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Numbness in arms and legs? What's wrong with this young man~ Yimaitong compiled and compiled, please do not reprint without authorization.
Case brief introduction The patient is a 40-year-old male who has "pins and numbness" in his left hand.
The symptoms of his left arm against the wall or neck extension will be aggravated.
In the next 2 months, the numbness progressed from the left arm to the right arm, and then to the upper chest area on both sides and the back thigh area on both sides.
The patient denied pain, weakness, fatigue, cognitive changes, visual changes, dysarthria or dysphagia, and changes in bladder/intestinal function.
The past history is hyperlipidemia (orally treated with atorvastatin 20 mg); there is no family history of neurological diseases or autoimmune diseases.
Vital signs and general physical examination were normal; system examination showed no obvious abnormalities; neurological examination showed no obvious abnormalities in consciousness and cranial nerve examination; gait, coordination and other motor examinations were normal; both arms, front chest and back of thighs Light touch in some areas is weakened, vibration, acupuncture and temperature are retained; Romberg’s sign is negative; deep tendon reflex is normal; bilateral plantar reactions are flexion.
Aquaporin-4 IgG was negative, and no abnormalities were found in infection, metabolism and hematological tests.
MRI showed extensive longitudinal transverse myelitis (LETM, in which T2 hyperintensity extends ≥ 3 vertebrae) (figure).
CSF examination showed normal cell number, normal glucose, elevated total protein (84 mg/dL), normal IgG index and zero oligoclonal band.
Figure MRI manifestations of myelitis before and after treatment.
(A and B) Sagittal and axial T2-weighted images, C4-C7 bilateral central gray matter and dorsal white matter showed high signal changes.
(C and D) T1 enhanced images show enhanced dorsal part of the lesion, suggesting that the spinal cord may be involved (arrow).
(EH) Re-examination of MRI 2 months after oral prednisone showed that the C4-C7 hyperintensity lesions were close to disappearing, and the dorsal enhancement persisted, which may indicate active granuloma.
Differential diagnosis The onset and progression of this patient were subacute.
The differential diagnosis of partial myelopathy includes structural (compressive), inflammatory, metabolic, toxic, infectious, paraneoplastic, vascular (especially spinal dural arteriovenous fistula) and malignant causes.
Inherited causes are usually more insidious.
For patients with acute onset myelopathy, imaging or cerebrospinal fluid confirms spinal cord inflammation, but when there is no evidence of a specific cause, it can be defined as idiopathic acute transverse myelitis, which usually reaches the peak of the disease in 4-21 days.
This case showed subacute partial myelitis.
A key clinical feature of this patient is that despite extensive longitudinal spinal cord lesions, it lacks early clinical dysfunction, so it tends to be neurosarcoidosis, which is different from the pathophysiology of neuromyelitis optica (NMOSD) (which usually leads to extensive tissue destruction).
Sexual disease, and there is obvious early dysfunction).
LETM is very rare in MS and is a characteristic manifestation of NMOSD, but it can also be seen in other inflammatory myelitis, especially neurosarcoidosis.
Compared with NMOSD, the enhancement of submental gadolinium on the back is a characteristic of neurosarcoidosis myelitis, and the ring enhancement is more related to NMOSD.
The "trident sign" refers to central canal enhancement with dorsal subcutaneous enhancement, which is seen in neurosarcoidosis myelitis, but also in other CNS infections (including granulomatous infection) and lymphoma.
The peripheral sensory loss pattern that does not conform to the cortical distribution supports CNS pathology.
The continuous expansion from the arm to the chest to the legs, but the face is not involved, indicates cervical spinal cord disease below the level of the trigeminal nucleus of the spinal cord.
The syndrome points to partial cervical spinal cord disease, affecting only one of the three spinal cord pathways (sensation, especially the dorsal column, not movement or bowel/bladder), rather than true transverse myelopathy.
Out of suspicion of neurosarcoidosis, the patient underwent an enhanced CT examination of the chest, and the results showed bilateral hilar and mediastinal calcified lymph nodes and peripheral pulmonary nodules, consistent with pulmonary sarcoidosis.
The needle biopsy showed that the non-necrotizing granuloma was consistent with sarcoidosis, and there was no evidence of infection or malignancy.
The final diagnosis was possible neurosarcoidosis, which manifested as partial longitudinal and extensive transverse cervical myelitis, and biopsy confirmed pulmonary sarcoidosis.
