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Here is the answer to the recent case.
Question 1: From history and examination, list the problems with this patient
Here is the answer to the recent case.
Question 1: From history and examination, list the problems with this patient
- Rapidly progressive weakness of the lower limbs with numbness
- Ascending sensory level to T10
- Bowel and bladder disturbance
- Loss of muscle power, deep tendon reflexes and Babinski absent in lower limbs
- Incomplete presence of light touch and vibratory sense; absent nociception and thermoreception
- Loss of anal tone and 'wink' sign
- Hypertension
- Smoker
- Mildly expansile aorta
Question 2: What immediate test or tests would you perform and why?
From the acute history of weakness and numbness progressing over minutes, loss of bowel and bladder function with progression over several more hours is consistent with a acute spinal cord condition either from external compression or impairment of blood supply or a transverse myelitis.
The patient was otherwise well and was afebrile on admission, making a viral infection less likely. Moreover, there was no history of previous back problems e.g. disc herniation. The fact that the patient is a smoker and hypertension are cardiovascular risk factors and with this history, it is consistent with an acute spinal cord infarction.
The immediate test would be do perform an MRI to rule in or out a spinal cord compression. An abdominal ultrasound or CT to look for an aortic aneurysm would also be prudent if there was a history of abdominal pain and/or an abdominal aorta consistent with aneurysmal change. An ECG should be performed to look for atrial fibrillation which might contribute to systemic embolisation. Echocardiogram would also be worthwhile to rule out a cardiac source of embolism e.g. intra-atrial thrombus, vegetation from endocarditis.
Question 3: What anatomical problem is this from the history and physical examination?
The anatomical problem is two-fold. Firstly, the blood supply to the spinal cord has been impaired and this has led to infarction. However, from the physical examination, the nociception and thermoreception plus motor are predominantly impaired whereas a modicum of light touch and vibratory sense are maintained.
In view that the anterolateral cord contains the descending long tracts (corticospinal tracts) and the ascending spinothalamic tracts (pain and temperature), it would appear that the anterolateral cord is most affected with less of the dorsal cord affected which convey senses such as light touch and vibratory sense. However, strangely, the joint position sense was impaired which also runs up the dorsal columns. This cannot be easily explained unless the patient already had an existing peripheral neuropathy prior to the onset of this disorder e.g. diabetes mellitus.
In view that there is a single anterior spinal artery supplying the anterior cord and two posterior spinal arteries, this problem is consistent with an anterior spinal artery occlusion and anterolateral cord infarction.
Question 4: What is the current evidence based treatment for such a condition?
There have been many proposed therapies including anti-platelet treatment, anti-coagulation, thrombolytic therapy. This condition is relatively rare and so being able to perform a randomised control trial is difficult. Hence, most treatments are based on a case by case basis.
However, therapy is similar to that of cerebral infarction.
To date, there has been only one published case of using thrombolysis in a patient with this problem, although I have heard of another case of spinal cord infarction where thrombolysis was also used [unpublished] with good results.
Tex Heart Inst J. 2007;34(1):134-5.
Acute spinal cord ischemia during aortography treated with intravenous thrombolytic therapy.
Restrepo L, Guttin JF.
In the above case, the infarction occurred after aortography rather than de novo without an inciting cause.
In this case, anti-platelet therapy was commenced without modification of the patient symptoms and following this, heparin was commenced. The patient did not receive rTPA.
There was a minimal response to therapy with only a slight improvement in sensation but no improvement in motor response. It is therefore not certain whether the mild improvement was related to the treatment or just the natural course of the condition.
The intial MRI scan showed some osteoarthritis high up in the neck but not considered to be the cause of the symptoms when reviewed by the neurosurgeons. The cord proper looked normal.
A repeat MRI taken after several days again looked normal.
CSF examination was not performed.
ECG showed sinus rhythm and the echocardiogram revealed no thrombus. Abdominal echo revealed no evidence of an abdominal aortic aneurysm.
Arteritis was also considered but the ESR was within the normal range for age and sex.
Several doctors have kindly responded to the case and include Professor Alan Lefor, Professor of Surgery, Jichi medical school, Japan gives a surgeons view of the history and physical followed by the kind Dr Masami Matsumura of Kanazawa University School of Medicine with an indepth and excellent break down of the case into its essential elements. Thanks to both these esteemed doctors for their opinions here.
