Multiple Brain and a Spinal Cavernous Malformation in same patient

A 40 yo male presented with sudden onset paraplegia. 

MRI Spine done first revealed an intra medullary focal lesion with T1 bright meth Hb staining and low signal intensity hemosiderin staining on GRE. 

Brain screening done in same setting revealed multiple similar intra cranial lesions. 

MRI brain and spine images of same patient.

Description of findings:
MRI Brain shows multiple intra axial focal lesions with typical pop corn ball appearance on T2w images with low signal intensity hemosiderin rim. Few T1 bright locules of meth Hb - a sub acute stage blood degradation product. Lesion show low signal intensity blooming on T2*GRE. No mass effect.
MRI Spine shows a similar intra medullary focal lesion at the level of C7, marked adjacent cord odema.

Diagnosis: Multiple Brain and a Spinal Cavernous Malformation.

Cavernoma (Cavernous Malformation)

A benign vascular hamartoma, composed of closely packed immature blood vessels with intra lesional micro hemorrhages, without any neuronal tissue.
Best diagnostic clue is "Pop corn ball" like appearance with hemosiderin rim on T2w images.
Occur anywhere in brain. Rare in Spinal cord.
Vary in size from few mms to cms. Average size is between 1 to 5cm.
Usually single solitary, may be multiple, discrete lesions.
MRI is more sensitive and specific for detection of lesion.
Hyper dense on non contrast CT. Calcification seen in ~ 50% cases. No to faint enhancement on post contrast.
On MRI lobulated appearance due to multiple locules which show variable signals, depending up on the blood degradation products. T1 bright locules attributed to Meth Hb - a sub acute stage blood degradation product. Low signal intensity hemosiderin rim on T2 images. Low signal intensity blooming on  T2*GRE images. No to faint enhancement on post contrast T1 images.
No to mild peri lesional odema.
No mass effect.

Clinical presentation:
Seizures 50%
Neuro deficit 25%
Asymptomatic 20%, detected as an incidnetal finding.
No intervention is a rule. But need follow up imaging as it may show progression or regression in size. Rarely show massive bleed.
Can affect any age group.
No gender preponderance.
Familial association in Hispanic Americans - Multiple Cavernoma Syndrome, carries higher risk for bleed and re bleed.

Similar cases: 

Cavernoma

cavernoma-with-bleed

Intraventricular-cavernoma

venous angioma with bleed

Antiphospholipid Antibody Syndrome MR Angio Brain

A 30 yo female with migraine like symptoms. Not responding to treatment. 

Findings:
3 D TOF Non contrast MR Angiography of Brain shows sparsity of cortical branches of left MCA compared to left.
2 D TOF Non contrast MR Angiography of Neck normal.
Brain FLAIR and Diffusion images unremarkable apart from few ischemic lesions in left parietal peri ventricular white matter.

Further evaluation : positive for Antiphospholipid Antibody. 

Antiphospholipid antibody syndrome

Syn: Antiphospholipid syndrome, APS, APLS, Hughes syndrome.
An autoimmune, hypercoagulable state caused by antibodies against cell-membrane phospholipids that provokes blood clots (thrombosis) in both arteries and veins as well as pregnancy-related complications such as miscarriage, stillbirth, preterm delivery, or severe preeclampsia. There are also associations between antiphospholipid antibodies and headaches, migraines, and oscillopsia. Some studies have shown the presence of antiphospholipid antibodies in the blood and spinal fluid of patients with psychological symptoms.
There is autoimmune production of antibodies against phospholipid (aPL), a cell membrane substance cardiolipin (anti-cardiolipin antibodies) and β2 glycoprotein I.
The term "primary antiphospholipid syndrome" is used when APS occurs in the absence of any other related disease.
APS however also occurs in the context of other autoimmune diseases, such as systemic lupus erythematosus (SLE), in which case the term "secondary antiphospholipid syndrome" is used.
In rare cases, APS leads to rapid organ failure due to generalised thrombosis; this is termed "catastrophic antiphospholipid syndrome" (CAPS) and is associated with a high risk of death.
Antiphospholipid syndrome is diagnosed with blood tests.
Requires treatment with anticoagulant medication such as heparin to reduce the risk of further episodes of thrombosis and improve the prognosis of pregnancy. Warfarin/Coumadin is not used during pregnancy because it can cross the placenta, unlike heparin, and is teratogenic.

