Clinical and epidemiological profiles of non-traumatic myelopathies

Pinto, Wladimir Bocca Vieira de Rezende; Souza, Paulo Victor Sgobbi de; Albuquerque, Marcus Vinícius Cristino de; Dutra, Lívia Almeida; Pedroso, José Luiz; Barsottini, Orlando Graziani Povoas

doi:10.1590/0004-282X20160001

Wladimir Bocca Vieira de Rezende Pinto

Universidade Federal de São Paulo, Divisão Geral de Neurologia, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.

Paulo Victor Sgobbi de Souza

Universidade Federal de São Paulo, Divisão Geral de Neurologia, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.

Marcus Vinícius Cristino de Albuquerque

Universidade Federal de São Paulo, Divisão Geral de Neurologia, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.

Lívia Almeida Dutra

Universidade Federal de São Paulo, Divisão Geral de Neurologia, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.

José Luiz Pedroso

Universidade Federal de São Paulo, Divisão Geral de Neurologia, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.

Orlando Graziani Povoas Barsottini

Universidade Federal de São Paulo, Divisão Geral de Neurologia, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brazil.

Correspondence: José Luiz Pedroso; Divisão Geral de Neurologia, Departamento de Neurologia e Neurocirurgia da UNIFESP; Rua Pedro de Toledo, 650; 04023-900 São Paulo SP, Brasil; E-mail: jlpedroso.neuro@gmail.com

Conflict of interest: There is no conflict of interest to declare.

Figures (2)
Tables (4)

Thumbnail

Figure 1
Some spinal cord MRI examples. (A) A 40-year-old man with a 8-month history of paraparesis, upper and lower limbs paresthesias with no sphincter dysfunction and a sagittal MRI section suggestive of Chiari type I malformation; (B) A 48-year-old man with a progressive history of recurrent longitudinally extensive transverse myelitis and a sagittal MRI section suggestive of a demyelinating disease with positive anti-aquaporin-4 antibody; (C) A 68-year-old man with a 4-month history of shoulder muscles twitching and weakness to lift objects with no sphincter dysfunction, and cervical spine MRI suggestive of cervical spondylotic myelopathy; (D) A 52-year-old man with a 3-week-progressive paraparesis with sagittal MRI section showing a pathological fracture of T10 vertebra originating from an extensive pedicular mass and giving rise to a compressive thoracic myelopathy from a neoplastic lesion (pathological diagnosis: plasmacytoma); (E) A 36-year-old woman with exacerbation of chronic low back pain associated with urinary sphincter dysfunction, paraparesis and hypoesthesia in the lower limbs, positive serological tests for schistosomiasis and transverse MRI section showing a T10-T11 level hyperintensity predominantly in dorsal roots, posterior column and lateral corticospinal tract, suggestive of schistosomal myeloradiculopathy.

Thumbnail

Figure 2
Myelopathy algorithm. A proposed algorithm to evaluate nontraumatic myelopathies and its main differential diagnosis.

Thumbnail

Table 1
Group of medical comorbidities and prior clinical conditions presented by patients in this study (n = 166). Only 7 patients (4,2%) did not show any significant past medical history. Percentage among myelopathic patients are represented between brackets.

Thumbnail

Table 2
Main etiological groups of non-traumatic myelopathies, according to initial clinical suspicion and to definitive diagnosis (n = 166). Percentage among myelopathic patients are represented between brackets.

Thumbnail

Table 3
Results from functional clinical severity scale and other myelopathy scores.

Thumbnail

Table 4
Main serum and cerebrospinal fluid laboratory and neuroimaging results.

Relative frequency	Relevant past medical history
> 25%	Smoking (39.2%), hypertension (30.7%), alcoholism (26.5%).
10-25%	Prior malignancy (13.8%), hypercholesterolemia (13.2%), depression (11.5%), hypothyroidism (10.2%).
5-10%	Chronic gastritis and hypertriglyceridemia (9.6%), urinary tract infection repeat (8.4%), diabetes mellitus type 2 (7.8%), megaloblastic anemia (6.0%).
< 5%	4.8%: AIDS. 4.2%: Schistosomiasis, Helicobacter pylori infection, prior sexually transmitted diseases. 3%: epilepsy, primary headache, deep vein thrombosis. 2.4%: Hepatitis C vírus infection, prior pulmonary tuberculosis. 1.8%: abortions, morbid obesity, viral poliomyelitis, prior stroke and acute myocardial infarction, hematologic malignancy, cirrhosis. 1.2%: herpes zoster, parkinsonism, chronic atrial fibrillation, illicit drug abuse, Chagas disease. 0.6%: chronic kidney disease on dialysis, renal tuberculosis, optic neuritis, anterior uveitis, pulmonary thromboembolism, histiocytosis, diabetes mellitus type 1.

