The symptoms of syringomyelia usually present slowly and may eventually lead to permanent disabilities. Signs and symptoms of significant syrinx formation are mostly observed in ages 25 to 40 years old. It is imperative to identify the early signs of the disease to merit prompt medical or surgical attention.

In the head and neck areas, signs are often times unilateral. The tongue may present with weakness and hemiatrophy. Difficulty in deglutition may be experienced early. Facial paresis or diminished sensation of the face may be experienced up to the nape area. The eyes will noticeably have slow reaction to tactile stimulus or altered reflexes. Skin areas where supplying nerves are affected by the lesion may experience diminished pain and temperature sensation. Pain may be felt in certain dermatomes supplied by the affected nerves. The lower extremities are more prone to weakness or spasms during caudal compressions although arm fasciculation may be experienced in cervical lesions. Stiffness of back muscles, shoulders and arms are common. Spine deformities may be observable in large spinal tumors while scoliosis of the spine is not infrequent. The lower abdomen may be affected in syringes within the thoracic spinal cavity causing disruption in bowel activity and urinary bladder functions. Chronic constipation and bladder atony may be observable in these cases.

• Arm Pain

  Signs of syringomyelia: Headaches Curved spine Increased sweating Tingling Back pain Neck pain Arm pain Shoulder stiffness Back stiffness Arm stiffness Leg stiffness Weak muscles Reduced reflexes Numbness Twitching Sexual issues Blood pressure changes [baptisthealth.com]

  […] depending on size and place of the syrinx cavity exaggerated sensation (hypersensitivity) in limbs, mostly in the arms Pain symptoms midline pain over the spine, particularly the trunk area burning pain in arms, over trunk and, rarely, legs joint pain [beaumont.edu]

• Hand Pain

  Other symptoms Painless ulcers of the hands are frequent. Oedema and hyperhidrosis can occur due to interruption of central autonomic pathways. [physio-pedia.com]

• Weight Loss

  GI symptoms include nausea, vomiting, weight loss, and abdominal spasms. Respiratory disturbances may manifest during sleep. Joint arthropathy and trophic skin changes may eventually develop. [medical-dictionary.thefreedictionary.com]

• Fecal Incontinence

  A 10-year-old patient who was diagnosed with DWM in infancy presented with progressive scoliosis and fecal incontinence. [ncbi.nlm.nih.gov]

  Unsteady gait, muscle atrophy, spasticity, urinary or fecal incontinence, or male impotence may also be described10,16.Neuropathic and Chronic Pain can be a sequel that presents during their thirties to forties 17. [now.aapmr.org]

• Diplopia

  Patient 2 was a 49-year-old man, who was admitted to the hospital with headache, diplopia, hoarseness, dysphagia and ataxia five months after SAH. [ncbi.nlm.nih.gov]

  Numbness - may replace pain Signs - Horner's syndrome, Nystagmus, Muscle wasting, LE spasticity, Charcot UE joints, Pes Cavus, Short neck, Low hairline, Limb length inequality, Hand/foot asymmetry, Diplopia, Giddiness, Dysphagia, Dysphonia, Salivation [gait.aidi.udel.edu]

  Brainstem involvement may cause dysphagia, ptosis, miosis, or diplopia. GI symptoms include nausea, vomiting, weight loss, and abdominal spasms. Respiratory disturbances may manifest during sleep. [medical-dictionary.thefreedictionary.com]

  Visual disturbances: Retro-orbital pressure and pain, visual phenomena such as flashing lights, floaters, blurred vision, photophobia, diplopia; possible horizontal, rotatory, or down-beat nystagmus on clinical examination because of the involvement of [statpearls.com]

  Box 2 Symptoms of Chiari malformation HEADACHES Valsalva-induced Occipital Generalised BULBAR “Dizziness” Tinnitus/hearing loss Dysphagia, dysphonia Diplopia/oscillopsia * Sleep apnoea† LONG TRACTS Ataxia/subjective sense of imbalance Subjective weakness [pn.bmj.com]

• Prolapse

  […] dysfunction Respiratory insufficiency Other: painless ulcers of the hands, Charcot joints, thoracic scoliosis Syringobulbia: a neurological disorder characterized by syringomyelia affecting the brainstem Diagnostics Differential diagnoses Cervical disk prolapse [amboss.com]

