic Syndrome (Frohlich Syndrome)

Answer 10

Frohlich syndrome is a disorder of caloric balance characterized by obesity (besides other symptoms).

The obesity reflects damage to the ventromedial nculeus fo the hypothalamus.

Question 11

Diabetes Insipidus

Answer 11

Absence of ADH induces excessive thrist and drinking and the secretion of large vol of urine. The deficiency in ADH can reflect lesions of the supraoptic and paraventricular nuclei of the hypothalamus or interruption of the supraopticohypophyseal tract, the connection between the hypotalamus to the post pituitary.

The condition is often treatable with **ADH aginist. **

Question 12

Hypothermia

Answer 12

Hypothermia is pathologically decreased body Temp. It can reflect lesions of the post hypothalamus. Such lesion can leave the anterior hypothalamus unopposed, which induces a decrease in metabolism and motor activity, and peripheral vasodilation.

Consequences are reduced heat productuon and increased heat loss, yielding lowered body Temp.

Question 13

Hypertheermia

Answer 13

Hyperthermia is pathologically increased body temp. It can reflect lesions of the anterior hypothalamus. Such lesions can leave the posterior hypothalamus unopposed, increasing metabolism and shivering and peripheral vasoconstriction (reduced heat loss).

Question 14

Lead Poisoning

Answer 14

• Children of 6 years and younger are mor vulnerable.
• Symptoms:
  
  • Children
    
    • Encephalopathy
    • Diminished IQ
    • Learning disabilities
    • High levels: mental retardation, coma, death
  • Adults:
    
    • Memory & concentration problems
    • Peripheral neuropathy

Question 15

Leprosy (Hansen Disease)

Answer 15

• Infectious disease caused by Mycobacterium leprae. Cutaneous and Peripheral damage.
• Symptoms: Sensory loss and Muscle weakness
• Peripheral injuries may relfect _loss of protective nociception. _
• Treatment: Antibiotics

Question 16

Alcoholic Polyneuropathy

Answer 16

• Sensory and motor losses symmetric
• Starts with sensory neuropathy from distal
• Later, motor losses starting at the lower leg.
• Nerve conduction is normal

Question 17

Diabetes Mellitus

Answer 17

• Insulin diabetics express polyneuropathy.
• Sensory (symmetric), motor (asymmetric) and autonomic (culminating in dry skin) deficits are seen.
• Snensory symptoms usually begin in both legs, and losees reflect abnormalities of unmyelinated axons carryign pain & Temp.
• THe snensory ending and axons of small unmyelinated DRG cells are vulnerable to hyperglycemia.
• Cases are often complicated by diabetic vasculopathies

Question 18

Hirschsprung’s Disease (Megacolon)

Answer 18

• Hirschprung disease is congenital condition characterized by deficient motility and peristalsis of the distal colon.
• Due to absence Myenteric and Submucosal plexuses.
• Consequences: Trapped feces cause dilation (megacolon)

Question 19

Complex Regional Pain Syndrome (CRPS)

Answer 19

• Chronic neuropathic pain often follows injury of bone, soft tissue, or nervous tissue.
• Pain persistent even after apparent healing.
• Sympathetic may contribute to increased output or sensitization of nociceptors to norepinephrine, a postganglionic sympa NS.

Question 20

Automatic Bladder

Answer 20

• Following surpasacral spinal transection, reflexes in the sacral segment of the spinal cord may recover.
• The bladder fills until a threshold in pressure is reached, leading to a spontaneous reflexive emptying of the bladder.
• The causal lesion disconnects to midsacral cord from higher centers. Therfore, nothing to regulate the reflex.

Question 21

Atonic Bladder

Answer 21

• Lesions to midsacral efferents or afferents interrupt the urinary reflex arc.
• The sensation of fullness of the urinary bladder may be lost.
• From the distended bladder, urine may dribble in the absence of a normal reflexive emptying.