Professor Alan Lefor:
Questions
1) From history and examination, list the problems with this patient.
Problem list:
#1 Numbness of the buttocks
#2 Rapid onset lower limb weakness, then paralysis of the lower limbs
#3 Reduced muscle tone
#4 Reflexes knee (L2-4) absent, ankle (S1) absent
#5 Light touch- decreased in lower limbs up to the umbilicus (Th10) region
#6 Vibratory sense (posterior column) was intact on the pelvic brim (S3) and right knee (L4) but absent further down
#7 Nociception and thermoreception were absent.
#8 Reduced anal tone (S2-4)
#9 Bowel and bladder disturbance.
#10 Absence of joint position sense (posterior column)
#11 Hypertension
#12 Smoking history
#13 Aorta mildly expansile
Assessment:
At the beginning, this patient is 70 years old woman who has history of HTN and smoking. I would use following system for thinking differential diagnosis in view of weakness. I would differentiate neoplastic or degenerative diseases first.
Vascular: possible, infarction of spinal cord, arterio-venous malformation
Infection: less likely,spinal epidural abscess,Lyme disease, syphilis
Neoplastic: possible, vertebral or spinal cord tumor, metastasis ofbreast cancer, lung cancer,gastrointestinal cancers,or renal cell carcinoma, non-Hodgkin's lymphoma, plasmacytoma, multiple myeloma, orparaneoplastic syndrome
Collagen: possible, sarcoidosis, polymyositis, vasculitis, Guillan-Barre syndrome, SLE (very rare)
Toxic/Metabolic: less likely, periodic paralysis, hypokalemia
Trauma/Degenerative: possible,intervertebral disk disease or spinal stenosis,
Congenital: less likely
Iatrogenic: less likely
Idiopathic: less likely,amyloidosis
Cauda equina syndrome is likely in this case from problem #1 to #10.
#1 Numbness of the buttocks
#2 Rapid onset lower limb weakness, then paralysis of the lower limbs
#3 Reduced muscle tone
#4 Reflexes knee (L2-4) absent, ankle (S1) absent
#5 Light touch- decreased in lower limbs up to the umbilicus (Th10) region
#6 Vibratory sense was intact on the pelvic brim (S3) and right knee (L4) but absent further down
#7 Nociception and thermoreception were absent.
#8 Reduced anal tone (S2-4)
#9 Bowel and bladder disturbance
#10 Absence of joint position sense (posterior column)
Cauda equina syndrome is included in concept of epidural spinal cord compression (ESCC).
UpToDate 16.1 describes as follows:
Neopalastic ESCC is a common complication of cancer that causes pain and sometimes irreversible loss of neurologic function. In adults, the tip of the spinal cord usually lies at the L1 vertebral level; below this level, the lumbosacral nerve roots form the cauda equina, which floats in cerebrospinal fluid. With cauda equina lesions, the weakness is associated with depressed deep tendon reflexes in the legs.
My first question is as follows:
Can we interpret problem #5 Light touch- decreased in lower limbs up to the umbilicus (Th10) region as a finding of cauda equina syndrome?
UpToDate 16.1 describes as follows:
When a spinal sensory level is present, it is typically one to five levels below the actual level of cord compression. Saddle sensory loss is commonly present in cauda equina lesions, while lesions above the cauda equina frequently result in sparing of sacral dermatomes to pinprick.
Cauda equina syndrome can cause decreased light touch in lower limbs up to the umbilicus (Th10) region.
My second question is as follows:
Why she doesn't have back pain?
UpToDate 16.1 describes as follows:
Pain is usually the first symptom of ESCC, being present in 83 to 95 percent of patients at the time of diagnosis.
This patient didn’t complain back pain. But 5 to 17 percent of ESCC cases don’t disclose back pain.
Third my question is as follows:
What is the cause of her cauda equina syndrome?
UpToDate 16.1 describes as follows:
The three most common are prostate cancer, breast cancer, and lung cancer, each of which accounts for about 20 percent of cases.
