Sequential peripheral neuropathy and myelopathy in a neurosarcoidosis patient

Abstract

A 66-year-old male was referred to our department for evaluation of severe quadriparesis along with marked sensory and autonomic dysfunction. His past medical history was remarkable only for a remote gastrectomy due to gastric ulcer. He was in his usual state of health until 18 months ago, when he noted numbness of the left hand and foot along with numbness and weakness of the right hand. These symptoms were gradually worsening, with numbness in all four limbs, gait imbalance and clumsiness of the right arm. Initial clinical and electrophysiological evaluation at another hospital suggested multiple root or plexus damage but MRI of the cervical spine and brachial plexus were unrevealing. Subsequent electrodiagnostic studies indicated a demyelinating polyneuropathy. The patient was treated with periodic infusions of intravenous immunoglobulin (IVIg), with little or no improvement. During the last 4 months the patient had been bedridden, with severe quadriparesis, numbness of the limbs and trunk, loss of sphincter control, dry mouth, dry eyes and episodic hyperidrosis. Neurologic examination on admission at our department revealed asymmetrical flaccid arm and leg weakness, a bilateral Babinski sign, a ‘gloves-stockings’ pattern of hypoesthesia with a sensory trunk level at Th9, severe proprioceptive loss especially in the legs and signs of autonomic dysfunction. Repeat nerve conduction velocity studies were consistent with the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) (Table 1), CSF contained 24 lymphocytes/mm with normal protein and glucose; CT scans of the chest, abdomen and pelvis were negative for tumor as was detailed autoimmune workup. Based on the signs implying a spinal cord lesion, the inflammatory CSF and lack of improvement with IVIg, a repeat MRI scan of the cervical spine revealed an intraspinal enhancing lesion extending from C2 to C6, as well as scattered enhancing lesions in the thoracic cord (Fig. 1a, b). The patient was treated with prednisolone 75 mg/day, with remarkable and immediate improvement of the weakness. Repeat MRI of the cervical and thoracic spine revealed diffuse areas of myelopathy mainly in the cervical level, with milder enhancement, no edema or mass effect (MRI of the brain was unremarkable). Repeat spinal tap confirmed mild inflammatory CSF with eight lymphocytes, normal protein and glucose, no oligoclonal bands, negative for infectious agents, mycobacteria, antitreponema antibodies or malignant cells. Nerve conduction studies were unchanged. A thorough review of the imaging studies identified small mediastinal lymphadenopathy as well as axillar lymphadenopathy, markedly on the right. A biopsy from the right axilla yielded several enlarged lymph nodes with histopathological features of chronic granulomatous inflammation, with few sites of necrosis. P. Maiovis (&) S. Michelaki P. Ioannidis D. Karacostas 2nd Department of Neurology, AHEPA University Hospital, St Kyriakidi 1, 54636 Thessaloniki, Greece e-mail: panmaio@hotmail.com