• Miosis

  Brainstem involvement may cause dysphagia, ptosis, miosis, or diplopia. GI symptoms include nausea, vomiting, weight loss, and abdominal spasms. Respiratory disturbances may manifest during sleep. [medical-dictionary.thefreedictionary.com]

• Neuropathic Arthropathy

  RATIONALE: Charcot arthropathy, also known as Neuropathic arthropathy (NA), is an unusual chronic degenerative disease. To date, there exists a paucity of research on NA caused by syringomyelia. [ncbi.nlm.nih.gov]

  Mitchell, in 1831, initially identified a destructive arthropathy associated with diseases involving peripheral nerves. Subsequently in 1868, Charcot described neuropathic arthropathy in a patient with tabes dorsalis. [medscape.com]

  Keywords Neuropathic arthropathy; Syringomyelia; Shoulder Introduction Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. [jneuro.com]

  […] joints: - neuropathic shoulder: - most commonly involved joint; - neuropathic elbow: - look for associated ulnar and radial nerve entrapment at the elbow; - ref: Neuropathic Arthropathy of the Elbow. [wheelessonline.com]

• Arm Weakness

  Patients may also experience cervical and occipital pain and hand and arm weakness. Treatment and Surgery for Syringomyelia Syringomyelia may be treated expectantly with clinical and radiographic follow-up in asymptomatic cases. [princetonneurologicalsurgery.com]

  and hands and feet Hand and arm weakness and loss of muscle function High muscle tone in the arms and legs (spasticity) Sleep apnea Syringomyelia is diagnosed with MRI. [childrenshospitaloakland.org]

• Muscular Atrophy

  It results in neurological deficits that generally consist of segmental muscular weakness and atrophy accompanied by a dissociated sensory loss (loss of pain and temperature sensation, with preservation of the sense of touch), and thoracic scoliosis is [medical-dictionary.thefreedictionary.com]

  Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation). [fpnotebook.com]

  These conditions include: multiple sclerosis ankylosing spondylitis amyotrophic lateral sclerosis central pontine myelinolysis spinal muscular atrophy diabetic neuropathy chronic inflammatory demyelinating polyneuropathy arteriovenous malformations Syringobulbia [medicalnewstoday.com]

  Muscular atrophies are found especially in the lower neck, the shoulders, arms and hands. Neurological examination revealed diminished or abolished tendon reflexes. [medicaldb.blogspot.com]

• Muscle Spasticity

  Syringomyelia is the development of a fluid-filled cavity or syrinx within the spinal cord that can cause loss of sensation and muscle spasticity. [ncbi.nlm.nih.gov]

• Facial Numbness

  Other symptoms which sometimes accompany the pain in the arms, back, and neck include bowel function problems, bladder problems, muscle spasms (especially in the legs), facial numbness or pain, and scoliosis (curvature of the spine). [disability-benefits-help.org]

  Patients may experience problems with sleeping (pons), breathing (medulla), swallowing, facial pain or numbness, hearing loss, irregular heartbeat, and digestion. [mayfieldclinic.com]

• Headache

  Tussive headaches: Classic of CM1. [statpearls.com]

  People may experience symptoms that range from headache to irritable bowel. The five most common symptoms are: 1. Pressure-like headaches at the back of the skull that worsen with physical strain or coughing; often with neck pain 2. [mayfieldclinic.com]

  Temporary deteriorations in the condition (headache worsening, meteosensitivity) were detected in 11 (8.9%) patients. These symptoms regressed by the end of the 1st postoperative month. There were no deaths. [ncbi.nlm.nih.gov]

  Other symptoms may include headaches and a loss of the ability to feel extremes of hot or cold, especially in the hands. Signs of the disorder tend to develop slowly, although sudden onset may occur with coughing or straining. [ninds.nih.gov]

• Tingling

  Symptoms • Loss of sensitivity, especially to hot and cold • Sensory loss in a "cape" distribution (over the tops of the shoulders) • Numbness and tingling in hands and feet • Muscle weakness and spasticity • Headaches (due to Chiari malformation) • Pain [mayfieldchiaricenter.com]

  This can include, for example, tingling and a feeling of numbness in the hands or feet, or a reduced temperature - or pain in the affected areas. [avicenna-klinik.com]

  Signs of syringomyelia: Headaches Curved spine Increased sweating Tingling Back pain Neck pain Arm pain Shoulder stiffness Back stiffness Arm stiffness Leg stiffness Weak muscles Reduced reflexes Numbness Twitching Sexual issues Blood pressure changes [baptisthealth.com]