Persistent Trigeminal artery MR Angio

Arterial communications between the carotid and vertebrobasilar systems in the fetus may occasionally persist in the adult.
A primitive trigeminal artery is the most cephalad and common of these persistent fetal anastomoses.
Arises from the presellar ICA extends posteriorly to join basilar artery usually between the origins of the superior and anterior inferior cerebellar arteries. The anomalous vessel usually has a parasellar course.
Direction of flow in the artery is usually from the ICA to the basilar.
Discovered incidentally.
Coexisting other intracranial vascular abnormalities in ~ 25% of patients are intracranial aneurysms, arteriovenous malformations, carotid-cavernous fistulae, and moyamoya.

'The tau sign' the configuration resembling the Greek letter 'T' formed by the joining of the proximal portion of the trigeminal artery to presellar portion of ICA as it turns from a vertical to a horizontal course

In this case an associated hypo plastic basilar noted.

Hemangioblastoma MRI

Axial T2w image show a cystic posterior fossa mass with an intense homogeneously enhancing eccentric mural nodule near right sigmoid sulcus.

Radiological and histopathological diagnosis : Hemangioblastoma.

Hemangioblastoma
A highly vascular tumor.
An intra axial posterior fossa mass with cyst and an enhancing mural nodule is a diagnostic clue.
Currently classified as meningeal tumor of uncertain histogenesis (WHO, 2000)
Locaion:
90% posterior fossa (m/c) in that 80% cerebellar hemispheres, 15% Vermis, 5% in other places  fourth ventricle, medulla.
10% Supratentorium.
In ~ 60% of cases mass present as cyst + mural nodule and in ~ 40% of cases only as a solid nodule.
Imaging:
Cyst is clear, density on CT and signal intensity on MRI same as that of Csf, non enhancing thin imperceptible wall.
Mural nodule on CT may be iso to hyper dense, intense and homogenous enhancement. On MRI hypo to iso intense on T1, hyperintense on T2 and FLAIR. May see flow voids within the nodule with adjacent vascular feeders on T2w images, intense and homogenous enhancement on T1 images implies to its highly vascular nature. May show low signal intensity hemosiderin staining on GRE if associated to with any bleed.

Presentation is usually with headache, dysequilibrium, dizziness may be due to its mass effect and hydrocephalus.
Age : for sporadic: 40-60 yr and for familial : can occur at younger age. Slight male predominance.

Closest DD is Pilocytic Astrocytoma; mural nodule show mild to moderate enhancement not this intense and homogeneous, not characterized by flow voids and feeders. Seen in relatively younger age group.

Similar case:
http://www.neuroradiologycases.com/2012/11/hemangioblastoma-mri.html

Ulnar Nerve Abscess – Leprosy

A 40 year known HIV positive female presented with diffuse swelling of left arm around elbow painful for last 15 days, it was initially pain less with an associated numbness of little finger since 6 months, numbness and swelling was increasing gradually. On examination a nodular swelling behind the elbow on the medial aspect with tenderness. Limited elbow extension. No any skin discoloration. 

MRI elbow joint advised with preference to ulnar nerve. 

MRI shows marked focal nodular enlargement of ulnar nerve at elbow joint, thickening of rest of the ulnar nerve with marked inflammation on STIR sequence in an adjacent muscle compartments and facial planes. 

Imaging wise possibility of Ulnar neuritis / Abscess was given and advised to rule out Leprosy clinically. 

Incision and drainage of the abscess done followed by culture and sensitivity test. Histopathology report mentions caseating epitheloid granuloma and giant cells. Disrupted native nerve fibers within and around the granuloma. Acid fast bacilli in singles and clusters consistent with tuberculoid Hansen’s disease.

Imaging and histopathological diagnosis : Ulnar nerve Abscess – Leprosy. 

Discussion: 

Leprosy is a chronic granulomatous infection, caused by mycobacterium leprae.

Primarily affecting the peripheral nerve trunks and cutaneous nerves.

Classically presents with neural or dermal signs and symptoms. 

Ulnar nerve is most commonly involved in fact Leprosy is the only disease in which abscess develop in nerve trunk. These abscesses are usually chronic cold abscess. 

