QuestionSBA
QuestionChoice
The dorsal columns are responsible for fine touch, vibration and proprioception. The patient has no issues with these sensory modalities and so this is not responsible for his symptoms. Progression of the syrinx can, over time, impinge on the dorsal columns and cause deficiencies within these sensory modalities.
53099
c
Dorsal columns
QuestionChoice
This patient has evidence of corticospinal tract involvement since there is evidence of motor dysfunction with associated hand clawing. However, this would not explain his sensory symptoms, which is the question being asked.
53098
b
Corticospinal tracts
QuestionChoice
The description of loss of pain and temperature over the neck and back (in a cape-like distribution) and predominant upper-limb dysfunction with bilateral hand clawing fits with a diagnosis of syringomyelia. Syringomyelia is a fluid-filled cyst (syrinx) that forms within the centre of the spinal cord, initially compressing the decussation of the spinothalamic tract and leading to localised loss of pain and temperature sensation. Over time, it gets larger and can expand to multiple levels of the cord as well as other structures, such as the anterolateral corticospinal tracts, which this patient has evidence of due to the presence of motor symptoms, and the dorsal columns.
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a
Spinothalamic tract
QuestionChoice
The motor cortex is a cerebral structure within the frontal lobe. A lesion in the motor cortex causes upper motor neurone pathology and spasticity (hyperreflexia, pyramidal distribution of weakness and a velocity-dependent increase in tone). Sensory involvement is spared. A lesion in the motor cortex will therefore not explain this patient's symptoms.
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d
Motor cortex
QuestionChoice
The somatosensory cortex is a cerebral structure within the parietal lobe. Depending on the precise location of the lesion in the somatosensory cortex, any sensory modality may be lost in the distribution as dictated by the sensory homunculus. However, sensory loss with cerebral lesions do not have such a defined pattern of presentation, with dorsal column sparing and a cape-like distribution. Loss of sensation in this pattern should raise clinical suspicion of a spinal lesion.
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Somatosensory cortex
QuestionComment
Spain = (s)pain = Spinothalamic 
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User
subscriber
Liver Anterior
QuestionComment
sPinoThalamic (Pain and Temp)
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User
subscriber
j.g.73
Concept
Chapter
# Summary

Syringomyelia is a neurological condition characterised by a fluid-filled cavity, known as a syrinx, that forms within the spinal cord. This condition typically manifests with distinct neurologic symptoms such as pain and temperature loss in a 'shawl-like distribution' across the upper body, tactile sensory loss, muscle weakness, and autonomic dysfunction including bladder, bowel, and sexual dysfunction. Diagnosis is typically confirmed via imaging techniques, primarily MRI of the spinal cord and CT of the bony spinal canal. Management approaches are generally tailored to the individual, ranging from physiotherapy and rehabilitation to surgical intervention when symptomatology and the nature of the lesion warrant such.

# Definition

Syringomyelia is a neurological disorder characterised by the formation of a fluid-filled cavity or cyst, known as a syrinx, within the spinal cord. This syrinx often expands and elongates over time, exerting pressure on the spinal cord and damaging nerve fibres.

# Epidemiology

Syringomyelia is a relatively rare condition, with the prevalence reported as approximately 8.4 per 100,000 individuals, with a slight male predominance. The condition is typically diagnosed in adulthood, most often between the ages of 20 and 40.

# Aetiology

The formation of a syrinx in syringomyelia can be attributed to a variety of causes:

* Cerebrospinal Fluid (CSF) blockage or disruption, often secondary to conditions such as arachnoiditis or presence of space-occupying lesions.
* Spina Bifida
* Post-traumatic syringomyelia following spinal cord injury.
* Spinal cord tumours, which may create a blockage of CSF flow.
* Idiopathic syringomyelia, where no specific cause can be identified.
* Familial predisposition has also been noted in some cases.

# Signs and Symptoms

The clinical manifestations of syringomyelia can be diverse and primarily revolve around damage to various neural pathways:

* Pain and temperature loss, typically presenting in a 'shawl-like distribution' over the arms, shoulders, and upper body due to damage to the spinothalamic tract.
* Impaired light touch, vibration and position senses as the syrinx enlarges and further compromises the spinal cord.
* Muscle weakness and atrophy may ensue as the syrinx extends and damages the anterior horn cells and their lower motor neurons.
* Autonomic dysfunction leading to bladder, bowel, and sexual dysfunction may also occur.

# Differential Diagnosis

The clinical presentation of syringomyelia can mimic other neurological disorders, warranting consideration for the following differential diagnoses:

* Multiple Sclerosis: Characterised by episodic neurologic deficits that change over time and space, including optic neuritis, limb weakness, and ataxia.
* Amyotrophic Lateral Sclerosis (ALS): Characterised by progressive muscle weakness, atrophy, and fasciculations due to a combination of upper and lower motor neuron damage.
* Spinal cord tumour: May present with back pain, sensory changes, motor weakness, and bladder/bowel dysfunction similar to syringomyelia.
* Cervical Spondylotic Myelopathy: Presents with neck pain, hand clumsiness, gait imbalance, and sometimes bladder symptoms.

# Investigations

The diagnosis of syringomyelia is typically confirmed through imaging studies:

* MRI of the spinal cord: This is the gold standard diagnostic modality, providing detailed visualisation of the syrinx and surrounding spinal cord structures.
* CT of the bony spinal canal: May provide supplementary information regarding any bony abnormalities or spinal cord compression.

# Management

The management of syringomyelia is patient-specific and depends on the severity of the symptoms and the underlying cause of the syrinx:

* Physiotherapy and Rehabilitation: Helps to manage symptoms such as muscle weakness and improve overall functional capacity.
* Surgery: May be recommended in cases where there are severe or worsening symptoms, or where the nature of the lesion (such as a tumour or significant CSF blockage) necessitates surgical intervention.

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Syringomyelia
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Neurology
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Syringomyelia causes a characteristic 'cape-like' distribution of pain and temperature loss due to compression of the spinothalamic tract in the spinal cord.
A 32-year-old man presents to the Neurology Clinic with impaired sensation over his neck and upper back. He says he cannot feel pain over this region when he pinches the skin, as well as weakness in his hands. On examination, he has a loss of pain and temperature sensation over his neck, back and upper shoulders. There is some clawing of his hands bilaterally with atrophy of the muscles of the forearm. Fine touch, proprioception and vibration are spared. There are no sensory or motor abnormalities of the lower limbs.

Where is the lesion responsible for his sensory symptoms?
a