Can you see why this patient has muscular pain and swelling?

This 6-year-old has a history of generalized muscular pain and swelling. The erythrocyte sedimentation rate is 90. You are shown seven images that include a coronal T1 (image 1), a coronal T2 with fat suppression (images 2, 5), an axial T2 without fat suppression (images 3, 6), and an axial proton-density SPIR or fat-suppressed proton-density (images 4, 7). Images 5, 6, and 7 are duplicates of images 2, 3 and 4, but with arrows added for key findings. How much can you see in the first four images?

Q1 – What is your diagnosis?

Q2 – If a diagnosis were not yet made, what test would be on your blood draw aside from the sedimentation rate?

Q3 – What sporadic disease is often confused with this one, and does not respond to treatment for months to years?

T1W TSE Coronal

STIR long TE

T2 TSE Axial

PDW SPIR Axial

STIR long TE

T2 TSE Axial

PDW SPIR Axial

 

A1 – Polymyositis.

A2 – Creatine kinase (CK); Creatine phosphokinase (CPK) or lactate dehydrogenase LDH might be part of your blood draw. Patients that are especially sick with this condition can develop muscle necrosis and myoglobinuria.

A3 – Sporadic inclusion body myositis (SIBM) is often confused with, and misdiagnosed as, polymyositis or dermatomyositis. It progresses more slowly, and does not respond to steroid treatment.

The hallmark of polymyositis is weakness and loss of muscle mass, proximally. The ability to contract the neck and torso are also affected. Often affected are large muscle groups such as the hip extensors which leads to difficulty climbing stairs or rising from  a seated position. Patients tend to experience muscle pain.

Pure polymyositis is not associated with skin lesions. However, other symptoms include abnormalities of esophageal motility or dysphagia, low grade fever, peripheral adenopathy, interstitial lung disease, and cardiac disease in severe systemic cases. When presenting in adulthood, fatigue while walking is a common complaint.

Polymyositis is an inflammatory myopathy mediated by cytotoxic T-cells. In contrast, dermatomyositis is humerally mediated, and is considered an angiopathy. Either one of these conditions may present as a manifestation of a paraneoplastic syndrome.

Inclusion body myositis, which is less well-known to imagers and clinicians, is an inflammatory muscle disease that is progressive, yet slow, but involves both distal and proximal muscles. It is most apparent in the arms and legs. Inclusion body myositis is usually sporadic, but there is a hereditary form. Sporadic inclusion body myositis is the most common cause of inflammatory myopathy in patients over the age of 50 years.

MRI is extremely valuable in assessing the presence of the disease itself since there are not very many conditions that produce this pattern of patchy large muscle group myoedema. Furthermore, the greatest value of MRI is in demonstrating a response to treatment with steroids or chemotherapy which manifests as diminishing myoedema. Such an assessment may be made volumetrically, both subjectively and measured objectively. To name a few, the heavily water-weighted sequences, especially the proton-density fat suppression and T2 fat suppression (e.g. SPAIR, SPIR, special, fat-sat, STIR) are fat suppression sequences. Please note that the T1-weighted images provide little value in assessing the presence of myoedema (image 1).

Pearl: With myopathy in an adult, always exclude adverse reaction to statins.

 

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For more case review, check out MRI Online.

 

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Can you make the five major observations in this anemia case?

This 13-year-old presents with hemolytic anemia. Look at the first three images. Images 1 and 2 are coronal T2-weighted images, while image 3 is a coronal 3D volumetric image from magnetic resonance cholangiopancreatography (MRCP). See how many observations you can make. There are five major ones. Also, think about what a plausible cause might be for this duct narrowing in a 13-year-old. Images 4-6 are duplicates of the first three images, but with arrows for key findings where applicable.

Coronal T2

Coronal T2

MRCP

Coronal T2

Coronal T2

MRCP

 

The five observations are:

  • Hepatomegaly with low liver signal consistent with iron overload.
  • Splenomegaly.
  • Hypointensity or hypointense mass within the gallbladder (yellow arrow, image 5).
  • Narrowing of the common hepatic duct (pink arrow, image 6).
  • Ascites (green arrows, image 6).