Leprosy has a high prevalence in India of about 5 per 10000 populations with about 70% of globally recorded cases. The leprosy bacilli have a characteristic feature of nerve involvement, involvement of testis, lymph nodes, spleen, liver, larynx, bone marrow.

Contribution of imaging is limited in leprosy but reorganization of the condition is important by radiologist as management is different as clinically and imaging wise other dd often include nerve sheath tumor. 

Broadly there are three types of leprosy, the tuberculoid, lepromatous and borderline. The differentiation is based on symptoms, bacterial load and individual’s immune response.

In Tuberculoid leprosy the primarily affected nerves are pressure/trauma dependent  The most commonly involved nerve is ulnar nerve followed by median nerve, sural nerve, radial and branches of facial nerve.

In lepromatous leprosy the nerve damage is widespread and symmetrical with extensive intracutaneous nerve involvement and resembles symmetric polyneuropathy. Sensory loss occurs in the coolest areas of body like dorsum of hand and feet, ear, dorsum of fore arm and anterolateral parts of leg.

Borderline leprosy has characteristic of both tuberculoid and lepromatous.

Management is often surgical after failure of steroid treatment and that is Epineurotomy by multiple longitudinal incisions and external decompression to relive the internal pressure through out the involved segment. 

Neurocysticercosis starry sky appearance

Multiple round cystic focal lesions with an eccentric scolex typical of Neurocysticercosis.
Lesions of various stages noted.
Few of them show mild perilesional odema on FLAIR, punctate low signal intensity of calcification of scolex on GRE and thin ring enhancement on post contrast T1. 

Neurocysticercosis (NCC) 


Intracranial parasitic infection caused by the pork tapeworm, Taenia solium.
Four pathologic stages: Vesicular, colloidal vesicular, granular nodular, nodular calcified.

Diagnostic clue is Cyst with "dot" inside.
Most common location is convexity subarachnoid spaces. May involve cisterns> parenchyma> ventricles. Basal cistern cysts may be racemose (grape-like). Parenchymal cysts often hemispheric, at gray-white junction. Intraventricular cysts are often isolated. Fourth ventricle is most common intra ventricular location. Rare locations are Sella, orbit, spinal cord.

Size of Cysts variable, typically 1 cm, range from 5-20 mm and contain a scolex; scolex 1-4 mm. Subarachnoid cysts may be larger, up to 9 cm reported. Cysts are often rounded or ovoid cyst, solitary in 20-50%. When multiple, usually small number of cysts, disseminated form ("miliary" NCe) is rare.

Imaging varies with developmental stage of cyst and host immune response. Lesions may be at different stages in same patient.
MR Findings
• TlWI
o Vesicular stage: Cystic lesion isointense to CSF. May see discrete, eccentric scolex (hyperintense)
o Colloidal vesicular stage: Cyst is mildly hyperintense to CSF.
o Granular nodular stage: Thickened, retracted cyst wall; edema decreases.
o Nodular calcified stage: Shrunken, Ca++ lesion.
o Useful to detect intraventricular cysts.
• T2WI
o Vesicular stage: Cystic lesion isointense to CSF. May see discrete, eccentric scolex. No surrounding edema.
o Colloidal vesicular stage: Cyst is hyperintense to CSF. Surrounding edema, mild to marked.
o Granular nodular stage: Thickened, retracted cyst wall; edema decreases
o Nodular calcified stage: Shrunken, Ca++ lesion
• FLAIR
o Vesicular stage: Cystic lesion isointense to CSF. May see discrete, eccentric scolex (hyperintense to
CSF); no edema.
o Colloidal vesicular stage: Cyst is hyperintense to CSF. Surrounding edema, mild to marked. Useful to detect intraventricular cysts (hyperintense).
• T2* GRE: Useful to demonstrate calcified scolex.
• DWI: Cystic lesion typically isointense to CSF
• TI C+
o Vesicular stage: No enhancement typical, may see mild enhancement.  May see discrete, eccentric scolex enhancement.
o Colloidal vesicular stage: Thick cyst wall enhances. Enhancing marginal nodule (scolex)
o Granular nodular stage: Thickened, retracted cyst wall; may have nodular or ring-enhancement
o Nodular calcified stage: Small calcified lesion, rare minimal enhancement

In children, may see "encephalitic cysticercosis" with multiple small enhancing lesions and diffuse edema. Intraventricular cysts may cause ventriculitis and/or hydrocephalus.
Cisternal NCC may appear racemose (multilobulated, grape-like), typically lacks scolex
E/o Complications: Meningitis, hydrocephalus, vasculitis


NCC is the most common cause of acquired epilepsy in developing countries like india.
It can present variably depending on the location and stage of cysts in the nervous system and the host immune response. The most common presentation of parenchymal NCC is seizures that are usually focal and brief. Status epilepticus occurs in some cases. About a third of cases have headache and vomiting.