Etiological groups

Relative frequency

Initial clinical suspicion

Undefined (36.1%; n = 60), Compressive (21.1%; n = 35), Subacute combined degeneration (vitamin B12 deficiency) (11.4%; n = 19), Demyelinating (9.0%; n = 15), Hereditary spastic paraparesis (3.6%; n = 6), Chiari type I malformation, Schistosomal myeloradiculopathy, Tropical spastic paraparesis (3.0%; n = 5), Postinfectious myelitis (2.4%; n = 4), Adrenomyeloneuropathy, syringomyelia, Motor Neuron Disease (1.8%; n = 3). Others: spinal cord malformations (1.2%; n = 2), HIV-associated vacuolar myelopathy (0.6%; n = 1).

Definitive diagnosis

Undefined (20.5%; n = 34), Subacute combined degeneration (11.4%; n = 19), Cervical canal stenosis with spondylotic myelopathy (9.6%; n = 16), Demyelinating (9%; n = 15) (Multiple Sclerosis 4,8%, Neuromyelitis optica 4.2%), Tropical spastic paraparesis (8.4%; n = 14), Hereditary spastic paraparesis (4.8%; n = 8), Schistosomal myeloradiculopathy (3.6%; n = 6), Spinal disk herniation (3.0%; n = 5), Chiari type I malformation, Vascular myelopathy and Motor Neuron Disease (2.4%; n = 4). Others: various polyneuropathies (4.8%; n = 8), isolated radiculopathy (3.6%; n = 6), movement disorder and syringomyelia (1.8%; n = 3), Somatoform disorder and spinal cord tumours (1.2%; n = 2), Spinocerebellar ataxia type 3, histiocytosis, Herpes zoster-related myelitis, actinic myeloradiculopathy, Sarcoidosis, Copper deficiency myeloneuropathy, CMV-related myelitis, cervical retrolisthesis, PSEN1 -related myelopathy, Post-Polio syndrome, HIV-associated vacuolar myelopathy, Larsen syndrome and Lumbar canal stenosis (0.6%; n = 1).

Scale used	Main results
EMS	48% score I (mild deficits), 19.3% score II (moderate deficits), < 5% score III (severe deficits)
CMS (upper and lower limbs compromise)	3.6% with 7 to 8 points (maximum scores), 4.2% with zero score (minimum score), 70% with scores 1 to 6 (60% with scores 1 to 3)
Nurick score (severity of functional impairment)	48% with scores 2 to 3 (moderate functional impairment), 26% with scores higher or equal to 4 (severe)
Prolo score (economic and functional scale)	60% with grades II/III, 20.5% with grade I, 4.2% with normal values (grade 0)

Additional tests	Main results
Serum laboratorial screening results	11.4% with cobalamin deficiency, 10.2% with high MCV, 9% with positive ANA, 4.2% with positive HTLV-I/II serologic test, 4.2% with positive anti-aquaporin-4 antibodies, < 2% with high VLCFA, 0.6% with copper deficiency
Cerebropinal fluid examination	3.6% with albuminocytologic dissociation, 3.6% with positive oligoclonal bands, 1.2% with hypereosinophilic content (> 10% of total pleocytosis)
Neuroimaging findings	23.5% with non-compressive signal changes in any segments in T2/FLAIR images; 9.6% of cases with cervical spine stenosis (16.9% of all cases with intervertebral disc protrusions in the cervical segment)
Neurophysiological studies	1.8% with anterior horn cell compromise; 24.2% with demyelinating polyneuropathy; 5.4% with isolated axonal polyneuropathy; 3.6% with isolated radiculopathy

[TFN2] EMS: European Myelopathy Score; CMS: Cooper Myelopathy Score.

Clinical and epidemiological profiles of non-traumatic myelopathies

Perfil clínico e epidemiológico das mielopatias não-traumáticas

ABSTRACT