Specific pattern of gadolinium enhancement in spondylotic myelopathy

Flanagan, Eoin P.; Krecke, Karl N.; Marsh, Roger R.; Giannini, Caterina; Keegan, B. Mark; Weinshenker, Brian G.

doi:10.1002/ana.24184

Cited by 91 publications

(91 citation statements)

References 22 publications

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“…It is the commonest cause of nontraumatic myelopathy and occurs in a similar age group as APBD [16]. Although both may coexist, as in one of our patients, spondylotic myelopathy typically presents with numbness, paresthesias and cervical pain of subacute onset [16], often with radicular and asymmetric involvement of the arms, all undocumented in our patients. In addition, associated cerebral white matter lesions in 97 % in this cohort represent important red flag to prefer other etiology [16].…”

Section: Discussionsupporting

confidence: 60%

Frequent misdiagnosis of adult polyglucosan body disease

et al. 2015

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Adult polyglucosan body disease (APBD) is a rare glycogenosis manifesting progressive spastic paraparesis, sensorimotor polyneuropathy and neurogenic bladder. Misdiagnosis of APBD may lead to unnecessary investigations and to potentially harmful therapeutic interventions. To examine the frequency of misdiagnosis of APBD, we retrospectively reviewed the clinical data of 30 patients diagnosed between 1991 and 2013. Diagnosis was based on the combination of typical clinical and imaging findings, reduced glycogen branching enzyme activity, and the presence of p.Y326S GBE1 mutation. Initial symptoms started in the 5th-6th decade with bladder dysfunction (47 %), gait problems (33 %) or both. Diagnosis of APBD was delayed by 6.8 (±4.8) years. Consistent signs at diagnosis were spasticity in the legs (93 %), decreased or absent ankle reflexes (100 %), bilateral extensor plantar response (100 %) and distal sensory deficit (80 %). Nerve conduction study showed invariable sensorimotor polyneuropathy, and MRI demonstrated cervical spinal cord atrophy (100 %) and leukoencephalopathy (97 %). All 30 patients were initially misdiagnosed. Common misdiagnoses included cerebral small vessel disease (27 %), multiple sclerosis (17 %), amyotrophic lateral sclerosis (17 %) and peripheral neuropathies (20 %). Consequently, 27 % received inappropriate therapy. In addition, lower urinary tract symptoms in 60 % of men were attributed solely to prostatic disorders but did not respond to medical treatment or prostatectomy. These findings suggest that despite limited clinical variability, APBD is invariably misdiagnosed and patients are often mistreated. Physicians' unfamiliarity with the typical clinical and imaging features of APBD appears as the main reason for misdiagnosis.

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Section: Discussionsupporting

confidence: 60%

Frequent misdiagnosis of adult polyglucosan body disease

et al. 2015

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“…At times subacute cervical spondylotic myelopathy may pose a diagnostic problem [30]. Being associated with long toms of urinary retention developed in 91 % several days prior to any motor symptoms.…”

Section: D) "Longitudinally Extensive Transverse Myelitis (Letm)"mentioning

confidence: 99%

“…Although DWI is a helpful (but technical demanding) tool in spinal cord infarction [16], to describe the diagnostic (and prognostic) value of DWI in LETM remains as an important task [28,29]. fusiform hyperintense lesions on T2 WI and cord enlargement, 40 out of 56 patients (71 %) were initially diagnosed as inflammatory or neoplastic disease [30]. However, a transverse "pancake-like" enhancement just caudal to the site of maximal stenosis suggests that cord compression is responsible.…”

Section: D) "Longitudinally Extensive Transverse Myelitis (Letm)"mentioning

confidence: 99%

Differential Diagnosis of Acute Myelopathies: An Update

Nichtweiß¹,

Weidauer

2015

Clin Neuroradiol

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Appropriate description may lead to adequate diagnostic and therapeutic measures, and therefore, a simple scheme to categorize and term the imaging findings of acute myelopathy is suggested based on current literature. Assigning imaging findings to five groups, that is (a) "segmental with rash," (b) "poliolike," (c) "granulomatous-nodular," (d) "longitudinally extensive transverse myelitis," (e) "short-segment ovoid or peripherally located," provides a rationale to lessen differential diagnoses. The key for understanding, proper description and differential diagnosis is the correlation of two time points: When did the first symptoms appear and when did imaging take place? Early infarction within the first 24 h will show neither swelling nor enhancement.

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“…4 Interestingly, enhancement may persist despite successful decompression and clinical recovery. 2,5 Contrast enhancement reflects disruption of the blood-spinal cord barrier at that level, but the exact mechanisms are not wellunderstood. 6 Cervical spondylotic changes are unusual in adolescents and children and should raise the issue of traumatic history.…”

mentioning

confidence: 99%