Although treatment with cysticidal therapy continues to be debated, there is increasing evidence that it helps through increased and faster resolution of CT lesions; whether there is any improvement in long-term seizure control needs further study. It should not be used in cysticercus encephalitis or in ophthalmic NCC and used with caution in extraparenchymal NCC. It is of no use in calcified lesions.
Corticosteroids are used simultaneously to reduce cerebral oedema.
Seizures respond well to a single antiepileptic, and the seizure recurrence rate is low in cases with single lesions. Those with multiple, persistent or calcified lesions usually have recurrent seizures.
Extraparenchymal NCC is often associated with intracranial hypertension, hydrocephalous and chronic meningitis; it has a guarded prognosis; surgical intervention is required in many cases.
Management of NCC needs to be individualized. NCC is potentially eradicable; proper sanitation, hygiene and animal husbandry are warranted.

Holoprocencephaly

Holoprocencephaly with an associated fused metopic suture /single frontal "plate" of bone. 

Formerly called arrhinencephaly. 

Best diagnostic clue is Monoventricle + fused (uncleaved) frontal lobes ~ absent anterior midline falx/fissures and Anteriorly displaced Sylvian fissures. 

Associated with Hypotelorism, fused metopic suture /single frontal "plate" of bone, Azygous / absent anterior cerebral artery (ACA) on MR Angio.

Metopic Synostosis

'Trigonocephaly' Metopic Synostosis

Note the associated excessive convolutional marking on inner table of skull with dilated parietal emissary foramen indicating long term raised intra cranial pressure.

Craniosynostosis
Craniosynostosis (from cranio, cranium; + syn, together; + ostosis relating to bone) is a condition in which one or more of the fibroussutures in an infant skull prematurely fuses by ossification.  thereby changing the growth pattern of the skull. Because the skull cannot expand perpendicular to the fused suture, it compensates by growing more in the direction parallel to the closed sutures. Sometimes the resulting growth pattern provides the necessary space for the growing brain, but results in an abnormal head shape and abnormal facial features. In cases in which the compensation does not effectively provide enough space for the growing brain, craniosynostosis results in increased intracranial pressure leading possibly to visual impairment, sleeping impairment, eating difficulties, or an impairment of mental development combined with a significant reduction in IQ.

Craniosynostosis occurs in one in 2000 births. Craniosynostosis is part of a syndrome in 15 to 40% of the patients, but it usually occurs as an isolated condition.

Causes of premature fusion
The dura mater plays an important role in determining closure or patency of the suture.
Many things are still not understood about the suture biology and the exact causative pathways remain yet to be completely understood.
Multiple potential causes of premature suture closure have been identified, such as the several genetic mutations that are associated with syndromic craniosynostosis.The cause of nonsyndromic craniosynostosis however, is still greatly unknown. Most likely, a role is played by biomechanical factors, as well as environmental, hormonal and genetical factors.
Biomechanical factors: fetal head constraint during pregnancy.
Environmental factors : maternal smoking and the maternal exposure to amine-containing drugs likely through effects on fibroblast growth factor receptor genes.
Hormonal factors: Hyperthyroid induced craniosynostosis is a hormone mediated premature closure due to high levels of thyroid hormone.
Genetic factors : fibroblast growth factor receptor 3 (FGFR3) and TWIST genes.

Scaphocephaly
The name providing a direct hint regarding the deformity of the skull. The literal meaning of the Greek derived word ‘scaphocephaly’ is boathead. A synonymous term is 'dolichocephaly' (the prefix dolicho- means elongated).
Premature sagittal suture closure restricts growth in a perpendicular plane, thus the head will not grow sideways and remain narrow.
This is best seen in a view standing above the child looking downwards at the top of the head.
A prominent forehead, called frontal bossing, and a prominent back portion of the head, called coning. When viewed from sideways the resulting shape of the head will look a bit like a boat.

