CASE 16628 Published on 04.03.2020

Subacute combined spinal cord degeneration

Section

Neuroradiology

Case Type

Clinical Cases

Authors

Alessandro Di Paola1, Juan Carlos Tortajada2, Alex Rovira2

1 Radiology Institute, Department of Medicine - DIMED, University of Padova, Via Giustiniani 2, 35100, Padova, Italy.

2 Section of Neuroradiology and Magnetic Resonance Unit, Department of Radiology (IDI), Vall d'Hebron Institut de Recerca, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Pg. Vall d'Hebron 119-129, Barcelona 08035, Spain.

Please address correspondence to Alessandro Di Paola MD - Radiology Institute, Department of Medicine - DIMED, University of Padova, Via Giustiniani 2, 35100, Padova, Italy.

e-mail: a.g.dipaola@gmail.com

Patient

61 years, female

Categories
Area of Interest CNS, Neuroradiology spine ; Imaging Technique MR
Clinical History

A 61-year-old female patient presented for a 3-months period with hypoaesthesia of both lower limbs and gait disturbance without motor symptoms. 

On past medical history the patient referred a previous B12-deficiency and a chronic atrophic gastritis. 

Neurological examination objectified tactile hypoaesthesia of both lower limbs and normal pain perception and proprioception. 

No pathological findings were observed on LCR and blood samples. 

Imaging Findings

Spinal cord MRI was performed revealing multiple and confluent lesions affecting both posterior and lateral columns of the entire spinal cord. Lesions were hyperintense on T2-weighted sequences, without enhancement after gadolinium injection. On the axial plane posterior columns lesions showed inverted “V”-morphology. Anterior columns were not affected. Brain MRI did not show pathological findings.

MRI findings, along with present and past medical history, suggested a subacute combined spinal cord degeneration, caused by the previous vitamin B12-deficiency. 

Discussion

Subacute combined spinal cord degeneration is an acquired progressive myelopathy caused by B12-deficiency, a vitamin necessary for normal turn-over of myelin sheath that surrounds nerve fibers. Its deficiency can be caused by inadequate dietary intake or, more frequently, by inadequate gastrointestinal absorption for autoimmune gastritis or previous surgery. Clinical presentation of subacute combined spinal cord degeneration consists mainly of sensory symptoms as paraesthesia, sensory loss and gait ataxia. If left untreated, the disease may progress to ataxic paraplegia. 

Normally, B12-deficiency goes along with megaloblastic anaemia, although lack of parallelism between haematologic and neurologic findings is well-known and frequently reported in literature [1–3]: this was the case for our patient. 

MRI findings consist of demyelinating lesions affecting posterior and, more rarely, lateral columns of the spinal cord. Lesions are typically T2-hyperintense showing the characteristic “V” inverted sign; contrast-enhancement is rare and when present very mild [4]. Long lasting lesions may result in spinal cord atrophy. 

Demyelinating lesions in cerebral deep white matter are uncommon but reported in literature [5].

Diagnosis is made by MRI and low serum B12-level or elevated levels of the metabolites homocysteine and methylmalonic acid [6].

Differential diagnosis is broad and includes copper deficiency and inflammatory and demyelinating disorders such as multiple sclerosis, neuromyelitis optica and neurosarcoidosis.

Treatment of B12-deficiency by monthly B12 intramuscular injections.

At an older age, presence of extensive lesions and longer duration of illness are negatively related to resolution of lesions and clinical signs. This relationship underscores the importance of early diagnosis[2]. 

Diagnosis of subacute combined spinal cord degeneration must be suspected in the presence of characteristic radiologic and clinical findings even in the absence of macrocytic anaemia.

The aforementioned patient received weekly B12 intramuscular injection with clinical improvement and was therefore discharged.

Written informed patient consent for publication has been obtained.

Differential Diagnosis List
Subacute combined spinal cord degeneration
Copper deficiency
Multiple sclerosis
Neuromyelitis optica
Neurosarcoidosis
Spinal cord ischaemia
Final Diagnosis
Subacute combined spinal cord degeneration
Case information
URL: https://www.eurorad.org/case/16628
ISSN: 1563-4086