Arnold-Chiari malformation history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Symptoms

Subtypes	Explanation
Acute Motor Axonal Neuropathy (AMAN)	The most common type (85-90%). Prior infection can trigger it. Autoimmune disorder. The target is schwann cell surface membrane or the myelin. Causes demyelination. In electrodiagnostic tests we can see slowing of nerve conduction. In pathology we can see lymphocytic infiltration of peripheral nerves and macrophage invasion of myelin sheath and schwann cells.
Acute Motor Axonal Neuropathy (AMAN)	It’s common among Chinese and Japanese people. It can be triggered by C. jejuni. It is associated with antiganglioside antibodies. Autoimmune disorder. Target is axonal membrane. Causes axonal degeneration in motor neurons. In electrodiagnostic study we can see reduction of compound muscle action potential.
Acute motor and sensory axonal neuropathy	The incidence rate is under 10%. Causes axonal degeneration. It is similar with AMAN but involves both motor and sensory axons.
Miller Fisher syndrome	Causes a clinical triad: ophthalmoplegia, ataxia and areflexia. Associated with ganglioside GQ1b antibody.

In infants, the most common symptoms are

• Stridor
• Swallowing difficulties

In older children

• Upper (and lower as age marches on) limb weakness
• Breathing difficulties may occur
• Patients may experience no symptoms or remain asymptomatic until early adulthood at which point they will often experience severe headaches and neck pain
• Fatigue
• Dizziness
• Vertigo
• Neuropathic pain
• Pain at the point of tethering
• Visual disturbances
• Difficulty swallowing
• Ringing in the ears
• Sleep apnea
• Impaired fine motor skills
• Muscle weakness
• Palpitations
• Excessive clearing of the throat with no obstructions
• Because of the complex combination of symptoms and the lack of experience with ACM1 had by many, even outstanding neurologists and neurosurgeons, many patients are frequently misdiagnosed.
• Some patients may go an entire lifetime without having noticeable symptoms. Or, symptoms can be minimal, then turn severe suddenly due to head trauma which alters the condition of the spine, brain, or cerebellar tonsils and begins to cause more difficulties.

• 
  Skin: Myelomeningocele with Hydrocephalus; Arnold Chiari; Note Underdevelopment of Legs

References

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