The natural history of the Chiari I malformation is unknown. Individual case studies provide clues. In a previous post, I presented a woman with the Chiari I malformation whose symptoms and MRI findings progressed over an 11 year period. Today’s post is about a 70 y/o woman whose symptoms began in 1969 but the diagnosis of Chiari I malformation with syringomyelia was not made for 38 years.
1974: “Terrified with Sailing”
In 1974, a then 32 y/o woman was seen in consultation by a neurologist who performed a careful history and neurological exam. According to his report:
PRESENT ILLNESS:“Her recent illness began about 7 weeks ago when she states she became terrified while sailing. ...However, subsequently she has experienced some vertigo when she turns her head to the left and possibly some tinnitus. She is also experiencing an exacerbation of what she describes as an inability to use her right hand. She states that she has no control over it and has difficulty writing and drops things. She also has had a recurrence of hypesthesia in the right arm, right trunk, right leg, right neck, and right face. All of these symptoms are improving.”
The neurologist’s review of her past history revealed “her biceps and brachioradialis being down on the right in1969” and complaints of “paresthesia with sneeze which went into the right arm.” She had been seen by a neurologist and ophthalmologist for “consideration of bulbar and cervical syringomyelia.” Other than rotatory nystagmus on right lateral gaze, the ophthalmologist “did not find any signs of syringobulbia”. By 1971, “she was able to resume her usual activities”.
EXAMINATION: The neurological examination in 1974 revealed “intact intellectual capabilities.” “She did not appear depressed or anxious.” Nystagmus on lateral gaze was present, otherwise cranial nerve function was normal. The “tympanic membranes appear congested.” Strength was normal and there was no atrophy in shoulders or arms. She had “mild difficulty with tandem gait.” Sensory function was variable in the arms, “an abrupt change from sharpness to dullness” was present over the trunk, and pin sensation was decreased in the right leg. Reflexes were normal.
IMPRESSION:“My initial impression is that the patient’s neurological complaints are hysterical in nature.”
RECOMMENDATION: A decongestant was prescribed for serous otitis media and a skull series and cervical spine films were ordered. A follow-up visit was scheduled in three weeks. (Those documents are not available.)
................................................................................
An MRI was not obtained in 1974 since it had not yet been invented. Computed tomography (CT) was still new - the first human scan was performed in England in 1972 - and its use in diagnosing the Chiari malformation was limited. Myelography, the best test available, required injection of an oil-based contrast agent into the spinal canal that required removal - by aspiration through the needle - once the study was completed.
She seems to have handled her diagnosis of hysteria well and, fortunately, had a long period or relative stability in until a fall in April 2007.
................................................................................
2007: Forearm gets “Really hot”
Present Illness:After a fall in April 2007, she noted the development of “a hot and burning sensation” in the right shoulder. Her forearm becomes “really hot” and the burning sensation extends to the axilla and under the right breast. Occasionally she notes numbness in right face.
Examination: Cranial nerve exam revealed rotatory nystagmus in all fields of gaze. There was slight weakness in the right deltoid and triceps and decreased sensation from C3 extending down the right arm, trunk, and leg. Reflexes were absent in right arm, hyperactive in the right leg, and normal in the left arm and leg. There were no pathological reflexes. Her gait was slightly wide-based and she had difficulty with tandem gait.
Diagnostic Studies:MRI of the brain and spine revealed 13 mm of tonsillar herniation, descent of the lower medulla into the upper cervical canal, and a syrinx from C2 to T10.
The cervical axial MRI above shows a fistula at the right dorsal root entry zone that connects the syrinx to the subarachnoid space. The syrinx in s the white bright area in the center of the image. At about the 7 o’clock position, the syrinx breaks through to the spinal canal.
Impression:Right shoulder, arm and chest pain secondary to syringomyelia associated with Chiari I malformation.
Plan:Her family physician prescribed pregabalin. A lthough she still has some bouts of intense pain, overall, she is improved. At the present time she wants to continue with medical management.
Take Home Points
1. The MRI has revolutionized the diagnosis of the Chiari malformation and syringomyelia. The sagittal T1 and T2 views provide the best overview of these conditions.
2. Some persons may have long plateaus of relative neurological stability interspersed with episodes to significant neurological symptoms that limit the quality of life.
3. A long period of stability may be due to spontaneous decompression of a large syrinx. This occurs when the syrinx breaks through a thin portion of the cord and drains into the spinal canal. Unfortunately, one cannot predict if, or when, spontaneous decompression will occur. Thus, development of a fistula cannot be relied upon to prevent serious progression of neurological deficits.
Posted by John Oro’, MD
Posted with permission of the patient.