  Additionally, numbness and tingling in the arm, chest, or back is often reported. The inability to feel the ground under the foot, or tingling in the legs and feet is also frequently experienced. [encyclopedia.com]

• Nystagmus

  Syringobulbia may cause vertigo, nystagmus, unilateral or bilateral loss of facial sensation, lingual atrophy and weakness, dysarthria, dysphagia, hoarseness, and sometimes peripheral sensory or motor deficits due to medullary compression. [merckmanuals.com]

  […] worse over time Pain down the arms, neck, or into the middle back or legs Weakness (decreased muscle strength) in the arms or legs Painless burn or injury of the hand Difficulty walking or toe walking in children Uncontrollable movements of the eyes ( nystagmus [nlm.nih.gov]

  Numbness - may replace pain Signs - Horner's syndrome, Nystagmus, Muscle wasting, LE spasticity, Charcot UE joints, Pes Cavus, Short neck, Low hairline, Limb length inequality, Hand/foot asymmetry, Diplopia, Giddiness, Dysphagia, Dysphonia, Salivation [gait.aidi.udel.edu]

  […] worse over time Pain down the arms, neck, or into the middle back or legs Weakness (decreased muscle strength) in the arms or legs Painless burn or injury of the hand Difficulty walking or toe walking in children Uncontrollable movements of the eyes (nystagmus [ufhealth.org]

• Cape-like Loss of Pain and Temperature Sensation

  Patients with this condition demonstrate a wide variety of neurological symptoms depending on where exactly the syrinx is located, but classically patients present with: a cape-like loss of pain and temperature sensation along the back and arms, known [radiopaedia.org]

  The disorder generally leads to a cape-like loss of pain and temperature sensation along the back and arms. Each patient experiences a different combination of symptoms. [urmc.rochester.edu]

  The disorder often leads to a cape-like loss of pain and temperature sensation along the back and arms. Each patient experiences a different combination of symptoms. [chrichmond.org]

• Paresthesia

  A 21-year-old man working as a carpenter presented with a 1-year history of paresthesias in his right arm. [ncbi.nlm.nih.gov]

  Syringomyelia is marked clinically by pain and paresthesia, followed by muscular atrophy of the hands and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved; later marked by painless whitlow, spastic paralysis [medical-dictionary.thefreedictionary.com]

  Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation). [fpnotebook.com]

  Paresthesia Paresis and paresthesias followed by pain. And some studies suggest that sensory disturbance is the common manifestation of syringomyelia.[2]. Sensory disturbance Common presentation. [explainmedicine.com]

A detailed medical history and clinical examination is paramount in the diagnosis of syringomyelia. The following imaging techniques offer the most reliable diagnostic choice [6]:

• Magnetic resonance imaging (MRI) is the most reliable tool to diagnose syringomyelia especially when it comes to the detailed magnetic image of a syrinx formation in spinal cord area. The entire spinal canal should be investigated with and without contrast. For a more detailed assessment of syringomyelia, flow-sensitive sequences are necessary.
• Computed tomography (CT) may demonstrate the anomaly in the spine or brain effectively and may demonstrated inherent new growths in the area like tumors.

Asymptomatic syringomyelia may be treated with constant monitoring and conservative treatment, in severe, symptomatic cases, surgery is necessary. Identification of the underlying etiology is paramount to identify the appropriate treatment. The conservative treatment includes analgesics and physical therapy. The following surgical approaches are available:

• Suboccipital and cervical decompression [7]
• Laminectomy with syringotomy
• Lumboperitoneal or syringoperitoneal shunt placement
• Fourth ventriculostomy
• Percutaneous needling
• Neuroendoscopic surgery

Successful surgery reduces the size of the syrinx. Regular follow-up with MRI is important to detect recurrence early.

The intensity and extent of the symptoms are not directly dependent on the size of the syrinx. In mild non-progressive asymptomatic cases of syringomyelia, an uneventful clinical course may be expected. In progressive and enlarging syrinx however, progressive loss of motor and sensory functions of the extremities may be observed. Unabated compression may cause severe disability to the patient in the long run. Surgical therapy and the underlying etiology may significantly affect prognosis and outcome of the disease [5].