Discussion Neurosarcoidosis is seen in about 5%-15% of patients with sarcoidosis.
It can manifest as leptomeningitis, meningoencephalitis, dura materitis, optic neuropathy, other cranial neuropathy, hypothalamic/pituitary involvement, myelitis, or nerves Root inflammation and other combinations.
Sarcoidosis is a typical multi-system disease.
About 10%-20% of neurosarcoidosis only affects the CNS.
On MRI, the parenchymal involvement of the spinal cord in neurosarcoidosis can be longitudinally extensive, smaller segments or multifocal.
In addition to the pattern of enhancement seen in this patient, enhancement may appear in the central canal, nerve roots, meninges, or other areas affected by neurosarcoidosis.
Neurosarcoid lesions can manifest as persistent T1 enhancement for several months or years, even after treatment.
Inflammatory demyelination of MS and NMO usually resolves within 1-2 months.
The patient’s chest CT showed bilateral hilar and mediastinal calcified lymph nodes and peripheral pulmonary nodules, consistent with pulmonary sarcoidosis.
If the CT is negative, the whole body FDG-PET has diagnostic value.
It can find lymph nodes that are metabolically active but still normal in size.
These lymph nodes can be used as the target of biopsy.
In addition, skin and eye biopsies are also helpful for diagnosis.
Angiotensin-converting enzyme (ACE) level detection can be used for the diagnostic evaluation of sarcoidosis, but it is a non-specific marker.
Compared with patients without sarcoidosis, patients with sarcoidosis (especially active pulmonary sarcoidosis) have higher serum ACE levels, with a sensitivity of 29%-60% and a specificity of about 89%.
In cerebrospinal fluid, the sensitivity and specificity of ACE to neurosarcoidosis are 24%-55% and 94%, respectively.
In short, normal ACE should not rule out neurosarcoidosis.
Elevated ACE may also be non-specific because it can be seen in other inflammations, infections, malignant or metabolic diseases.
Sarcoidosis inflammation is characterized by non-caseating (non-necrotic) granulomas, which contain monocytes and macrophages, T lymphocytes, B lymphocytes, and fibroblasts, among other cell types.
Granulomas in the central nervous system tend to form around blood vessels.
The granulomatous inflammation of sarcoidosis is mainly mediated by T cells, usually Th1, but there are also studies suggesting that Th17 is also involved.
Common cytokines involved in sarcoidosis signal transduction include IFNγ, TNFα, and various interleukins and chemokines.
Environmental and infectious exposure are possible reasons for susceptibility to sarcoidosis, but further studies are still needed to confirm.
The genetic susceptibility of sarcoidosis is related to specific human leukocyte antigen alleles, thus supporting autoimmune etiology.
For the diagnosis of neurosarcoidosis, the latest consensus diagnostic criteria was published in 2018.
Among them, the diagnosis of "clear" neurosarcoidosis requires CNS biopsy support, clinically prompts sarcoidosis and strictly excludes other causes; "probable" neurosarcoidosis, the biopsy evidence is from non-CNS biopsy results.
Sarcoidosis; cases where sarcoidosis is suspected but not confirmed by a biopsy are classified as "probable" neurosarcoidosis.
At present, there is still a lack of randomized controlled trials for the treatment of CNS neurosarcoidosis.
Although glucocorticoids are effective for most patients with neurosarcoidosis, the dose required to achieve or maintain remission may be higher due to the toxicity of glucocorticoids.
Immunosuppressants commonly used in clinical practice include methotrexate, azathioprine, mycophenolate mofetil, leflunomide, hydroxychloroquine, and infliximab.
In a retrospective analysis, infliximab, a TNFα inhibitor, can benefit patients, including refractory patients.
Taking into account the adverse effects of glucocorticoids, the patient chose infliximab plus weekly oral methotrexate, and gradually reduced glucocorticoids within 4 months.
The symptoms gradually disappeared without functional limitation. Re-examination of MRI at 7-month and 12-month follow-up showed complete remission of the lesion.
Original index: Romeo AR, Lisak RP, Meltzer E, Fox EJ, Melamed E, Lucas A, FreemanL, Frohman TC, Costello K, Zamvil SS, Frohman EM, Gelfand JM.
A young man withnumbness in arms and legs: From the National Multiple Sclerosis Society CaseConference Proceedings.
Neurol Neuroimmunol Neuroinflamm.
2018 Oct23;5(6):e509.
doi: 10.
1212/NXI.
