A 48-year old woman comes with bilateral progressive weakness of both lower limbs, spasticity and mild impairement of respiratory movements. MRI shows an intradural mid-dorsal midline enhancing lesion. What is the diagnosis?
**Question:** A 48-year old woman comes with bilateral progressive weakness of both lower limbs, spasticity and mild impairment of respiratory movements. MRI shows an intradural mid-dorsal midline enhancing lesion. What is the diagnosis?
A. Multiple Sclerosis
B. Primary CNS lymphoma
C. Motor Neuron Disease (MND)
D. Syringomyelia
**Correct Answer:** D. Syringomyelia
**Core Concept:**
The clinical presentation described in this question involves symptoms and signs of spinal cord compression and cord dysfunction. These symptoms are due to spinal cord injury, which can result from various conditions including tumors, infections, vascular lesions, and developmental anomalies. Syringomyelia is a condition characterized by the formation of a fluid-filled cavity (syrinx) within the spinal cord, typically secondary to a Chiari malformation or other structural abnormalities of the brain and skull base.
**Why the Correct Answer is Right:**
The correct answer, Syringomyelia (option D), is the most suitable diagnosis for the given symptoms and imaging findings. The MRI shows an intradural mid-dorsal midline enhancing lesion, which is indicative of a spinal cord lesion. In this case, the lesion is located in the mid-dorsal region of the spinal cord.
**Why Each Wrong Option is Incorrect:**
A. Multiple Sclerosis (option A) is an autoimmune disease affecting the central nervous system (CNS) causing demyelination, which typically presents with a combination of demyelinating lesions affecting different parts of the CNS (brain and spinal cord). Multiple sclerosis would not typically present with a midline spinal cord lesion.
B. Primary Central Nervous System (CNS) lymphoma (option B) is a type of cancer that usually affects the brain or spinal cord. While spinal cord lymphomas can be seen, they are not as commonly associated with a midline spinal cord lesion.
C. Motor Neuron Disease (option C) is a group of disorders affecting the motor neurons, leading to muscle weakness, wasting, and spasticity. While the symptoms of muscle weakness and spasticity are present, the spinal cord lesion in MND is usually diffuse rather than a midline lesion.
**Clinical Pearls:**
Syringomyelia often presents with symptoms such as pain, motor and sensory deficits, and bladder and bowel dysfunction, but typically, the clinical presentation is not as severe as described in the question. Additionally, syringomyelia is usually associated with a Chiari malformation or other structural abnormalities of the skull base. In this case, the midline spinal cord lesion is consistent with syringomyelia.
**Why Each Wrong Option is Incorrect:**
A. Multiple sclerosis typically presents with a combination of demyelinating lesions affecting different parts of the CNS (brain and spinal cord). The midline spinal cord lesion is not characteristic of multiple sclerosis.
B. Primary CNS lymphoma is a type of cancer affecting the brain or spinal cord, but the midline spinal cord lesion is not typical of this condition.
C. Motor Neuron Disease (MND) affects spinal cord motor neurons,