A syringomyelia may be congenital or acquired [1]. Congenital forms are common and in about 50% associated with malformations such as Arnold Chiari malformation, platybasia and Klippel-Feil syndrome. There are a number of diseases that can lead to the development of syringomyelia, including:

• Intramedullary neoplasms
• Tumors of the posterior fossa
• Meningeal carcinomatosis
• Trauma
• Infection (Meningitis, arachnoiditis, etc.)
• Ischemia
• Radiation
• Cysts
• Scoliosis
• Herniated disc

In about 20 to 30% of cases, no cause can be found, this is referred to as idiopathic syringomyelia.

The prevalence of syringomyelia is relatively low and is estimated at about 84 cases per 1,000,000 inhabitants. In Japan only 19 cases per million population were described. In most cases the disease occurs in the third and fourth decade of life, childhood cases have been reported.

The exact pathophysiology in syringomyelia remains unknown. However there are leading theories to explain it:

• The hydrodynamic theory as postulated by Gardner states that the syrinx formation is created due to the pulsatile pressure created by the cerebrospinal fluid during its flow from the brain ventricles to the central canal of the spinal cord. The theory suggest that any impedance like the closure of the foramen of Magendie may initiate the process to form the cyst [2].
• The difference of the intraspinal pressure and the cerebrospinal pressure gradient causes the cerebrospinal fluid to collect in the caudal area due to active regulation at the foramen magnum to prevent intracerebral accumulation of fluid during situations such as Valsalva or coughing [3]. Narrowing at the foramen magnum due to for example Chiari malformation causes syringomyelia.
• The caudal movement of the cerebellar tonsils during systole forces cerebrospinal fluid into the lesion increasing intramedullary pressure [4].

Patients diagnosed early with syringomyelia should avoid all occasion of straining and lifting to prevent the enlargement of the syrinx. For those with neurologic complications, physical therapy may significantly reduce pain symptoms. Chronic pain may be submitted for pain management to prevent aggravation of symptoms [8].

Syringomyelia is an acquired or congenital disease that involves the development of a fluid-filled cavity or “syrinx” within the spinal cord. Small, slowly progressive lesions may be asymptomatic. However, typical symptoms of the disease are dissociated sensory loss at the level of the lesion, pain, trophic and vegetative disorders. Since obstruction of CSF pulsation is the cause of the syringomyelia, a potential therapy is surgical removal of this cause. Although the syringomyelia is not operated directly, it will be smaller when the cause has been removed successfully. The risk of the surgical procedure has to be considered when discussing possible surgical treatment options. In some patients, in spite of all diagnostic possibilities, no cause of the disease can be found. Here, an operation is rarely indicated and conservative treatment options, such as physical therapy or antiepileptic medicatio ncan be considered.

Syringomyelia is the development of a fluid-filled cyst or cavity, called syrinx, within the spinal cord. The most common cause of syringomyelia is an abnormality of the brain (Arnold-Chiari malformation), other causes include tumors, injuries and inflammation. Common symptoms are pain, abnormal sensations, loss of sensation, weakness, stiffness, among others. If the condition is not causing severe symptoms, monitoring and conservative treatment (painkillers and physical therapy) may be sufficient. In other cases, surgery will be needed.

1. Lin JW, Lin MS, Lin CM, Tseng CH, Tsai SH, Kan IH. Idiopathic syringomyelia: case report and review of the literature. Acta Neurochir Suppl. 2006;99:117-20.
2. Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry. 1965 Jun. 28:247-59.
3. Williams B. Progress in syringomyelia. Neurol Res. 1986 Sep. 8(3):130-45.
4. Oldfield EH, Muraszko K, Shawker TH, Patronas NJ. Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment. J Neurosurg. 1994 Jan. 80(1):3-15.
5. Williams B. Progress in syringomyelia. Neurol Res. Sep 1986;8(3):130-45
6. Gruber DP, Crone KR. Neuroendoscopy. In: Grossman RG, Loftus CM, eds. Principles of Neurosurgery. 2nd ed. Philadelphia: Lippincott-Raven; 1998:757-62
7. Prat R, Galeano I. Pain improvement in patients with syringomyelia and Chiari I malformation treated with suboccipital decompression and tonsillar coagulation. J Clin Neurosci. Apr 2009;16(4):531-4.
8. Greitz D. Unraveling the riddle of syringomyelia. Neurosurg Rev. Oct 2006;29(4):251-63; discussion 264