0000000000000509.
eCollection 2018 Nov.
Case brief introduction The patient is a 40-year-old male who has "pins and numbness" in his left hand.
The symptoms of his left arm against the wall or neck extension will be aggravated.
In the next 2 months, the numbness progressed from the left arm to the right arm, and then to the upper chest area on both sides and the back thigh area on both sides.
The patient denied pain, weakness, fatigue, cognitive changes, visual changes, dysarthria or dysphagia, and changes in bladder/intestinal function.
The past history is hyperlipidemia (orally treated with atorvastatin 20 mg); there is no family history of neurological diseases or autoimmune diseases.
Vital signs and general physical examination were normal; system examination showed no obvious abnormalities; neurological examination showed no obvious abnormalities in consciousness and cranial nerve examination; gait, coordination and other motor examinations were normal; both arms, front chest and back of thighs Light touch in some areas is weakened, vibration, acupuncture and temperature are retained; Romberg’s sign is negative; deep tendon reflex is normal; bilateral plantar reactions are flexion.
Aquaporin-4 IgG was negative, and no abnormalities were found in infection, metabolism and hematological tests.
MRI showed extensive longitudinal transverse myelitis (LETM, in which T2 hyperintensity extends ≥ 3 vertebrae) (figure).
CSF examination showed normal cell number, normal glucose, elevated total protein (84 mg/dL), normal IgG index and zero oligoclonal band.
Figure MRI manifestations of myelitis before and after treatment.
(A and B) Sagittal and axial T2-weighted images, C4-C7 bilateral central gray matter and dorsal white matter showed high signal changes.
(C and D) T1 enhanced images show enhanced dorsal part of the lesion, suggesting that the spinal cord may be involved (arrow).
(EH) Re-examination of MRI 2 months after oral prednisone showed that the C4-C7 hyperintensity lesions were close to disappearing, and the dorsal enhancement persisted, which may indicate active granuloma.
Differential diagnosis The onset and progression of this patient were subacute.
The differential diagnosis of partial myelopathy includes structural (compressive), inflammatory, metabolic, toxic, infectious, paraneoplastic, vascular (especially spinal dural arteriovenous fistula) and malignant causes.
Inherited causes are usually more insidious.
For patients with acute onset myelopathy, imaging or cerebrospinal fluid confirms spinal cord inflammation, but when there is no evidence of a specific cause, it can be defined as idiopathic acute transverse myelitis, which usually reaches the peak of the disease in 4-21 days.
This case showed subacute partial myelitis.
A key clinical feature of this patient is that despite extensive longitudinal spinal cord lesions, it lacks early clinical dysfunction, so it tends to be neurosarcoidosis, which is different from the pathophysiology of neuromyelitis optica (NMOSD) (which usually leads to extensive tissue destruction).
Sexual disease, and there is obvious early dysfunction).
LETM is very rare in MS and is a characteristic manifestation of NMOSD, but it can also be seen in other inflammatory myelitis, especially neurosarcoidosis.
Compared with NMOSD, the enhancement of submental gadolinium on the back is a characteristic of neurosarcoidosis myelitis, and the ring enhancement is more related to NMOSD.
The "trident sign" refers to central canal enhancement with dorsal subcutaneous enhancement, which is seen in neurosarcoidosis myelitis, but also in other CNS infections (including granulomatous infection) and lymphoma.
The peripheral sensory loss pattern that does not conform to the cortical distribution supports CNS pathology.
The continuous expansion from the arm to the chest to the legs, but the face is not involved, indicates cervical spinal cord disease below the level of the trigeminal nucleus of the spinal cord.
The syndrome points to partial cervical spinal cord disease, affecting only one of the three spinal cord pathways (sensation, especially the dorsal column, not movement or bowel/bladder), rather than true transverse myelopathy.
Out of suspicion of neurosarcoidosis, the patient underwent an enhanced CT examination of the chest, and the results showed bilateral hilar and mediastinal calcified lymph nodes and peripheral pulmonary nodules, consistent with pulmonary sarcoidosis.
The needle biopsy showed that the non-necrotizing granuloma was consistent with sarcoidosis, and there was no evidence of infection or malignancy.
The final diagnosis was possible neurosarcoidosis, which manifested as partial longitudinal and extensive transverse cervical myelitis, and biopsy confirmed pulmonary sarcoidosis.
Discussion Neurosarcoidosis is seen in about 5%-15% of patients with sarcoidosis.
