Cerebral Microbleeds and Intracerebral Hemorrhage in CADASIL

Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most common genetic cause of stroke. In addition to ischemic stroke, CADASIL predisposes to development of cerebral microbleeds (CMB).

CMB are well-defined MRI-demonstrable brain lesions consisting of tiny perivascular hemosiderin deposits, detectable using T2*-weighted gradient echo or susceptibility-weighted imaging (SWI). CMB are associated with hypertension, cerebral amyloid angiopathy (CAA), and chronic kidney disease. CMB are also associated with intracerebral hemorrhage (ICH), which accounts for 20–30% of stroke in Asian countries such as Korea and Japan. Hypertension and CAA are the most common causes of ICH, and CAA is known to be associated with apolipoprotein E (ApoE)-ε4 genotype.

Intracerebral hemorrhage (ICH) has been described only sporadically for patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). However, cerebral microbleeds (CMBs) were found in 31% to 69% of the patients with CADASIL, and this predicted an increased risk of ICH. In this study, the authors found that 25% of the symptomatic patients with CADASIL had ICHs, and their development was closely related to the number of CMBs.

Reference:

Front. Neurol., 15 May 2017 | https://doi.org/10.3389/fneur.2017.00203

Recurrent Lumbar Disc Herniation.

Recurrent lumbar disc herniation is defined as disc herniation at a previously operated disc level, regardless of ipsilateral or contralateral herniation, in patients who experienced a pain-free interval of at least 6 months after surgery.

The highest revision rate 42% occurred at level L4–L5, followed by level L5–S1 in 25.0%.

There is significant association between disc degeneration assessed with the Pfirrmann disc degeneration grade and recurrent herniated lumbar disc (HLD) revision.

Cinotti et al. reported that, in severe degenerative disc patients, the annulus fibrosus showed a markedly low recovery rate after primary lumbar discectomy (LD), and the attenuated portion of the annulus fibrosus would aggravate recurrent HLD.

However, Dora et al. reviewed the relationship between disc degeneration assessed with five grading systems and HLD recurrence. They reported that patients with relatively low-grade disc degeneration had a greater risk of recurrence. However, they included 60 non-randomized patients into each of the study and control groups. The correlation between age and Pfirrmann grade was found to be significant by Okada et al.

Although various factors contribute to the failure of disc surgery, recurrent disc herniation remains the major source of disability. Recurrent lumbar disc herniation has been reported in widely varying incidences between 3% and 18% of the patients and depends on the duration of the follow-up. Recurrent disc herniation is a significant problem, as scar formation may lead to increased morbidity after traditional posterior reoperation. Furthermore, persistent low back pain or re-recurrent sciatica may develop in some cases after repeated surgery. Also, it is important to consider the possibility of iatrogenic instability during surgery on the lumbar spine for the treatment of recurrent disc herniation.

The optimal surgical technique for treating recurrent lumbar disc herniation is controversial. Some authors believe that in the absence of objective evidence of spinal instability, recurrent lumbar disc herniation may be adequately treated by repeated laminectomy and discectomy alone. While others believe that various factors contribute to the failure of repeated lumbar disc surgery and discectomy alone without fusion remains the major source of disability.

Fusion with repeated lumbar discectomy can be broadly categorized as posterolateral fusion (PLF) and interbody fusion. Various techniques for interbody fusion have been described, including anterior lumbar interbody fusion (ALIF), posterior lumbar interbody fusion (PLIF), and transforaminal lumbar interbody fusion (TLIF).

Revision discectomy is effective in patients with recurrent lumbar disc herniation with satisfactory rate up to 88.9%. Fusion with revision discectomy improves the postoperative low back pain, decreases the intraoperative risk of dural tear or neural damage and decreases the postoperative incidence of mechanical instability or re-recurrence. TLIF and PLF have comparable results when used with revision discectomy, but PLF has significantly less total cost than TLIF.

case 5

References:

10.4103/1793-5482.121685

10.3340/jkns.2019.0085

Acromegaly secondary to pituitary macroadenoma; Craniofacial changes.

Growth hormone cell adenomas cause gigantism in children and acromegaly in adults. The most common cause of acromegaly in adults is pituitary adenoma.

Skull in acromegaly demonstrates calvarial thickening, frontal bossing, enlarged paranasal sinuses and enlarged sella turcica. The mandible also enlarges resulting in prognathism and increased gaps between the teeth.

Reference:

(PMID: 9352815)

Persistent trigeminal artery:  Saltzman type 2

Saltzman first described the angiographic appearance of PTA and its classification into three types according to angiographic appearance.