The natural history of the Chiari I malformation is unknown. Individual case studies provide clues. In a previous post, I presented a woman with the Chiari I malformation whose symptoms and MRI findings progressed over an 11 year period. Today’s post is about a 70 y/o woman whose symptoms began in 1969 but the diagnosis of Chiari I malformation with syringomyelia was not made for 38 years.
1974: “Terrified with Sailing”
In 1974, a then 32 y/o woman was seen in consultation by a neurologist who performed a careful history and neurological exam. According to his report:
PRESENT ILLNESS:“Her recent illness began about 7 weeks ago when she states she became terrified while sailing. ...However, subsequently she has experienced some vertigo when she turns her head to the left and possibly some tinnitus. She is also experiencing an exacerbation of what she describes as an inability to use her right hand. She states that she has no control over it and has difficulty writing and drops things. She also has had a recurrence of hypesthesia in the right arm, right trunk, right leg, right neck, and right face. All of these symptoms are improving.”
The neurologist’s review of her past history revealed “her biceps and brachioradialis being down on the right in1969” and complaints of “paresthesia with sneeze which went into the right arm.” She had been seen by a neurologist and ophthalmologist for “consideration of bulbar and cervical syringomyelia.” Other than rotatory nystagmus on right lateral gaze, the ophthalmologist “did not find any signs of syringobulbia”. By 1971, “she was able to resume her usual activities”.
EXAMINATION: The neurological examination in 1974 revealed “intact intellectual capabilities.” “She did not appear depressed or anxious.” Nystagmus on lateral gaze was present, otherwise cranial nerve function was normal. The “tympanic membranes appear congested.” Strength was normal and there was no atrophy in shoulders or arms. She had “mild difficulty with tandem gait.” Sensory function was variable in the arms, “an abrupt change from sharpness to dullness” was present over the trunk, and pin sensation was decreased in the right leg. Reflexes were normal.
IMPRESSION:“My initial impression is that the patient’s neurological complaints are hysterical in nature.”
RECOMMENDATION: A decongestant was prescribed for serous otitis media and a skull series and cervical spine films were ordered. A follow-up visit was scheduled in three weeks. (Those documents are not available.)
................................................................................
An MRI was not obtained in 1974 since it had not yet been invented. Computed tomography (CT) was still new - the first human scan was performed in England in 1972 - and its use in diagnosing the Chiari malformation was limited. Myelography, the best test available, required injection of an oil-based contrast agent into the spinal canal that required removal - by aspiration through the needle - once the study was completed.
She seems to have handled her diagnosis of hysteria well and, fortunately, had a long period or relative stability in until a fall in April 2007.
................................................................................
2007: Forearm gets “Really hot”
Present Illness:After a fall in April 2007, she noted the development of “a hot and burning sensation” in the right shoulder. Her forearm becomes “really hot” and the burning sensation extends to the axilla and under the right breast. Occasionally she notes numbness in right face.
Examination: Cranial nerve exam revealed rotatory nystagmus in all fields of gaze. There was slight weakness in the right deltoid and triceps and decreased sensation from C3 extending down the right arm, trunk, and leg. Reflexes were absent in right arm, hyperactive in the right leg, and normal in the left arm and leg. There were no pathological reflexes. Her gait was slightly wide-based and she had difficulty with tandem gait.
Diagnostic Studies:MRI of the brain and spine revealed 13 mm of tonsillar herniation, descent of the lower medulla into the upper cervical canal, and a syrinx from C2 to T10.
The cervical axial MRI above shows a fistula at the right dorsal root entry zone that connects the syrinx to the subarachnoid space. The syrinx in s the white bright area in the center of the image. At about the 7 o’clock position, the syrinx breaks through to the spinal canal.
Impression:Right shoulder, arm and chest pain secondary to syringomyelia associated with Chiari I malformation.
Plan:Her family physician prescribed pregabalin. A lthough she still has some bouts of intense pain, overall, she is improved. At the present time she wants to continue with medical management.
Take Home Points
1. The MRI has revolutionized the diagnosis of the Chiari malformation and syringomyelia. The sagittal T1 and T2 views provide the best overview of these conditions.
2. Some persons may have long plateaus of relative neurological stability interspersed with episodes to significant neurological symptoms that limit the quality of life.
3. A long period of stability may be due to spontaneous decompression of a large syrinx. This occurs when the syrinx breaks through a thin portion of the cord and drains into the spinal canal. Unfortunately, one cannot predict if, or when, spontaneous decompression will occur. Thus, development of a fistula cannot be relied upon to prevent serious progression of neurological deficits.
Posted by John Oro’, MD
Posted with permission of the patient.