It can manifest as leptomeningitis, meningoencephalitis, dura materitis, optic neuropathy, other cranial neuropathy, hypothalamic/pituitary involvement, myelitis, or nerves Root inflammation and other combinations.
Sarcoidosis is a typical multi-system disease.
About 10%-20% of neurosarcoidosis only affects the CNS.
On MRI, the parenchymal involvement of the spinal cord in neurosarcoidosis can be longitudinally extensive, smaller segments or multifocal.
In addition to the pattern of enhancement seen in this patient, enhancement may appear in the central canal, nerve roots, meninges, or other areas affected by neurosarcoidosis.
Neurosarcoid lesions can manifest as persistent T1 enhancement for several months or years, even after treatment.
Inflammatory demyelination of MS and NMO usually resolves within 1-2 months.
The patient’s chest CT showed bilateral hilar and mediastinal calcified lymph nodes and peripheral pulmonary nodules, consistent with pulmonary sarcoidosis.
If the CT is negative, the whole body FDG-PET has diagnostic value.
It can find lymph nodes that are metabolically active but still normal in size.
These lymph nodes can be used as the target of biopsy.
In addition, skin and eye biopsies are also helpful for diagnosis.
Angiotensin-converting enzyme (ACE) level detection can be used for the diagnostic evaluation of sarcoidosis, but it is a non-specific marker.
Compared with patients without sarcoidosis, patients with sarcoidosis (especially active pulmonary sarcoidosis) have higher serum ACE levels, with a sensitivity of 29%-60% and a specificity of about 89%.
In cerebrospinal fluid, the sensitivity and specificity of ACE to neurosarcoidosis are 24%-55% and 94%, respectively.
In short, normal ACE should not rule out neurosarcoidosis.
Elevated ACE may also be non-specific because it can be seen in other inflammations, infections, malignant or metabolic diseases.
Sarcoidosis inflammation is characterized by non-caseating (non-necrotic) granulomas, which contain monocytes and macrophages, T lymphocytes, B lymphocytes, and fibroblasts, among other cell types.
Granulomas in the central nervous system tend to form around blood vessels.
The granulomatous inflammation of sarcoidosis is mainly mediated by T cells, usually Th1, but there are also studies suggesting that Th17 is also involved.
Common cytokines involved in sarcoidosis signal transduction include IFNγ, TNFα, and various interleukins and chemokines.
Environmental and infectious exposure are possible reasons for susceptibility to sarcoidosis, but further studies are still needed to confirm.
The genetic susceptibility of sarcoidosis is related to specific human leukocyte antigen alleles, thus supporting autoimmune etiology.
For the diagnosis of neurosarcoidosis, the latest consensus diagnostic criteria was published in 2018.
Among them, the diagnosis of "clear" neurosarcoidosis requires CNS biopsy support, clinically prompts sarcoidosis and strictly excludes other causes; "probable" neurosarcoidosis, the biopsy evidence is from non-CNS biopsy results.
Sarcoidosis; cases where sarcoidosis is suspected but not confirmed by a biopsy are classified as "probable" neurosarcoidosis.
At present, there is still a lack of randomized controlled trials for the treatment of CNS neurosarcoidosis.
Although glucocorticoids are effective for most patients with neurosarcoidosis, the dose required to achieve or maintain remission may be higher due to the toxicity of glucocorticoids.
Immunosuppressants commonly used in clinical practice include methotrexate, azathioprine, mycophenolate mofetil, leflunomide, hydroxychloroquine, and infliximab.
In a retrospective analysis, infliximab, a TNFα inhibitor, can benefit patients, including refractory patients.
Taking into account the adverse effects of glucocorticoids, the patient chose infliximab plus weekly oral methotrexate, and gradually reduced glucocorticoids within 4 months.
The symptoms gradually disappeared without functional limitation. Re-examination of MRI at 7-month and 12-month follow-up showed complete remission of the lesion.
Original index: Romeo AR, Lisak RP, Meltzer E, Fox EJ, Melamed E, Lucas A, FreemanL, Frohman TC, Costello K, Zamvil SS, Frohman EM, Gelfand JM.
A young man withnumbness in arms and legs: From the National Multiple Sclerosis Society CaseConference Proceedings.
Neurol Neuroimmunol Neuroinflamm.
2018 Oct23;5(6):e509.
doi: 10.
1212/NXI.
0000000000000509.
eCollection 2018 Nov.