Now that we’ve made those five key observations, I’d like to take a deep dive into the cause of this patient’s stricture, one of the longest explanations we’ve had in our case review series. If you’re pressed for time, I’d encourage you to bookmark this page and come back later. If you’ve got a few moments, let’s learn together.

The most plausible explanation for the patient’s duct narrowing is a stricture. Such a stricture in a 13-year-old with no surgical history or trauma other than the images provided most likely would be related to passage of a common duct stone or possibly Mirizzi syndrome. Since a globular mass exists in the gallbladder, it is plausible that this mass consists of a conglomeration of stones, one of which migrated from the cystic duct or eroded the adjacent common hepatic duct causing this stricture. Mirizzi syndrome is defined as hepatic duct obstruction (partial or complete) caused by extrinsic compression from an impacted stone in the cystic duct or Hartmann’s pouch of the gallbladder. While a stone is not currently identified, a passed stone from Mirizzi syndrome could easily account for the findings seen. The obstruction can occur at either the common bile duct or common hepatic duct level.

Mirizzi syndrome is more common in underdeveloped countries, particularly in Latin America. Essentially, an inflammatory phenomenon due to a pressure ulcer caused by an impacted gallstone at the gallbladder infundibulum causes external obstruction of the bile duct.

Such an erosion may result in a cholecystocholedochal or cholecystohepatic fistula. Pablo Luis Mirizzi, a surgeon from Argentina, originally described the syndrome. Mirizzi syndrome has been subdvided into various types (four types to be exact). The two major types include type 1, where there is external compression of the bile duct by a large stone or stones impacted in the cystic duct or Hartmann’s pouch and type 2, which consists of a proper cholecystobiliary fistula caused by gallstone(s) that have eroded directly into the bile duct. If the stone passes, and / or the ulcers heal, then a stricture may evolve.

Hemochromatosis or iron overload refers to iron in the body from any cause. Hereditary hemochromatosis (HHC) is a genetic disorder, while acquired iron overload occurs from transfusional iron overload.

Genetic forms of hemochromatosis are more common in people of Celtic (Irish, Scottish, Welsh, Cornish, British, etc.), English and Scandinavian origin. The mutation is the C282Y mutation on the HFA gene associated with HLA-A3. People with hemochromatosis manifest a very “black liver” on all MR sequences, including the ones displayed here. Whether it is a T1-weighted image, T2-weighted image, and especially on gradient-echo images (not shown), the liver is inordinately hypointense. If the overload is substantial, patients can develop secondary splenomegaly, liver failure and ascites. Our patient has all of these signs.

A second mutation has recently been discovered, H63D. Hemochromatosis is extremely rare in African-Americans, Asian-Americans, Hispanics, Latinos and Native Americans.

Hemochromatosis is associated with liver cirrhosis, bronzed diabetes, involvement of the heart with arrhythmias and cardiomyopathy, arthritis and erectile dysfunction. However, liver damage is the most notable, important and recognized sequela.

The two most important blood tests in making this diagnosis are the transferrin saturation test and serum ferritin test.

The transferrin saturation test shows how much iron is bound to the protein that carries iron in the blood. Transferrin saturation values equal to or greater than 45 percent are abnormal.

The serum ferritin test is more commonly used. Ferritin is a protein that stores iron in the blood. Levels above 300 mcg/L in men and 200 mcg/L in women are considered abnormal. Levels above 1000 mcg/L in men or women are most likely associated with clinically significant iron overload and organ damage. MR can be used to measure the degree of liver iron overload and quantify it. We will discuss this at a later date.

For more case review, head to MRI Online.

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Tinnitus and left facial droop and hearing loss (oh my)

This is a 57-year-old female with external ear vesicles, tinnitus, left facial droop and hearing loss. Have a look at the first two images without arrows, and see if you can answer the following questions. Afterwards, have a look at the duplicate images with arrows which highlight the key findings.