Trigonocephaly
A result from the premature closure of the metopic suture result in a narrow forehead, which is even further emphasized by ridging of the suture. The resulting shape can best be assessed from a top view again, which will reveal a somewhat triangular form of the head.
Trigonocephaly is also a Greek derived word, which can be translated as triangular shaped head. A facial feature of metopic synostosis is hypotelorism.

Plagiocephaly
The Greek word plagios means skew. Plagiocephaly can be sub classified in Anterior Plagiocephaly and Posterior Plagiocephaly.
Anterior Plagiocephaly
Anterior plagiocephaly is a clinical description of unilateral coronal synostosis. Children born with unilateral coronal synostosis develop due to compensatory mechanisms a skew head; a plagiocephaly.
The sagittal suture ‘divides’ the coronal suture in two halves; unilateral meaning that either the right side or the left side to the sagittal suture is fused. This fact immediately raises an important point. Unlike closure of the sagittal or the metopic suture, right and left are not the same in unilateral coronal synostosis. This asymmetry shows in the skull deformity, as well as in the facial deformity and the complications.
This time, the skull deformity can only partly be predicted using Virchow’s law. Growth is arrested in the plane perpendicular to the fused suture and the forehead is flattened, but only at the ipsilateral side of the head. Ipsilateral indicates the same side of the head as where the suture is closed. Compensatory growth occurs in a parallel plane, as well as in a perpendicular plane. An increase in growth at the metopic and the sagittal suture accounts for the parallel plane and will result in bulging at the temporal fossa and an increase in width of the skull. Compensatory growth in the perpendicular plane occurs on the side of the head with the patent coronal suture, the contralateral side. Half of the forehead will bulge forwards as a result.
Assessment of the skull from a top view shows asymmetry of the frontal bones, an increased width of the skull and a forward displacement of the ear at the ipsilateral side of the head.Assessment of the skull from a frontal view will show asymmetrical features of the face, including a displacement of the chin point of the jaw and a deviation of the tip of the nose.The chin point is located more to the contralateral side of the head, due to the ipsilateral forward displacement of the temporomandibular joint together with the ear.The tip of the nose will also point towards the contralateral side.Complications based on the skull deformation include malocclusion of the jaw and in as many as 90% - a subtle form of - strabismus, the last being caused by the asymmetrical placement of the orbits.
Posterior Plagiocephaly
Unilateral lambdoid synostosis is also called posterior plagiocephaly, indicating that this gives, just like unilateral coronal synostosis, a ‘skew head’. The difference is that this time, the deformity mostly shows at the occiput.
Restriction of growth will occur at the ipsilateral side of the head; compensatory growth will occur at the contralateral side of the head. This growth pattern exerts an effect at the base of the skull, which is not even when the child is assessed from a point of view standing behind the child, as well as on the cervical spine, which shows a curvature. In addition, an asymmetry of the ears can be seen, with the ear on the ipsilateral side placed further to the back. Also, again from a point of view standing behind the child, a bulging of the mastoid can be seen. Minimal forehead asymmetries are typically seen.

Brachycephaly
Brachycephaly, or a ‘short head’, is the result of a closure of both the coronal sutures result in a child’s head with a restriction of growth in the forward direction and in the backward direction; recessed frontal bones and a flattened occiput. compensatory growth will occur sideways, due to the sagittal suture, and upwards, due to the lambdoid sutures.

Oxycephaly
Oxycephaly, also known as turricephaly and high-head syndrome, is a type of cephalic disorder. This is a term sometimes used to describe the premature closure of the coronal suture plus any other suture, like the lambdoid,

Pansynostosis
The word pansynostosis is also Greek derived and can be translated as ‘all one bone’, indicating that all of the sutures are closed. The term is used to describe the children with three or more sutures closed. Pansynostosis can present in several ways. The appearance can be the same as that seen with primary microcephaly: a markedly small head, but with normal proportions.

Sinken skin flap syndrome

Syn : Sunken skin flap syndrome, Trephine Syndrome , Syndrome of Trephined skull,Post hemicraniectomy Paradoxical Herniation, 

Sinken skin flap syndrome 

Sinken skin flap syndrome   

An uncommon late post operative complication usually occurs after a month of surgery.

