Department of Neurosurgery, Care Complex University of Salamanca. Spanish Transition Walk, 37007, Salamanca, Spain.
*Corresponding Author: Patricia Alejandra Garrido Ruiz
Department of Neurosurgery, Care Complex University of
Salamanca, Spanish Transition Walk, 37007, Salamanca, Spain.
Email: agrjandri_92@hotmail.com
Received : Aug 02, 2023
Accepted : Sep 01, 2023
Published : Sep 08, 2023
Archived : www.jclinmedimages.org
Copyright : © Garrido Ruiz PA (2023).
We present a case of a 12-year-old patient suffering from progressive spastic paraparesis secondary to multiple contrastenhancing ependymal spinal cord lesions. The patient has been presenting varied symptoms: patellar hyperreflexia, urinary and fecal incontinence without sensory disorder, motor deficit in the right lower limb, posterior uveitis with retinal vasculitis, abdominal pain and vomiting of four years of evolution, arthralgia and deformity in one finger of the hand, hypogammaglobulinemia and mild hereditary thrombocytopenia. MRI revealed extensive vascular enhancement, beaded-shaped, at the medullary and cerebral periependymal level, bilateral subcortical, cerebellar peduncles, and right cerebellar hemisphere; and punctate enhancement at the medullary level with extensive medullary myelopathy involving the conus medullaris [1-6,7-10]. All complementary studies to date had been negative.
Keywords: Rosary-like; Neurosarcoidosis; Periventricular; Periependymal.
The differential diagnosis was between an inflammatory disease or a tumor (ganglioneuroma as a feasible possibility) [2-4], with infectious suspicion being the least likely. It was decided to perform a neuronavigation-guided biopsy and endoscopy of ventricular lesions in the left frontal horn with septostomy and biopsy in the contralateral caudate in which the uptake area can be seen [1].
Neurosarcoidosis was the anatomopathological finding. Corticosteroids and immunosuppressants were prescribed and the patient’s condition improved [2,6].
Neurosarcoidosis is a rare complication of sarcoidosis. Patients undergo several tests until the diagnosis is reached. When periependymal beaded-shaped lesions are observed in the MRI and a broad spectrum of symptoms appears in addition to neurological focality, it should be suspected. Some cases disappear spontaneously within 4 to 6 months, other cases continue intermittently. Prognosis is acceptably favorable with treatment, so early diagnosis is important. The treatment of choice should be individualized but a high dose of steroids and suppression of the immune system with chemotherapy, and immunotherapy drugs, such as infliximab, are usually included. Treatment is necessary for at least 5 years.
Conflict of interest: The authors declare no conflicts of interest.