T1 Coronal HD POST

T1 Axial HD POST

 

Q1 – The most likely diagnosis is:

(a) Bell’s palsy
(b) Lyme disease
(c) Sarcoidosis
(d) Ramsay-Hunt syndrome
(e) Atypical mycobacterial infection

Q2 – Bell’s palsy typically affects cranial nerve(s):

(a) V (five)
(b) VII, V & VIII (seven, five and eight)
(c) VIII (eight)
(d) IX (nine)
(e) VII (seven)

Q3 – The most likely organism responsible in this case is:

(a) Borrelia Burgdorfii
(b) Herpes simplex
(c) Herpes Zoster
(d) Mycobacterium avium intracellulare
(e) Echovirus 7

T1 Coronal HD POST

T1 Axial HD POST

 

A1 – (d) Ramsay-Hunt syndrome
A2 – (e) VII (seven)
A3 – (c) Herpes Zoster

Ramsay-Hunt syndrome or herpes zoster oticus is caused by varicella zoster infection involving the sensory fibers of cranial nerves 7, 8, and a portion of the external ear supplied by the auriculotemporal nerve.  Pathologic enhancement of multiple nerves both within the IAC fundus and outside the fundus are tip offs to the diagnosis along with external ear vesicles!

The top differential diagnosis is Bell’s Palsy which does not have vesicles, does not involve as many cranial nerves, and typically restricts itself to the 7th. Other differential diagnoses include meningitis with meningeal symptoms, sarcoid with multiple bizarre lesions, and meningeal carcinomatosis which is not localized to the cranial nerves.  Involvement of the membranous labyrinth, as well as all portions of the facial nerve and other cranial nerves, are important tip offs to the diagnosis of Ramsay Hunt syndrome.

Pearls regarding Ramsay Hunt syndrome:

  1. Facial palsy alone with external ear vesicles is still considered part of the Ramsay Hunt syndrome.
  2. Facial paralysis is more severe than with Bell’s palsy.
  3. There is a deep burning pain in the ear.
  4. Sensorineural hearing loss, tinnitus and vertigo are common, but not with Bell’s palsy. The 5th cranial nerve may be involved specifically with the ophthalmic division, but not with Bell’s palsy.
  5. Vertigo develops after pain, but either before or after vesicular disruption.
  6. Ear pain is usually followed by the vesicles in about seven days.
  7. Cranial neuropathies occur within days after the ear pain, but when they occur before, the vesicles may be missed.
  8. The peak damage to the 7th nerve is at approximately 17 days compared with Bell’s palsy which peaks at 10 days.

For more case review, check out MRI Online.

References

1. Grose, C. et al:  Chickenpox and the Geniculate Ganglion: Facial Nerve Palsy, Ramsay-Hunt syndrome and acyclovir treatment.  Pediatric Infectious Disease Journal.  21 (7):  615 to 617, 2002.

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Don’t mix up the primary and secondary forms of this disorder

This 53-year-old male presents with popping and pain on top of the left shoulder with decreased range of motion. You are shown five images: a coronal T1 (image 1), coronal T2 (images 2, 4), and a sagittal T2 turbo spin echo SPAIR or fat-suppression sequence (images 3, 5). Images 4 and 5 are duplicates of images 2 and 3, but with arrows added for key findings. See what you can figure out just by looking at the first three images.

Q1 – What is the most likely diagnosis?

Q2 – What is your differential diagnosis?

Q3 – What is the important difference between the primary and secondary forms of this disorder?

Coronal T1

Coronal T2

Sagittal T2

Coronal T2

Sagittal T2

 

A1 – Primary synovial chondromatosis.

A2 – Differential diagnosis includes secondary synovial chondromatosis as a manifestation of osteoarthritis; donated chondral bodies from osteochondral defects; osseous bodies; and synovial hyperplasia.

Secondary synovial chondromatosis is usually associated with osteoarthritis and you would have been shown an arthritic joint. Chondral bodies are usually not this organized in their shape and size, namely elliptical and smooth. Further, you would have been shown the osteochondral donor site. Osseous bodies would be marrow containing or brighter on T1 and more hypointense on T2. Synovial hyperplasia is not quite so organized and elliptical in shape. The fronds of synovial hyperplasia are smaller. Furthermore, this is a 53-year-old male and synovial hyperplasias are associated with rheumatoid arthritis and seen in women. You would have also been shown other signs of rheumatoid arthritis.

Other unusual intraarticular hypointensities include amyloid (rare) which is not quite so organized in shape; gouty tophi also not so organized in shape; and localized PVNS (more hypointense on T2 and more hyperintense on T1).

A3 – It is important to distinguish primary from secondary synovial chondromatosis as the primary form can be associated with malignant degeneration while the secondary form has no association with increased incidence of malignancy.

