Revisiting the spectrum of lower motor neuron diseases with snake eyes appearance on magnetic resonance imaging

Lebouteux, M.; Franques, Jérôme; Guillevin, Rémy; Delmont, Émilien; Lenglet, Timothée; Bede, Peter; Desnuelle, Claude; Pouget, J; Pascal-Mousselard, H.; Pradat, P

doi:10.1111/ene.12465

Cited by 54 publications

(33 citation statements)

References 36 publications

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“…However, none had contrast MR study with neck flexion. Further, there were patients with proximal affection unlike our cohort and their mean age at onset ranged from 30 to 40 years (12).…”

Section: Discussionmentioning

confidence: 81%

Distal bimelic amyotrophy (DBMA): Phenotypically distinct but identical on cervical spine MR imaging with brachial monomelic amyotrophy/Hirayama disease

Preethish‐Kumar

Nalini

Singh

et al. 2015

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Our objective was to characterize the MR imaging features in a large and distinct series of distal bimelic amyotrophy (DBMA) from India. We utilized a retrospective and prospective study on 26 cases. Results demonstrated that upper limb distal muscle wasting and weakness was predominantly symmetrical in onset. Mean age at onset was 20.9 ± 7.0 years, mean duration 83.0 ± 102.6 months. MRI carried out in 22 patients with flexion studies showed forward displacement of posterior dura in 19 (86.4%). Crescent shaped epidural enhancement on contrast was seen in 20/24 cases (83.3%), and bilateral T2W hyperintensities of cord in17 (65.4%) - symmetrical in15 cases. Maximum hyperintensity was noted at C5-C6, C6-C7 levels. Cord atrophy was noted in 24 (92.3%) cases (most affected: C5-C6, C6-C7) - symmetrical atrophy in 21cases. Cervical spine straightening occurred in six (23.1%) cases and reversal of lordosis in 15 (57.7%). In conclusion, this study confirms that DBMA is phenotypically distinct but pathophysiologically the same as brachial monomelic amyotrophy (BMMA) on MR imaging. Typical MRI features were seen in all. It is important to differentiate this disorder from ALS, which could present at a younger age as often seen among Indians. The clinical and MR imaging features are highly suggestive that DBMA, as with BMMA/Hirayama disease, occurs due to dynamic alterations at the cervical spine level.

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“…However, none had contrast MR study with neck flexion. Further, there were patients with proximal affection unlike our cohort and their mean age at onset ranged from 30 to 40 years (12).…”

Section: Discussionmentioning

confidence: 81%

Distal bimelic amyotrophy (DBMA): Phenotypically distinct but identical on cervical spine MR imaging with brachial monomelic amyotrophy/Hirayama disease

Preethish‐Kumar

Nalini

Singh

et al. 2015

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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“…It has been reported in a number of diseases, such as complex cervical spondylosis, Hirayama disease, poliomyelitis, encephalitis caused by HIV infection, spinal cord infarction, neuromyelitis optica, paraneoplastic neurological syndrome and flail arm syndrome [20, 21]. Neuroimaging-documented appearance of the “owl’s eye” sign may reflect cystic necrosis in the central gray matter near the ventrolateral posterior column, and is consequently regarded as an unfavorable prognostic factor for the recovery of upper-extremity motor weakness, due to the destruction of gray matter accompanied by significant neuronal loss in the anterior horn [22].…”

Section: Discussionmentioning

confidence: 99%

A case of neuropsychiatric lupus Erythematosus characterized by the Owl’s eye sign: a case report

Zhou

et al. 2017

BMC Neurol

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BackgroundSystemic lupus erythematosus (SLE) is an autoimmune inflammatory disorder characterized by multiple affected systems. More than half of SLE patients will suffer from neuropsychiatric lupus erythematosus (NPSLE) during the course of their disease. Although nearly half of the NPSLE patients have normal MRI manifestations, the abnormalities found in the remainder can be located anywhere in the brain, and especially in the subcortical white matter of the frontal and temporal lobe. However, NPSLE involving the medulla oblongata and spinal cord which presents as the “owl’s eye” sign has to our best knowledge not been reported to date.Case presentationA 19-year-old girl presented at our hospital with a 7-day history of fever and headache since a one day’s exertion, accompanied by 2 days of weakness. The patient had slurred speech. Neurological examination revealed the presence of horizontal nystagmus and a limitation of bilateral eye movement when looking up and down. At the same time, she showed difficulty in raising the jaw, accompanied by a weak pharyngeal reflex. Muscle strength was remarkably decreased in all four extremities: the MRCS grade of the upper limbs was 4/5, while in the lower limbs it was 0/5. Hypotonia was apparent in the lower extremities. Regarding subjective sensation, the patient appeared to be experiencing an increased sense of pain in the whole body, and especially in the cervical region, abdomen, and feet. An examination of shallow reflex documented the reinforcement of the abdominal reflex. Deep tendon reflexes were symmetric: absent in lower, normal in upper extremities. The patient also had a stiff neck with a positive Kernig’s sign. The laboratory examination showed elevated C - reactive protein and rheumatoid factor, as well as complement components 3 and 4. Symptomatic treatments were applied, but she did not respond well, after which we did immunological laboratory examinations. The results showed the presence of anti-nRNP/Sm, anti-dsDNA and anti-AMA M2 antibodies. An MRI scan and enhancement of the cervical and thoracic regions displayed abnormal signs in the medulla and bilateral anterior horn of the lower thoracic spine. Following the exclusion of other possible diseases, neuropsychiatric lupus was diagnosed. High-dose intravenous gamma-globulin combined with methylprednisolone gradually improved her condition.ConclusionWe report the first case of NPSLE presenting with medulla oblongata and spinal cord involvement, manifesting as the “owl’s eye” sign in MRI.

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“…Qualitative cues may be nonspecific and require careful appraisal. The specificity and sensitivity of 'owl's eyes' or 'snake's eye' signs on axial imaging is relatively poor but may indicate cord infarction, PLS or ALS in the appropriate clinical context [9]. Similarly, the 'inverted V sign' or hyperintensities of the dorsal columns is nonspecific and may be observed in subacute combined degeneration of the cord, copper deficiency myeloneuropathy and tabes dorsalis.…”

Section: Current Practice: Methods Challenges and Limitationsmentioning

confidence: 99%

From Pneumomyelography to Cord Tractography: Historical Perspectives on Spinal imaging

2017

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Revisiting the spectrum of lower motor neuron diseases with snake eyes appearance on magnetic resonance imaging

Cited by 54 publications

References 36 publications

Distal bimelic amyotrophy (DBMA): Phenotypically distinct but identical on cervical spine MR imaging with brachial monomelic amyotrophy/Hirayama disease

Distal bimelic amyotrophy (DBMA): Phenotypically distinct but identical on cervical spine MR imaging with brachial monomelic amyotrophy/Hirayama disease

A case of neuropsychiatric lupus Erythematosus characterized by the Owl’s eye sign: a case report

From Pneumomyelography to Cord Tractography: Historical Perspectives on Spinal imaging

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