Spinal canal decompression for hypertrophic neuropathy of the cauda equina with chronic inflammatory demyelinating polyradiculoneuropathy:A case report

BACKGROUND Hypertrophic neuropathy of the cauda equina(HNCE)is a rare disease,especially in children.It can be caused by different etiological agents such as inflammation,tumor or hereditary factors.Currently,there is no uniform standard for clinical treatment of HNCE.Furthermore,it is unclear whether spinal canal decompression is beneficial for patients with HNCE.CASE SUMMARY We report the case of a 13-year-old boy with enlargement of the cauda equina.The onset of the disease began at the age of 6 years and was initially marked by radiating pain in the buttocks and thighs after leaning over and weakness in the lower limbs when climbing a ladder.The child did not receive any medical treatment.As the disease slowly progressed,the child needed the help of others to walk,and he had a trendelenburg gait.He underwent spinal canal decompression and a nerve biopsy during his hospital stay.A diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy was made based on electrophysiological findings and pathological examination results.Immunoglobulin or hormone therapy was recommended during hospitalization,but his mother refused.After discharge,the boy’s mother helped him carry out postoperative rehabilitation training at home.His lower-limb muscle strength gradually increased,and he could stand upright and take steps.Six mo after surgery,the child was readmitted and began immunoglobulin therapy.Long-term oral steroid treatment was initiated after discharge.The movement and sensation of the lower limbs were further improved,and the boy could walk normally 1 year after surgery.CONCLUSION Spinal canal decompression can improve the clinical symptoms of HNCE caused by inflammation,even in children.When combined with specific etiological interventions,spinal cord decompression can lead to optimal outcomes.

Tacrolimus treatment for relapsing-remitting chronic inflammatory demyelinating polyradiculoneuropathy:Two case reports

BACKGROUND This study describes the efficacy of a tacrolimus treatment regimen used to treat two patients with relapsing-remitting chronic inflammatory demyelinating polyradiculoneuropathy(CIDP).CASE SUMMARY Two patients(17-year-old female and 27-year-old male)were enrolled in the current study and were followed up for 12 mo.The first patient was administered tacrolimus(2 mg/d)for 12 mo and prednisolone(40 mg/d)for six months.The second patient was administered tacrolimus(3 mg/d)for six months.Both patients were followed up for 12 mo and the degree of recurrent weakness or normalized motor function was monitored.In addition,nerve conduction studies and tacrolimus levels were recorded.Following tacrolimus treatment,both patients showed marked improvement in clinical outcomes.In the first patient,prednisolone treatment was successfully withdrawn after six months.Sensory as well as motor nerve conduction velocities showed evident recovery following treatment.However,conduction velocities did not completely return to normal,suggesting that electrophysiological recovery can be slower than clinical recovery.CONCLUSION Neither patient exhibited any adverse effects due to the tacrolimus therapy.Therefore,tacrolimus can be effective for the treatment of patients with steroidresistant CIDP.

Nerve biopsy findings contribute to diagnosis of multiple mononeuropathy: 78% of findings support clinical diagnosis

Multiple mononeuropathy is an unusual form of peripheral neuropathy involving two or more nerve trunks. It is a syndrome with many different causes. We reviewed the clinical, electrophysi- ological and nerve biopsy findings of 14 patients who suffered from multiple mononeuropathy in our clinic between January 2009 and June 2013. Patients were diagnosed with vasculitic neurop- athy （n = 6）, perineuritis （n = 2）, chronic inflammatory demyelinating polyradiculoneuropathy （n = 2） or Lewis-Sumner syndrome （n = 1） on the basis of clinical features, laboratory data, elec- trophysiological investigations and nerve biopsies. Two patients who were clinically diagnosed with vasculitic neuropathy and one patient who was clinically diagnosed with chronic inflamma- tory demyelinating polyradiculoneuropathy were not confirmed by nerve biopsy. Nerve biopsies confirmed clinical diagnosis in 78.6% of the patients （11/14）. Nerve biopsy pathological diagno- sis is crucial to the etiological diagnosis of multiple mononeuropathy.

Clinical and electrophysiological profiles in early recognition of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome

Background:The detection of polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes (POEMS) syndrome at early stage is challenging for neurologists.Since polyneuropathy could be the first manifestation,it could be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP).The present study aimed to determine the clinical and electrophysiological features of POEMS syndrome to distinguish from CIDP.Methods:The data of a group of patients with POEMS (n =17) and patients with CIDP (n =17) in Zhongshan Hospital Fudan University from January 2015 to September 2017 were analyzed in this retrospective study.The clinical features,neurological symptoms,and electrophysiological findings were compared between the two groups.Results:Clinically,patients with POEMS demonstrated significantly more neuropathic pain in the lower extremities than patients with CIDP (58.8 % vs.11.8 %,P =0.01).Multisystem features like edema,skin change,organomegaly,and thrombocytosis were also pointed towards the diagnosis of POEMS syndrome.Electrophysiologically,terminal latency index (TLI) was significantly higher in patients with POEMS than that in patients with CIDP (median nerve:0.39 [0.17-0.52] vs.0.30 (0.07-0.69),Z =-2.413,P =0.016;ulnar nerve:0.55 [0.23-0.78] vs.0.42 [0.12-0.70],Z =-2.034,P =0.042).Patients with POEMS demonstrated a higher frequency of absent compound muscle action potential of the tibial nerve (52.9% vs.17.6%,P =0.031),less conduction block (ulnar nerve:0 vs.35.3%,P =0.018),and less temporal dispersion (median nerve:17.6% vs.58.8%,P =0.032) than CIDP group.The combination of positive serum monoclonal protein and high TLI (if either one or both were present) discriminated POEMS from CIDP with a sensitivity of 94.1% and 47.1% and specificity of 76.5% and 100.0%,respectively.Conclusions:POEMS syndrome could be distinguished from CIDP through typical clinical and electrophysiological characteristics in practice.The combination of serum monoclonal protein and high TLI might raise the sensitivity of detecting POEMS syndrome.