For more shoulder case review, check out MRI Online.

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Why are there still problems post-Chiari decompression?

This 40-year-old female presents with Chiari malformation post decompression with occipital headaches. Why are there still problems? In this instance the foramen magnum has been decompressed. Focus on the first two images, then look at duplicates of the same images with arrows indicating key findings.

RAPID Sagittal T2 FLAIR

Axial T2 FSE

RAPID Sagittal T2 FLAIR

Axial T2 FSE

 

Causes for failure of Chiari decompression can include inadequate decompression or pseudomeningocele formation. Another less frequently appreciated cause of recurrent symptoms is the possibility of cerebellar ptosis. The decompression in this case goes above the equator of the cerebellum. This can result in inferior migration and ptosis of the cerebellum with reproduction of obstruction at the foramen magnum. In this instance, there is a subtle impression of the occipital bone on the cerebellum with effacement of the retrocerebellar cerebrospinal fluid spaces which can indicate the development of ptosis. Not only can this result in recurrent headaches, but it can also result in re-expansion of a syrinx if one was present or the development of one. This topic was addressed here.

For more case review, check out MRI Online.

Dr. Stephen Pomeranz

Dr. Malcolm Shupeck

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What can be learned from this 17-year-old’s knee injury?

This 17-year-old male reports with knee pain following a football injury. Be sure to size up the case by looking at the first two images without arrows before looking at the same images with arrows to indicate key findings.

What are the anatomical structures indicated by the arrows? Hint: While this patient’s anterior cruciate ligament (ACL) is intact, these structures are very important in the setting of acute ACL tear.

Sagittal T2 FSE

Sagittal T2 FSE

 

The green arrow on image 3 indicates the popliteus muscle (there is a mytotendinous strain in this case), and the red arrows on image 4 point to the arcuate ligament (there is an arcuate ligament tear with fluid leaking from the joint).

Popliteus muscle injuries seldom occur in isolation and are an important ancillary finding of internal derangement of the knee joint. Associated injuries include cruciate and collateral ligament injuries as well as meniscal tears and bone bruises. It is important not to overlook posterolateral corner injuries in the presence of an anterior cruciate ligament tear because the reconstructed cruciate ligament will likely fail without repair of the posterolateral corner injuries.

Sagittal T2 FSE

Sagittal T2 FSE

 

Bonus Question: What is an arcuate sign?

Answer: The arcuate sign is an avulsion fracture of the proximal fibula at the site of insertion of the arcuate ligament complex and is usually associated with cruciate ligament injury, though not in this case. The avulsion fracture is usually small, less than one centimeter, and involves the styloid process of the fibula.

For more knee case review, head to MRI Online. For further reading on the arcuate sign, click here.

 

Dr. Stephen Pomeranz

Dr. Robert Wissman

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I know it’s my Aunt Minnie because it looks like Aunt Minnie

This 65-year-old woman presents with limited range of motion which radiates down to the elbow. She felt pain in her shoulder after reaching backward in a car three weeks ago. What findings do you see on the two sagittal and two coronal images below?

Sagittal T2

Sagittal T2

Coronal PD Mid-glenoid

Coronal PD Anterior

The clinical history is typical, and the demographic is also classic (middle-aged female with decreased range of motion, worse with reaching backward in the dominant arm). This is an important cause of shoulder pain as the differential diagnosis includes labral and rotator cuff pathology. On the current study, note ill-defined intermediate smudgy T1 / PD (arrows, Image 3) signal in the superior labrum / axillary, inferior glenohumeral ligament (IGHL) recess and rotator interval. The rotator cuff (vertical arrow, image 4) is infiltrated by inflamatory tissue and appears “grey”. The green oblique arrows (images 2 and 4) represent secondary bursitis. Finally, a “corona sign” (e.g. high signal) surrounding the glenoid cup (arrows, image 1) is definitive. Our Aunt Minnie is the MR manifestation of clinical adhesive capsulitis.

For more case review, check out MRI Online.

Dr. Stephen Pomeranz

Dr. Nick Trane

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What type of tear is causing this 20-year-old athlete’s knee pain?

