Spinal Cord Pathology Study Guide

Spinal Cord Pathology Video

Spinal Cord Lesions

Spinal cord lesions are pathologic issues that effect the spinal cord.

Examples of spinal cord lesions include:

Syringomyelia
Poliomyelitis
Werdnig-Hoffman disease
Amyotrophic lateral sclerosis (ALS)
Friedrich ataxia

Syringomyelia

Syringomyelia is a cyst in the spinal cord.

Syringomyelias can enlarge, causing spinal compression neurologic symptoms.

Causes of syringomyelia include:

Cystic degeneration of the spinal cord when there has been trauma
Arnold-Chiari malformation

Syringomyelia typically occur between C8 and T1.

When the anterior white commissure of the spinothalamic tract is involved a syringomyelia, sensory loss of pain and temperature occurs with sparing of fine touch and position perception in the upper extremities in a “cape like” distribution.

Syringomyelia growth causes other spinal tracts to become involved, which results in weaker, atrophying muscles that have lower muscular tone.

Damage to the lower motor neurons of the anterior horn causes hyporeflexia (diminished reflexes).

Syringomyelia. Nevit Dilmen. Not altered. CC BY-SA 3.0
Syringomyelia. T2-weighted MRI of a syrinx located at C6–C7 in the cervical spine. Not altered. CC BY 4.0
Arnold Chiari Malformation. Syringomyelia associated with Chiari malformation © Nevit Dilmen Dichromatic, False-color MRI. Cervical spine, T1 Red, T2, Green& Blue Permission details You may select the license of your choice. Not altered. CC BY-SA 3.0
Pathological findings. Hematoxylin and eosin staining showed fibrous tissue on the wall of the cyst, and no epithelial cells were observed; the patient was diagnosed with arachnoid cyst (×200).Syringomyelia Associated with a Huge Retrocerebellar Arachnoid Cyst: A Case Report. Hue SB, Seong HY, Kwon SC, Lyo IU, Sim HB - Korean Journal of Spine (2015). Not Altered. CC.

Poliomyelitis

Poliomyelitis is due to infection with the poliovirus causes damage to the anterior motor horn.

Lower motor neuron symptoms of poliomyelitis include:

Flaccid paralysis with atrophying muscles
Fasciculations
Weakness with decreased muscle tone
Poor reflexes
A negative Babinski sign (down going toes when the bottom of the foot is stroked)

Poliomyelitis. A child receiving an oral polio vaccine USAID - USAID Bangladesh. Not altered. Public Domain
Poliomyelitis. A girl with genu recurvatum of her right leg due to polio Photo Credit: Content Providers(s): CDC - Not altered. Public Domain.
Poliomyelitis. Denervation of skeletal muscle tissue secondary to poliovirus infection can lead to paralysis. Photo Credit: Content Providers(s): CDC/Dr. Karp, Emory University - Not altered. public Domain
Poliomyelitis. A blockage of the lumbar anterior spinal cord artery due to polio (PV3) Photo Credit: Content Providers(s): CDC/Dr. Karp, Emory University - Not altered. Public Domain.
Poliomyelitis. Man on street with atrophy and paralysis of the right leg and foot due to polio. Photo Credit: Content Providers(s): CDC/NIP/ Barbara Rice - Not altered. Public domain.

Werdnig-Hoffman Disease

Werdnig-Hoffman disease is an inherited anterior motor horn degenerative disorder.

Werdnig-Hoffman disease is autosomal recessive.

Werdnig-Hoffman disease presents as a limp, floppy baby that passes away not long after birth.

Werdnig-Hoffman Disease. Spinal muscular atrophy has an autosomal recessive pattern of inheritance. Domaina, Kashmiri and SUM1 - Not altered. CC BY-SA 3.0
Werdnig-Hoffman Disease. X-ray showing bell-shaped torso due to atrophy of intercostal muscles and using abdominal muscles to breathe. Bell-shaped torso is not specific to individuals with SMA. An x-ray of a child with a bell-shaped torso due to muscle atrophy of the intercostal muscles. Prominent diaphragm. This image is not of a child with SMA, nor is it specific to SMA. There is a lead overlying the torso Not altered. CC BY-SA 4.0

Amytrophic Lateral Sclerosis

Amytrophic lateral sclerosis (ALS) is a corticospinal tract degenerative disease of the upper and lower motor neurons.

Amyotrophic lateral sclerosis is also known as Lou Gehrig disease.

Lower motor neuron signs are caused by anterior motor horn degeneration.

Amyotrophic lateral sclerosis (ALS) lower motor neuron signs include:

Fasciculations
Weakness
Decreased muscle tone
Poor reflexes
A negative Babinski sign
Paralysis

Amyotrophic lateral sclerosis (ALS) upper motor neuron signs include:

Spastic paralysis
Hyperreflexia
Increased muscular tone
Positive Babinski sign

Amyotrophic lateral sclerosis (ALS) results in lateral corticospinal tract degeneration.

Hand muscle atrophy and weakness are early warning signs of amyotrophic lateral sclerosis (ALS).

The majority of amyotrophic lateral sclerosis (ALS) cases are sporadic and affect middle-aged adults.

Some familial forms of amyotrophic lateral sclerosis are due to zinc-copper superoxide dismutase (SODl) mutation.

The familial zinc-copper superoxide dismutase (SODl) mutation causes free radical damage to neurons, leading to amyotrophic lateral sclerosis.

Amytrophic Lateral Sclerosis. MRI (axial FLAIR) demonstrates increased T2 signal within the posterior part of the internal capsule, consistent with the diagnosis of ALS. Frank Gaillard - Not altered. CC BY-SA 3.0
Amytrophic Lateral Sclerosis. This figure shows ten proposed disease mechanisms for ALS and the genes associated with them. Philip Van Damme, Wim Robberecht, and Ludo Van Den Bosch -Not altered. CC BY 3.0
Amytrophic Lateral Sclerosis. Typical or "classical" ALS involves neurons in the brain (upper motor neurons) and in the spinal cord (lower motor neurons).[33] Rcchang16 - Not altered. CC BY-SA 4.0
Amytrophic Lateral Sclerosis. Amyotrophic lateral sclerosis Frank Gaillard -Not altered. CC BY-SA 3.0

Friedreich Ataxia

Friedreich ataxia is a disorder that causes spinal cord and cerebellar degeneration.

Friedreich ataxia is brought on by cerebellar degeneration.

Symptoms of Friedreich ataxia include:

Muscle weakness in the lower limbs
Loss of deep tendon reflexes
Loss of vibratory sensation
Loss of proprioception

Friedreich ataxia is an autosomal recessive condition.

Friedreich ataxia is due to the growth of an unstable trinucleotide repeat (GAA) in the frataxin gene, which causes the frataxin protein to not form or to be abnormally complicated.

Frataxin normally prevents abnormal iron accumulation through it’s effect with mitochondria.

Abnormal or absent frataxin causes an accumulation of iron and free radical damage.

Complications of Friedreich ataxia include:

Difficulty walking (may need a wheelchair)
Hypertrophic cardiomyopathy