This patient is woman and smoker.Vertebral or spinal cord tumor, metastasis of breast cancer, lung cancer, gastrointestinal cancers, or renal cell carcinoma, non-Hodgkin's lymphoma, plasmacytoma, or multiple myeloma must be differentiated.
Problem #13 Aorta mildly expansile is probably caused by #11 Hypertension.
Questions
2) What immediate test or tests would you perform and why?
MR imaging
UpToDate 16.1 describes as follows:
MR imaging offers several potential advantages over myelography.
It produces anatomically faithful images of the spinal cord and intramedullary pathology and is even more sensitive than radionuclide bone scans at identifying bony metastases. It can image the entire thecal sac regardless of whether a spinal subarachnoid block is present. It is not contraindicated with large brain metastases, thrombocytopenia, or coagulopathy. It spares the patient a lumbar puncture.
Questions
3) What anatomical problem is this from the history and physical examination?
From the history and physical examination, cauda equina syndrome is likely. Neoplastic disease or degenerative disease including spinal canal stenosis might be the cause of L1 or L2 vertebral level compression.
Questions
4) What is the current evidence based treatment for such a condition?
Administration of corticosteroids is first strategy. Next surgical management with anterior decompression should be considered. If diagnosis is neplastic, radiotherapy or chemotherapy should be planned.
UpToDate 16.1 describes as follows:
Management include the administration ofcorticosteroids(dexamethasone ), followed either by surgery and/or radiotherapy (RT). Aggressive surgical management with anterior decompression appears to give better results than RT alone in patients who are candidates for surgical intervention. Compared to RT alone, surgery followed by RT is associated with better neurologic outcomes. Chemotherapy may be beneficial in patients with chemosensitive tumors.
Interestingly, both Dr Matsumura and Professor Lefor listed spinal cord ischaemia / infarction as part of their differential diagnoses from the history and physical examinations. This is the most important thing.
The case by itself without the advanced imaging studies is very difficult to make an accurate diagnosis and in this case, MRI did not show an obvious mass lesion or even the usual cord infarction pattern. However, with the history of rapid onset, paralysis and sensory loss affecting specific sensory modalities, and a non-diagnostic MRI scan, spinal cord infarction was still considered the most likely.
This was later confirmed by a most excellent neurologist.
Summary
In summary, spinal cord ischaemia is rare accounting for <1% of all strokes. Most spinal cord ishaemia occurs due to abdominal surgery. The spinal cord is supplied by one anterior spinal artery and two posterior spinal arteries. Superiorly the vessels originate from the vertebral arteries and they receive feeding radicular arteries from many levels with many coming from the intercostal arteries.
The Adamkiewicz artery is most commonly affected. Studies have shown that the most probable cause of infarcts is related to mechanical stress affecting the radicular arteries which can extend for several levels. Vertebral body infarcts can also occur and are associated with cord infarction and can be another sign of radicular artery occlusion.
There is an association of spinal cord ischaemia and spinal disease and in such chronic compressive diseases there is impairment of blood flow which can impair collateral blood supplies putting at risk the compensatory mechanisms in the event of ischaemia.
Transient ischaemic attacks affecting the spinal cord can also occur.
Clinical Features Associated with Various Types of Spinal Cord Ischaemia
Anterior spinal artery territory- bilateral motor impairment with spinothalamic impairment
Posterior Spinal artery territory- bilateral motor impairment with dorsal column sensory impairment
Complete [transverse] infarct- Bilateral motor impairment with total sensory loss at and below the area of the ischaemia.
Unilateral infarcts produce a hemiparesis, in the case of anterior impairment, a contralateral spinothalamic deficit and with a posterior impairment, a unilateral dorsal column impairment.
[please review the neuroanatomy of the various sensory pathways-- spinothalamic neurones cross over at the level of entry and traverse to the contralateral side and ascend in the lateral spinothalamic tracts whereas the dorsal column input ascends directly from the site of entry and cross over in the brainstem region. The routes of these two pathways explain why there is a difference in the sensory impairment within the spinal cord and different findings on physical examination].
There are no clear guidelines for treatment as there have been no prospective clinical studies and various anti-thrombotic / anti-coagulation measures have been tried in previous cases. In my opinion, a prospective study seems unlikely to be able provide a clear answer because of the paucity of such strokes.
