Quando não é esclerose múltipla: abordagem baseada em casos

Nicolau, Zaira Fernanda Martinho; Oliveira, Enedina Maria Lobato de; Bichuetti, Denis Bernardi

doi:10.1590/0004-282X20150163

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ARTICLES • Arq. Neuro-Psiquiatr. 73 (12) • Dez 2015 • https://doi.org/10.1590/0004-282X20150163 copiar

Quando não é esclerose múltipla: abordagem baseada em casos

Autoria SCIMAGO INSTITUTIONS RANKINGS

Objetivo

Apresentar uma série de casos de pacientes previamente diagnosticados como esclerose múltipla (EM) que tiveram um diagnóstico final alternativo, e descrever os sinais clínicos e paraclínicos que levaram à esta mudança.

Método

Nove pacientes encaminhados para confirmação diagnóstica de EM. Revisamos os prontuários, imagens de ressonância magnética (MRI) e líquido cefalorraquidiano.

Resultados

Houve uma média de três sintomas típicos de EM e quatro sinais de alerta por paciente. Sinais de alerta de ressonância magnética foram encontrados em 88,9% dos casos, com média de 3 por paciente.

Conclusão

Identificou-se que, não só os pacientes com diagnóstico incorreto não cumprem critérios de diagnóstico, mas também como os sinais de alerta já descritos são uma ferramenta útil na diferenciação de EM de outras doenças. Esta informação é importante para orientar a assertividade diagnóstica, especialmente para os clínicos e neurologistas gerais, com interferencia direta no tratamento, prognóstico e qualidade de vida do paciente.

esclerose múltipla; diagnóstico; clínico; ressonância magnética

Patient ID	Age at first symptom	Sex	First symptom	Clinical Course (progressive x relapsing)	Age at MS diagnosis (suspicion)	Age at first evaluation at UNIFESP	Therapies before diagnostic definition	Final diagnosis	Therapies after diagnosis definition
1	36 years	F	Aggression outbreaks, left hand stiffness, hypoesthesia of the lower limbs and sudden bilateral amaurosis	Relapsing	38 years	43 years	Methylprednisolone pulse therapy; interferon beta-1a	Multiple cerebral emboli due to atrial mixoma	Suspension of interferon beta-1a
2	53 years	F	Somnolence, tremor and left side weakness	Progressive within weeks	53 years	53 years	Levodopa	Yellow fever vaccine associated encephalitis	Prednisone
3	21 years	F	Paresthesia, walking difficulty and sensory ataxia	Relapsing, single episode	21 years	21 years	Methylprednisolone sodium succinate	Syringomyelia	None
4	27 years	M	Paresthesia and limb weakness	Relapsing	27 years	27 years	Prednisone	NeuroBehçet	Prednisone, pulse IV methylprednisolone, IV cyclophosphamide and tocilizumab
5	29 years	F	Blurred vision, walking difficulty	Relapsing	29 years	38 years	Methylprednisolone IV pulse therapy, Interferon beta-1b, Interferon beta-1a, paroxetin	Susac syndrome or Primary CNS vasculitis	Prednisone and pulse IV cyclophosphamide
6	39 years	F	Lower back and thoracic pain, difficult walking	Progressive	39 years	41 years	Vitamin B12	Polyneuromyopathy and myelopathy due to severe nutritional deficiency	Parenteral nutrition, copper, zinc, iron and B12 supplementation
7	35 years	M	Visual and hearing loss, cognitive impairment and ataxia	Relapsing-Progressive	35 years	35 years	Pulse IV methylprednisolone, fingolimode and prednisone	Susac syndrome	Prednisone, azathioprine and pulse IV cyclophosphamide
8	36 years	M	Headache, hemiparesis and partial motor seizure	Progressive	36 years	36 years	Oral steroids	Primary CNS lymphoma (under review)	Dexamethasone and hematological consultation
9	33 years	F	Headache and visual loss	Progressive	33 years	33 years	None	Mithocondrial disorder (under review)	Sent for the Neuromuscular unit for evaluation