The Saltzman type I PTA connects the basilar artery at the level between superior cerebellar artery (SCA) and AICA. The proximal basilar artery and posterior communicating artery are usually hypoplastic and subsequently both posterior cerebral arteries and SCAs are supplied through the PTA.

The Saltzman type II PTA connects the basilar artery above the origin of the SCAs. The posterior communicating arteries are present and provide the posterior cerebral arteries.

The Saltzman type III PTA is considered a combination of types I and II. Ali et al. reviewed Saltzman classification and included variations of PTA in type III, in which there is no connection of the basilar artery. The variations of PTA type III arise from the internal carotid and terminate directly as the SCA (type IIIa), AICA (type IIIb), and PICA (type IIIc) without interposition of the basilar artery.

In one study of 4.650 patients that underwent brain MRA, the prevalence of each type using the Saltzman classification was as follows: type I, 24%; type II, 16%; type III, 60%

Many authors state in their discussion that persistent trigeminal, hypoglossal, and proatlantal arteries have been associated with aneurysms distant from the persistent vessels. Such an association is dubious. The prevalence of aneurysms associated with persistent trigeminal artery is approximately 3%, which is similar to the prevalence of aneurysms in the general population.

Reference:

Chen YC, Li MH, Chen SW, Hu DJ, Qiao RH. Incidental findings of persistent primitive trigeminal artery on 3-dimensional time-of-flight magnetic resonance angiography at 3.0 T: an analysis of 25 cases. J Neuroimaging. 2011;21(2):152–8.

Cloft HJ, Razack N, Kallmes DF. Prevalence of cerebral aneurysms in patients with persistent primitive trigeminal artery. J Neurorsurg 1999;90:865–867

10.1177/1591019919863110

https://doi.org/10.3348/jksr.2016.75.4.309

Middle Cerebral Artery Duplication Type A.

A duplicated middle cerebral artery (DMCA) is an anomalous vessel arising from the internal carotid artery (ICA). The origin of the DMCA lies between the anterior choroidal artery and the distal end of the ICA. Although there are several explanations of DMCA, its embryological origin is still an open question. Komiyama et al. proposed that the development of DMCA involves the anomalously early ramification of the early branches of the middle cerebral artery (MCA), based on their similarity to the cortical supply by the early branches of the MCA. Yamamoto et al. suggested that DMCA is a variant of the normal branching of the MCA. Kai et al. reported two types of DMCA : one with a course parallel to that of the main MCA (type A) and the other coursing toward the temporal lobe (type B).

DMCAs themselves have no clinical significance. However, rare aneurysms have been reported at the origin of the DMCA. It is unclear whether this association is a chance occurrence or is related by an unknown mechanism. Kai et al. reported that all aneurysms associated with DMCAs were found at the origins of type B DMCAs. They insisted that type B DMCAs can be expected to be subject to higher hemodynamic stress and that this is a factor in the development of aneurysms on the type B DMCA.

Reference:

 Kai Y, Hamada J, Morioka M, Yano S, Kudo M, Kuratsu J. Treatment of unruptured duplicated middle cerebral artery aneurysm : case report. Surg Neurol. 2006;65:190–193. discussion 193

10.3340/jkns.2011.49.2.102

Adult Tethered Cord Syndrome- asymptomatic patients with an elongated cord and a thick filum

Diagnosis and treatment of ATCS are a challenging task because of its rarity, and its presentation mimics a wide spectrum of pathologies. The clinical features of ATCS are widely recognized as nonspecific, including diffuse and nondermatomal back and leg pain. Other common symptoms include bladder dysfunction, skin abnormalities, scoliosis, and decreased motor and sensory function.

Tethered cord syndrome is a stretch-induced functional disorder of the spinal cord with its caudal part anchored by an inelastic structure. Garceau first described the “filum terminale syndrome” in 1953 in 3 patients. Yamada and others broadened the stretch-induced functional disorder to patients with other anomalies in 1981. Myelomeningocele, lipoma, lipomyelomeningocele, diastematomyelia, meningocele manqué, and dermoid sinus were included in this category but only when parallelism between the oxidative metabolic changes and neurologic status exists.

Symptoms related to a congenital tethered cord occur most commonly in childhood, so it was initially regarded as a pediatric problem; but in many patients, the diagnosis is not established until symptoms manifest in adulthood.

Three categories of TCS were recently described by Yamada and colleagues;

The first category includes lumbosacral cord anchored by an inelastic filum.

The second category includes caudal myelomeningoceles and many sacral myelomeningoceles.

The third category is divided into 2 groups. The first group includes patients with paraplegia and lipomyelomeningocele and myelomeningocele who apparently have no functional lumbosacral neurons. No neurologic benefit is expected from surgery in this group. The second group includes The second group includes asymptomatic patients with an elongated cord and a thick filum.