From the acute history of weakness and numbness progressing over minutes, loss of bowel and bladder function with progression over several more hours is consistent with a acute spinal cord condition either from external compression or impairment of blood supply or a transverse myelitis.
The patient was otherwise well and was afebrile on admission, making a viral infection less likely. Moreover, there was no history of previous back problems e.g. disc herniation. The fact that the patient is a smoker and hypertension are cardiovascular risk factors and with this history, it is consistent with an acute spinal cord infarction.
The immediate test would be do perform an MRI to rule in or out a spinal cord compression. An abdominal ultrasound or CT to look for an aortic aneurysm would also be prudent if there was a history of abdominal pain and/or an abdominal aorta consistent with aneurysmal change. An ECG should be performed to look for atrial fibrillation which might contribute to systemic embolisation. Echocardiogram would also be worthwhile to rule out a cardiac source of embolism e.g. intra-atrial thrombus, vegetation from endocarditis.
Question 3: What anatomical problem is this from the history and physical examination?
The anatomical problem is two-fold. Firstly, the blood supply to the spinal cord has been impaired and this has led to infarction. However, from the physical examination, the nociception and thermoreception plus motor are predominantly impaired whereas a modicum of light touch and vibratory sense are maintained.
In view that the anterolateral cord contains the descending long tracts (corticospinal tracts) and the ascending spinothalamic tracts (pain and temperature), it would appear that the anterolateral cord is most affected with less of the dorsal cord affected which convey senses such as light touch and vibratory sense. However, strangely, the joint position sense was impaired which also runs up the dorsal columns. This cannot be easily explained unless the patient already had an existing peripheral neuropathy prior to the onset of this disorder e.g. diabetes mellitus.
In view that there is a single anterior spinal artery supplying the anterior cord and two posterior spinal arteries, this problem is consistent with an anterior spinal artery occlusion and anterolateral cord infarction.
Question 4: What is the current evidence based treatment for such a condition?
There have been many proposed therapies including anti-platelet treatment, anti-coagulation, thrombolytic therapy. This condition is relatively rare and so being able to perform a randomised control trial is difficult. Hence, most treatments are based on a case by case basis.
However, therapy is similar to that of cerebral infarction.
To date, there has been only one published case of using thrombolysis in a patient with this problem, although I have heard of another case of spinal cord infarction where thrombolysis was also used [unpublished] with good results.
Tex Heart Inst J. 2007;34(1):134-5.
Acute spinal cord ischemia during aortography treated with intravenous thrombolytic therapy.
Restrepo L, Guttin JF.
In the above case, the infarction occurred after aortography rather than de novo without an inciting cause.
In this case, anti-platelet therapy was commenced without modification of the patient symptoms and following this, heparin was commenced. The patient did not receive rTPA.
There was a minimal response to therapy with only a slight improvement in sensation but no improvement in motor response. It is therefore not certain whether the mild improvement was related to the treatment or just the natural course of the condition.
The intial MRI scan showed some osteoarthritis high up in the neck but not considered to be the cause of the symptoms when reviewed by the neurosurgeons. The cord proper looked normal.
A repeat MRI taken after several days again looked normal.
CSF examination was not performed.
ECG showed sinus rhythm and the echocardiogram revealed no thrombus. Abdominal echo revealed no evidence of an abdominal aortic aneurysm.
Arteritis was also considered but the ESR was within the normal range for age and sex.
Several doctors have kindly responded to the case and include Professor Alan Lefor, Professor of Surgery, Jichi medical school, Japan gives a surgeons view of the history and physical followed by the kind Dr Masami Matsumura of Kanazawa University School of Medicine with an indepth and excellent break down of the case into its essential elements. Thanks to both these esteemed doctors for their opinions here.
Professor Alan Lefor:
To me, a case of acute paralysis and anal sphincter loss of tone, is an acute spinal cord problem such as trauma (no history here), spinal cord ischemia (like a dissection, but in the absence of pain might not be as high on the list, although she does have hypertension) or spinal cord compression (like a tumor, hematoma).
My workup would start with a CT scan to evaluate the aorta and spinal canal looking for dissection, aneurysm, and spinal canal mass.
