CASE 1
Mattia is a 6-year-old boy with no previous medical history. He came for intermittent cramp-like abdominal pain in the last 48 hours, no fever, vomiting or diarrhea. Since some signs of inflammation in the appendix were found at an initial abdominal ultrasound scan, Mattia was sent to Pediatric Emergency Room (PER). Physical examination showed signs of acute abdomen. Therefore, he underwent a video-assisted trans-umbilical appendectomy with histological confirmation of acute appendicitis. After an initial clinical improvement, that allowed him to start walking independently on postoperative day four, we observed a progressive exacerbation of abdominal pain associated with difficulty in maintaining the sitting position.
The next day we also noticed an intermittent tenderness in the lower limbs and inability to keep the upright position and nocturnal enuresis. During a neuropsychiatric evaluation, the patient reported that he did not feel the urge to urinate and refused to leave autonomously the wheelchair. The neurological evaluation highlighted the presence of hypotonia and hyposthenia of the lower limbs, abolished patellar osteotendinous reflexes, abnormal plantar reflex in bilateral toe extension (Babinski sign). An electroneurography, a lumbar puncture and a magnetic resonance imaging (MRI) have been recommended to complete the diagnosis. The neurographic study showed only an asymmetry between left and right. Lumbar puncture showed elevated protein level (>700 mg/dl) in the cerebrospinal fluid, which supported the suspect of a Guillain-Barre syndrome (GBS). Therefore, a treatment with intravenous immunoglobulin at a dose of 0.5 g/kg/day was started. The following day the clinical picture of the patient appeared unchanged. An encephalic and spinal MRI was executed, showing an epidural intracanal tissue located from D7 to D11, associated with blurred signs of medullary suffering (Figure 1A and 1B).
An urgent surgical decompressive laminotomy on 5 levels was performed, with removal of most of the pathological tissue (Figure 1C and 1D). The histological examination reported a pre-B lymphoblastic lymphoma. Mattia has been subsequently transferred to the Onco-hematology department to undertake a specific chemotherapy treatment for the underlying disease. High-dose steroid therapy with methylprednisolone succinate was prescribed.
After surgery Mattia showed a persistent severe hypotonia in the lower limbs, absence of spontaneous movements with weak patellar and Achilles’ osteotendinous reflexes and recovery of tactile sensitivity.
During the hospitalization, the initial paraparesis gradually improved thanks to a targeted rehabilitation program. Distal motor deficiency was associated with intestinal dysmotility, anal sphincter disfunction and spastic neurogenic bladder. The initial difficulty in perceiving the stimulus to defecate and the anal sphincter atony has progressively improved, until resolution. Neurogenic bladder, characterized by an increase in daily urinations with a small amount of urine and mintional urge, was treated by oxybutynin. Intermittent catheterizations were performed six times a day as prophylaxis of urinary tract infections. The patient was found to have 3 asymptomatic bacteriuria, from catheter urinary sample cultures, treated according to the antibiogram. Currently, the number of daily urinations has partially reduced and mintional urge has improved.