Patient who undergo hemi craniectomy for reasons like malignant MCA Infarction, hemispheric contusion or sub dural hematoma present with new onset of complaints like headaches, seizures, dizziness,easy fatiguability and mood changes. 

On examination often a depressed skin flap that is sunken appearance of the skin flap noted.

On CT / MR imaging a depressed galeal dural complex at the craniectomy site and concave deformity of the underlying brain parenchyma.

Incidence: 
In a study of 108 patients who underwent decompressive craniectomy, trephine syndrome was reported in 13% and occurred between 28 and 188 days after surgery (reference : Yang XF, Wen L, Shen F, et al. Surgical complications secondary to decompressive craniectomy in patients with a head injury: a series of 108 consecutive cases. Acta Neurochir Wien) 2008;150(12): 1241–1247;discussion 1248)

Patholophysiology: Exposure of the intracranial contents to atmospheric pressure, which alters CSF hydrodynamics, deforms the brain, and reduces cerebral perfusion. (Reference: Akins PT, Guppy KH  Sinking skin flaps, paradoxical herniation, and external brain tamponade: a review of decompressive craniectomy management.Neurocrit Care 2008;9(2):269–276) 

Management : Some patients may show clinical improvement after the cranial defect repaired with cranioplasty the reason may be improved cerebral blood flow after cranioplasty. 

Paradoxical Herniation

Hemicraniectomy with Paradoxical herniation.
Note the VP shunt tube in situ. 

A very uncommon and further advanced complication of decompressive craniectomy and that is paradoxical internal herniations. Seen in patients with a large craniectomy defect who then undergo CSF drainage by either lumbar puncture or ventriculoperitoneal shunt result in marked decrease in Csf pressure, which leads to reduction in intracranial pressure making intra cranial content vulnerable to atmospheric pressure. This pressure imbalance particularly the negative intara cranial pressure deforms brain and mid line. 

On CT / MR Imaging a significant mid line shift away from the craniectomy side with subfalcine and or transtentorial herniations. Uncal herniation if severe may results in mid brain compression.

Clinically patient present with depressed level of consciousness, autonomic instability, signs of brainstem release, and focal neurologic deficits.

Management: Paradoxical herniation is a neurosurgical emergency and urgent treatment is necessary in order to increase intracranial pressure, to stop any CSF leakage, and restore the continuity of the calvaria.

Options include urgent placing the patient in Trendelenburg position and head inclined towards to craniectomy side, clamping ventricular shunts or drains, administering intravenous fluid. Cranioplasty as soon as possible. Paradoxical herniation also has been reported to be effectively and quickly reversed with a lumbar epidural blood patch or clamping ventricular shunt tube. 

Retrodental Cervical Synovial Cyst MRI

A 30 yo male presented with short history spastic quadriparesis and sensory loss. 

No history of trauma.

No any known caugulopathy.

RA factor negative. 

On admission MRI Cervical spine:

Findings : 

An extra medullary cyst iso intense to Csf causing significant cord compression.

No obvious extension out of adjacent neural foramen.

No any bony spinal canal remodelling.

No any adjacent osseous involvement or altered marrow signals. 

No any associated abnormal soft tissue. 

Cyst non enhancing on post contrast. Normal enhancing epidural venous plexus noted.

Imaging wise possibility given was Arachnoid cyst.

Near complete excision of cyst done by left side posterior laminectomy, appeared extra dural on table. Histopathological diagnosis : Synovial Cyst.

Retrodental Cervical Synovial Cyst

Spinal synovial cysts are generally found in the lower lumbar spine. Synovial cysts of the cervical spine are
extremely rare. Retro dental / atlanto axial synovial cyst to the best of my knowledge, only thirty-five cases have been documented in the literature.
Often there is an associated CV junction bony abnormalies like hypoplastic dense, Atalnto dental instblity or os odontoideum is reported.
In our case hypo plastic dense with possible atlantoaxial instability appears to be the cause of excess
stress might have produced the synovial cyst.

Presenting symptoms CV Junction usually include signs of cervical myelopathy secondary to cord compression. Treatment is surgical to decompress cord and stabilizing CV junction.