Rx for a dissection would be htn control.
Dr Masami Matsumura:Questions
1) From history and examination, list the problems with this patient.
Problem list:
#1 Numbness of the buttocks
#2 Rapid onset lower limb weakness, then paralysis of the lower limbs
#3 Reduced muscle tone
#4 Reflexes knee (L2-4) absent, ankle (S1) absent
#5 Light touch- decreased in lower limbs up to the umbilicus (Th10) region
#6 Vibratory sense (posterior column) was intact on the pelvic brim (S3) and right knee (L4) but absent further down
#7 Nociception and thermoreception were absent.
#8 Reduced anal tone (S2-4)
#9 Bowel and bladder disturbance.
#10 Absence of joint position sense (posterior column)
#11 Hypertension
#12 Smoking history
#13 Aorta mildly expansile
Assessment:
At the beginning, this patient is 70 years old woman who has history of HTN and smoking. I would use following system for thinking differential diagnosis in view of weakness. I would differentiate neoplastic or degenerative diseases first.
Vascular: possible, infarction of spinal cord, arterio-venous malformation
Infection: less likely,spinal epidural abscess,Lyme disease, syphilis
Neoplastic: possible, vertebral or spinal cord tumor, metastasis ofbreast cancer, lung cancer,gastrointestinal cancers,or renal cell carcinoma, non-Hodgkin's lymphoma, plasmacytoma, multiple myeloma, orparaneoplastic syndrome
Collagen: possible, sarcoidosis, polymyositis, vasculitis, Guillan-Barre syndrome, SLE (very rare)
Toxic/Metabolic: less likely, periodic paralysis, hypokalemia
Trauma/Degenerative: possible,intervertebral disk disease or spinal stenosis,
Congenital: less likely
Iatrogenic: less likely
Idiopathic: less likely,amyloidosis
Cauda equina syndrome is likely in this case from problem #1 to #10.
#1 Numbness of the buttocks
#2 Rapid onset lower limb weakness, then paralysis of the lower limbs
#3 Reduced muscle tone
#4 Reflexes knee (L2-4) absent, ankle (S1) absent
#5 Light touch- decreased in lower limbs up to the umbilicus (Th10) region
#6 Vibratory sense was intact on the pelvic brim (S3) and right knee (L4) but absent further down
#7 Nociception and thermoreception were absent.
#8 Reduced anal tone (S2-4)
#9 Bowel and bladder disturbance
#10 Absence of joint position sense (posterior column)
Cauda equina syndrome is included in concept of epidural spinal cord compression (ESCC).
UpToDate 16.1 describes as follows:
Neopalastic ESCC is a common complication of cancer that causes pain and sometimes irreversible loss of neurologic function. In adults, the tip of the spinal cord usually lies at the L1 vertebral level; below this level, the lumbosacral nerve roots form the cauda equina, which floats in cerebrospinal fluid. With cauda equina lesions, the weakness is associated with depressed deep tendon reflexes in the legs.
My first question is as follows:
Can we interpret problem #5 Light touch- decreased in lower limbs up to the umbilicus (Th10) region as a finding of cauda equina syndrome?
UpToDate 16.1 describes as follows:
When a spinal sensory level is present, it is typically one to five levels below the actual level of cord compression. Saddle sensory loss is commonly present in cauda equina lesions, while lesions above the cauda equina frequently result in sparing of sacral dermatomes to pinprick.
Cauda equina syndrome can cause decreased light touch in lower limbs up to the umbilicus (Th10) region.
My second question is as follows:
Why she doesn't have back pain?
UpToDate 16.1 describes as follows:
Pain is usually the first symptom of ESCC, being present in 83 to 95 percent of patients at the time of diagnosis.
This patient didn’t complain back pain. But 5 to 17 percent of ESCC cases don’t disclose back pain.
Third my question is as follows:
What is the cause of her cauda equina syndrome?
UpToDate 16.1 describes as follows:
The three most common are prostate cancer, breast cancer, and lung cancer, each of which accounts for about 20 percent of cases.