The incidence of occult spinal dysraphism is unknown, and although it is likely that some patients remain asymptomatic and a diagnosis is never made, a subset of patients with the congenital syndrome develops progressive symptoms and signs in adulthood.

Reference:

 10.1080/10790268.2008.11760722

https://doi.org/10.1155/2015/926185

Solitary Lytic Lesion of the Skull.

Calvarial lesions are uncommon and may constitute a diagnostic challenge. Pertinent clinical information such as patient age and history of trauma or underlying systemic disease should always be taken into consideration.

Among all imaging features, the extent, multiplicity, and attenuation of a calvarial lesion, in combination with knowledge of the patient’s age, are enough to narrow the differential diagnosis-

Recognition of benign and malignant imaging features is important for the radiological diagnosis. In general, benign tumours have well-defined borders with a narrow transition zone; sclerotic margins are frequently present. On the other hand, malignant tumours have poorly defined margins, a wide transition zone, aggressive periosteal reaction and often have a soft tissue component; these lesions cause dramatic bony destruction with intracranial or extracranial extension. Skull lesions can be lytic or sclerotic, single or multiple with varied composition; they may arise from osteogenic, chondrogenic, fibrogenic, vascular and/or other elements of bone.

Reference:

https://doi.org/10.1148/rg.2021200198

https://doi.org/10.1007/s13244-018-0653-y

Lumbar Meningioma

Spinal meningiomas, which are characteristically intradural and extramedullary, are relatively rare and account for less than 10% of all meningiomas and 25% of all spinal cord tumors.

Spinal meningiomas are intradural extramedullary lesions, usually benign, that are most commonly thoracic and posterolateral in location. Accounting for ~25% of spinal tumors, they are the second most common tumor in the intradural extramedullary location, second only to tumors of the nerve sheath. Most occur in middle-aged or older adults, with female predominance

Clinically, manifestations in patients with a meningioma of the lumbar spine depend on the tumor location, in relation to the spinal cord transection and nerve roots. Neurological symptoms include back or radicular pain, motor weakness, sensory disturbances, and urinary or fecal incontinence as a late finding.

On MRI, characteristics of spinal meningiomas include well-circumscribed, broad-based dural attachment and/or a dural tail sign, which are similar signal characteristics to typical intracranial meningiomas. Spinal meningiomas typically demonstrate isointensity to slightly hypointensity on T1-weighted images, isointensity to slightly hyperintensity on T2-weighted images, and moderate homogeneous enhancement on T1-weighted images with gadolinium enhancement. Densely calcified meningiomas are sometimes hypointense on T1 and T2 images, and show only minimal contrast enhancement.

These radiological characteristics are usually nonspecific. It is difficult for physicians to differentiate benign or malignant tumors simply on images. The “dural tail sign”, for example, can be seen in intradural-extramedullary meningiomas but is also found in metastatic tumors and lymphomas.

A meningioma with intradural and extradural components occasionally mimics a nerve sheath tumor and a nerve sheath tumor with a predominant intradural component may mimic a meningioma; however, nerve sheath tumors are usually hyperintense on T2, lumbar and ventral in location, rarely calcified, more common in men, and do not have a dural tail

Reference:

Yeo Y, Park C, Lee JW, et al. Magnetic resonance imaging spectrum of spinal meningioma. Clin Imaging 2019; 55:100-106

doi: 10.1016/j.ejrad.2006.04.003

10.1080/10790268.2018.1564993

Acromegaly Axial Arthropathy.

Vertebral Enlargement

Acromegaly is a chronic and slowly developing disease caused by hypersecretion of GH and consequently of IGF-I.

The spine, noticeably targeted by chronic GH excess, presents widened intervertebral spaces, vertebral enlargement, and osteophyte formation, which are caused by endocondral, marginal, and subligamentous growth of vertebral bone.

The hyperostotic changes may be so relevant as to resemble to those observed during the diffuse idiopathic skeletal hyperostosis (DISH) syndrome.

Reference:

https://doi.org/10.1210/jc.2003-031283

Drusen optic disc

Drusen are a rare inherited pathology , usually bilateral and asymptomatic, but they can lead to a visual field defect or to migraine-like headaches.

Drusen are classified into two categories: retinal and optic disc.

A benign calcification at the optic nerve head junction affects 0.25%-2.0% of the population and is more prevalent in White people. The condition is a result of acellular calcific deposition within the optic nerve that may mimic papilledema on fundoscopic exam, thus representing a diagnostic challenge for clinicians. 

Reference:

Kumaev B, Soule E, Rao D, Fiester P.  Optic Disc Drusen.  Appl Radiol.  2020;49(6):54-55.

DOI: 10.1594/EURORAD/CASE.1595