This is a 20-year-old college athlete who plays soccer, competing for a national championship in his senior year. He has anterolateral knee pain. You are shown two coronal PD SPIR images (image 1 and image 2), a sagittal T2 SPIR or fat-suppression image (image 3), and a sagittal T1 (image 4).

Q1 – How would you describe this tear (arrows)? Would you let him play?

Coronal PD SPIR

Coronal PD SPIR

Sagittal T2 SPIR

Sagittal T1

 

The tear is a horizontal tear (yellow arrows). While most horizontal or cleavage tears are chronic and degenerative in older adults, they occur traumatically. The tear (higher signal) is like the turkey on a sandwich with the meniscus (lower signal) sitting on either side like the pieces of bread.

Because there is no clear articular surface communication on any of the sequences, one might describe this as a “closed” traumatic tear, predominantly horizontal, of at least 2cm in length. One should carefully point out that the tear has precipitated active inflammation which manifest as swelling just anterior to the meniscal pathology, sagittal image 3 and 4 (anterior oblique green arrow).

Initial reaction to a tear that is so conspicuous in a young patient is to sit the patient down. However, given the fact that this patient is a senior in college, his college career is coming to an end, there is an important game coming forth, and the risk of splitting this meniscus into two or exacerbating the horizontal tear is very low in the absence of discoid meniscus, this patient was allowed to play. However, he did receive a steroid injection to diminish his pain. Another medication that is used in performance athletes, especially professional athletes in situations such as this, is Toradol. Finally, “closed” cleavage tears, as suggested above, are particularly common in discoid meniscus.

 

Check out MRI Online for more case review.

If you enjoyed this case, you may also enjoy:

What can be learned from this 17-year-old’s knee injury?
What type of tear is causing this 20-year-old athlete’s knee pain?
How should you approach this 24-year-old’s knee case?

 

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When finger pain and the patient’s stated history don’t add up

This 54-year-old male complains of chronic right index finger pain with no reported injury. Be sure to look at the three images without arrows before looking at the same images with arrows.

A. What has likely happened to this patient?

B. What does the red arrow on image 4 point to?

C. What does the green arrow on image 4 indicate?

D. What do the orange arrows on image 5 signify?

E. Finally, the pink arrows on image 6?

Sagittal T2

Axial T2 STIR

Coronal T2 STIR

Sagittal T2

Axial T2 STIR

Coronal T2 STIR

A. The patient dislocated his finger at the second metacarpophalangeal (MCP) joint resulting in significant capsular injury.

B. Volar subluxation of the second proximal phalanx.

C. Torn volar (palmar) plate proximally with disruption of the check rein ligaments.

D. Edema/partial tearing of the sagittal bands of the extensor hood.

E. Sprain of both the radial and ulnar collateral ligaments.

This is a complicated case which provides a good review of the MCP capsular anatomy, along with a healthy reminder to not be afraid to question the supplied history when the imaging findings are not concordant with that history. For more case review, check out MRI Online.

Dr. Stephen Pomeranz

Dr. David Downs

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Make sure to account for this thumb complication

A 48-year-old female presents complaining of thumb pain since a fall which occurred two weeks ago. Take a look at the first three images without arrows first, then look at the same three images with arrows.

A. What do you think is the cause of the patient’s pain?

B. What are the more common names for this condition?

C. What additional complication has occurred?

D. What is the significance of this complication?

Coronal PD Fat Sat

Coronal 3D Gradient

Coronal T1

Coronal PD Fat Sat

Coronal 3D Gradient

Coronal T1

A. Torn ulnar collateral ligament at the first metacarpophalangeal (MCP) joint.

B. Gamekeeper’s or skier’s thumb

C. Stener lesion. The torn ulnar collateral ligament is retracted proximally and superficially to the adductor aponeurosis (yellow arrows on images 4 and 5 points to adductor aponeurosis).The red arrows on image 4 and 5 point to the torn and contracted ulnar collateral ligament (UCL) of the first MCP joint. The orange arrow in image 6 points to the intact radial collateral ligament, while the pink arrow again makes note of the torn UCL.

D. The lesion requires surgery to heal properly. Since the UCL (red arrow) is prevented by the adductor aponeurosis (yellow arrow) from finding its way back to its attachment, and therefore healing, only surgical repair will do.

For more case review, visit MRI Online.

Dr. Stephen Pomeranz

Dr. David Downs

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