Patient ID	Typical MS symptoms	Clinical Red Flags	Final diagnosis	CSF cells (cell/mm³)	CSF OCB	Paraclinical red flags	MRI judged typical for MS*	Barkhof-Tintoré DIS**	Barkhof-Tintoré DIT**	Swanton DIS***	Swanton DIT***	MRI Red Flags****
1	Limb weakness, hypoesthesia, neurogenic bladder	Cardiac disease, thrombotic events, seizure, sudden bilateral painfull amaurosis, aggression outbreaks, early cognitive decline with dysarthria.	Multiple cerebral emboli due to atrial mixoma	1.0	NA	Atrial mixoma Branch retinal artery occlusion on ophthalmologic evaluation Normal brain biopsy	-	+	-	+	-	Absence of temporal horn lesion, infratentorial and spinal cord lesion. Most lesions are sucortical
2	Limb weakness, paresthesia, urge incontinence, spasticity	Myalgias, anxiety, somnolence, abrupt onset, onset after age 50	Yellow fever vaccine associated encephalitis	1.0	NA	Brain biopsy with atypical inflammatory infiltrate and white matter with gliosis	-	+	-	+	-	Absence of temporal horn lesion, infratentorial and spinal cord lesion. Most lesions are large and subcortical, all enhance at the same time
3	Limb weakness, paresthesia, sensory ataxia	Normal neurological evaluation	Syringomyelia	1.2	NA	Normal visual evoked potentials	-	+	-	+	-	Lesion compatible with syringomielia. No lesions on brain MRI.
4	Limb weakness, paresthesia, diplopia, hypoesthesia	Seizure, Headache, mucosal ulcers, aggression, early dysarthria and dysphagia	NeuroBehçet	Up to 250.0 cell/mm³with neutrophil predominance	NA	CSF pleocytosis	-	+	+	+	+	Absence of spinal cord lesion. Most lesions are large and subcortical or large and in the brainstem
5	Limb weakness, fatigue, gait ataxia, sensory loss, spasticity	Extrapyramidal features, retinopathy, early and severe dysarthria, early cognitive impairment and abrupt onset	Susac syndrome or Primary CNS vasculitis	13.0	Presence	Rethynopathy Brain biopsy with necrotizing vasculitis	-	+	+	+	+	Absence of temporal horn lesion and infratentorial lesion. Most lesions are large and subcortical.
6	Limb weakness, pain, spasticity	Polyradiculopathy, myopathy and muscle pain, amyotrophy, past medical history of gastric bypass surgery	Polyneuromyopathy and myelopathy due to severe nutritional deficiency	2.0	NA	Anemia, low serum vitamin D, iron, copper and zinc	+	+	-	+	+	No
7	Gait ataxia, spasticity, sensory loss, tetraparesis, urinary incontinence	Retinopathy, hearing loss, cutaneous involvement, early severe cognitive impairment and dysarthira	Susac syndrome	2.0	Absence	Skin biopsy with leukocytoclastic vasculitis, hearing loss on audiologic evaluation, elevated CSF protein (114mg/dL)	-	-	-	-	-	Punctiform lesions, diffusion restriction and lesions restricted to the corpos callosum (snowball lesions)
8	Hemiparesis, sensory loss, spasticity	Headache, seizure, thrombotic events, progressive severe early hemiparesis	Primary CNS lymphoma (under review)	9.0	NA	Brain biopsy not typical of MS with atypical lymphocytic infiltrate	-	-	-	-	-	Exclusively unilateral hemispheric lesion
9	Depression, vertigo	Headache, hearing loss, bilateral ptosis	Mithocondrial disorder (under review)	1.0	NA		-	-	-	+	-	Absence of temporal horn lesion, infratentorial and spinal cord lesion. Most lesions are small, round and scarce in the subcortical white matter

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[1] Correspondence: Denis Bernardi Bichuetti; Disciplina de Neurologia - UNIFESP; Rua Botucatu, 740; 04023-900 São Paulo SP, Brasil; E-mail:bichuetti@unifesp.br