Dados do Trabalho

Título

Tuberculous pachymeningitis presenting as motor neuron disease

RESUMO

Case presentation: A 63-year-old man presented with bilateral frontoparietal headache that progressed within 6 months to generalized weakness, hoarseness, dysphagia, left hemifacial neuralgic pain, unintentional weight loss of 20kg and no history of febrile illness. On examination, there was cranial nerve palsies of nerves V, VII, VIII, IX, X, XI XII on right side and V on left side; tongue and upper limb fasciculation, distal amyotrophy in upper limbs and global hyperreflexia. Brain MRI showed pachymeningeal thickening in the left middle cranial and posterior fossa, nodular lesions with low T2 signal in cerebellar vermis and right cerebellar hemisphere with growth towards cerebelomedullary cistern and fourth ventricle. Cervical spine MRI was normal. Cerebrospinal fluid examination showed pleocytosis of 52/mm3 with lymphocytic predominance, hyperproteinorraquia and no glucose consumption. Ziehl-Neelsen, India ink, Gram stain and Xpert MTB negative. Serologies for syphilis and HIV negative. Electroneuromyography (EMG) presented chronic and subacute denervation of preganglionic origin of bulbar, cervical, thoracic and lumbosacral myotomes, without spontaneous activity. Based on clinical and paraclinical data, empiric treatment for tuberculosis was started. Diagnosis was confirmed by bronchoalveolar lavage with a positive Xpert MTB/RIF test. Despite therapy, patient presented clinical complications and died after 2 months. Discussion: Tuberculous hypertrophic pachymeningitis usually presents with cranial signs and symptoms such as headache and cranial nerve palsies, or spinal symptoms such as radicular pain. Reported patient showed progressive cranial nerve palsies and on brain MRI, the extensive dural thickening involving the skull base explains all the cranial nerve involvement. He also had signs of upper and lower motor neuron involvement without radicular pain, sensory deficits or sphincter complaints, which is not typical of tuberculous myeloradiculopathy. On cervical spine MRI there was no thickening or enhancement of meninges, but EMG indicated symmetric pre-ganglionic involvement of nerves, probably secondary to a radiculopathy only of the anterior root. Our case shows that this topography should be considered in patients who presents with motor neuron disease and have some alarming features such as new onset headache. Final comments: We presented a case of tuberculous pachymeningitis with extensive cranial nerve involvement and moto neuron symptoms.

Palavras Chave

Meningitis; Tuberculosis, Central Nervous System; Motor Neuron Disease