This patient is woman and smoker.Vertebral or spinal cord tumor, metastasis of breast cancer, lung cancer, gastrointestinal cancers, or renal cell carcinoma, non-Hodgkin's lymphoma, plasmacytoma, or multiple myeloma must be differentiated.
Problem #13 Aorta mildly expansile is probably caused by #11 Hypertension.
Questions
2) What immediate test or tests would you perform and why?
MR imaging
UpToDate 16.1 describes as follows:
MR imaging offers several potential advantages over myelography.
It produces anatomically faithful images of the spinal cord and intramedullary pathology and is even more sensitive than radionuclide bone scans at identifying bony metastases. It can image the entire thecal sac regardless of whether a spinal subarachnoid block is present. It is not contraindicated with large brain metastases, thrombocytopenia, or coagulopathy. It spares the patient a lumbar puncture.
Questions
3) What anatomical problem is this from the history and physical examination?
From the history and physical examination, cauda equina syndrome is likely. Neoplastic disease or degenerative disease including spinal canal stenosis might be the cause of L1 or L2 vertebral level compression.
Questions
4) What is the current evidence based treatment for such a condition?
Administration of corticosteroids is first strategy. Next surgical management with anterior decompression should be considered. If diagnosis is neplastic, radiotherapy or chemotherapy should be planned.
UpToDate 16.1 describes as follows:
Management include the administration ofcorticosteroids(dexamethasone ), followed either by surgery and/or radiotherapy (RT). Aggressive surgical management with anterior decompression appears to give better results than RT alone in patients who are candidates for surgical intervention. Compared to RT alone, surgery followed by RT is associated with better neurologic outcomes. Chemotherapy may be beneficial in patients with chemosensitive tumors.
Interestingly, both Dr Matsumura and Professor Lefor listed spinal cord ischaemia / infarction as part of their differential diagnoses from the history and physical examinations. This is the most important thing.
The case by itself without the advanced imaging studies is very difficult to make an accurate diagnosis and in this case, MRI did not show an obvious mass lesion or even the usual cord infarction pattern. However, with the history of rapid onset, paralysis and sensory loss affecting specific sensory modalities, and a non-diagnostic MRI scan, spinal cord infarction was still considered the most likely.
This was later confirmed by a most excellent neurologist.
Summary
In summary, spinal cord ischaemia is rare accounting for <1% of all strokes. Most spinal cord ishaemia occurs due to abdominal surgery. The spinal cord is supplied by one anterior spinal artery and two posterior spinal arteries. Superiorly the vessels originate from the vertebral arteries and they receive feeding radicular arteries from many levels with many coming from the intercostal arteries.
The Adamkiewicz artery is most commonly affected. Studies have shown that the most probable cause of infarcts is related to mechanical stress affecting the radicular arteries which can extend for several levels. Vertebral body infarcts can also occur and are associated with cord infarction and can be another sign of radicular artery occlusion.
There is an association of spinal cord ischaemia and spinal disease and in such chronic compressive diseases there is impairment of blood flow which can impair collateral blood supplies putting at risk the compensatory mechanisms in the event of ischaemia.
Transient ischaemic attacks affecting the spinal cord can also occur.
Clinical Features Associated with Various Types of Spinal Cord Ischaemia
Anterior spinal artery territory- bilateral motor impairment with spinothalamic impairment
Posterior Spinal artery territory- bilateral motor impairment with dorsal column sensory impairment
Complete [transverse] infarct- Bilateral motor impairment with total sensory loss at and below the area of the ischaemia.
Unilateral infarcts produce a hemiparesis, in the case of anterior impairment, a contralateral spinothalamic deficit and with a posterior impairment, a unilateral dorsal column impairment.
[please review the neuroanatomy of the various sensory pathways-- spinothalamic neurones cross over at the level of entry and traverse to the contralateral side and ascend in the lateral spinothalamic tracts whereas the dorsal column input ascends directly from the site of entry and cross over in the brainstem region. The routes of these two pathways explain why there is a difference in the sensory impairment within the spinal cord and different findings on physical examination].
There are no clear guidelines for treatment as there have been no prospective clinical studies and various anti-thrombotic / anti-coagulation measures have been tried in previous cases. In my opinion, a prospective study seems unlikely to be able provide a clear answer because of the paucity of such strokes.
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