Neuropathies and almitrine. 14 cases

Previously reported cases of peripheral neuropathies occurring during almitrine therapy had only a few weeks follow-up after having stopped the drug. We have studied clinical and electrophysiological data 6 to 12 months after almitrine had been given up in 9 patients from a group of 14 whose epidemiologic, clinical, electrophysiological and pathological data had been registered. In 7 of them, without any chronic respiratory deficiency, almitrine was administered as almitrine bismésilate and raubasine, and in 7 others (6 with chronic respiratory deficiency) as almitrine bismesilate alone. In patients who had another possible cause of neuropathy, clinical disorders appeared after a lesser total quantity of almitrine (p less than 0.05). Clinical data were suggestive of sensory peripheral neuropathies of feet and lower third of legs. Electrophysiological data suggested distal axonopathy in spite of the absence of denervation: amplitudes of sensory potentials were reduced and nerve conduction velocities were moderately decreased. Biopsies revealed mild neurogenic atrophy of muscles and distal axonopathy. Clinical improvement was very slow and 6 to 12 months later, most of the patients still presented decreased vibration sense and ankle reflexes loss, but all of them were still improving. Amplitudes of sensory potentials and sensory nerve conduction velocities were significantly improved (p less than 0.05) but motor nerve conduction velocities were not (p greater than 0.05). Our study shows: 1) clinical, electrophysiological and pathological data similar to those previously reported; 2) subclinical disturbances of motor functions in lower limbs and sensory functions in upper limbs; 3) some patients presented with unusual signs: posture tremor (3 cases), painful legs and moving toes (1 case); 4) peripheral neuropathies may occur during almitrine therapy even in patients without any chronic respiratory insufficiency; 5) peripheral neuropathies occurred with lower doses in patients with other factors predisposing to neuropathies; 6) patients' improvement was very slow; 7) in 9 cases the imputability of these peripheral neuropathies to almitrine is plausible. We suggest not to prescribe almitrine without caution, especially in patients with other factors of neuropathy. Treatment should be regularly interrupted.     

周围髓鞘蛋白22基因重复突变致夏科-马里-图斯病1A亚型的临床和神经电生理特征

目的探讨周围髓鞘蛋白22(PMP22)基因重复突变阳性的夏科-马里-图斯病(CMT)lA亚型患者临床和神经电生理改变特点。方法总结21例PMP22基因重复突变阳性的CMTlA患者的临床特点,并分析其神经电生理特征。结果 21例患者中,10例临床特征符合四肢远端萎缩无力的典型CMTl型表现,另外11例呈不典型性,如仅有头晕、合并听力障碍、上肢姿势性震颤、反复发作性肢体无力、伴有小脑性共济失调及癫疒间等。10例患者肌电图出现纤颤电位和(或)正锐波,15例患者运动单位电位时限延长。神经传导存在广泛异常,所有患者被检的运动或感觉神经传导速度存在不同程度的减慢或消失。结论 PMP22重复突变阳性的CMTlA患者具有较高的临床异质性,其电生理特点为肌电图呈神经源性损害,感觉神经病变重于运动神经,下肢受累程度重于上肢,神经电生理检查对CMT1A的诊断很重要。     

Postvaccinal inflammatory neuropathy: peripheral nerve biopsy in 3 cases

Autoimmune inflammatory polyneuropathy (PN) can be triggered by vaccination. We report 3 such cases. A 36-year-old female nurse presented 15 days after a hepatitis B vaccination (HBV) with acute sensory disturbances in the lower limbs. She had severe ataxia but no weakness. Cerebrospinal fluid (CSF) protein level was 84 mg/100 mL, with 3 lymphocytes. A 66-year-old man presented 21 days after HBV with severe motor and sensory PN involving all 4 limbs. A 66-year-old man presented 15 days after a yellow fever vaccination with progressive motor and sensory PN involving all 4 limbs and bilateral facial paralysis. CSF protein level was 300 mg/100 mL, with 5 lymphocytes. Six weeks later, a tracheostomy was performed. In these 3 patients, the nerve deficits lasted for months. In each case, peripheral nerve biopsy showed KP1-positive histiocytes but no T-lymphocytes in the endoneurium. On ultrastructural examination, there was axonal degeneration in the first 2 cases; in case 2, a few myelinated fibers exhibited an intra-axonal macrophage but the myelin sheath was preserved. There was only 1 example of macrophage-associated demyelination in case 2, but these were numerous in case 3. It is likely that in the first 2 cases, an autoimmune reaction against some axonal or neuronal components was triggered by HBV. It induced an acute sensory ataxic PN in case 1 and an acute motor and sensory axonal neuropathy (AMSAN) in case 2. The third patient had a chronic inflammatory demyelinating PN, likely triggered by yellow fever vaccination.     

Friedreich''s ataxia: a clinical review of 20 childhood cases

The authors report the clinical review of 20 childhood cases with Friedreich's ataxia. The mean age at onset of symptoms was 6.1 years. The main presenting symptom was abnormal gait (100%). Ataxia of gait and limbs and depressed or absent tendon reflexes were found in all cases. Clinical findings are in accordance with the findings of Harding and Werdelin. Neurophysiological studies (especially sensory) are important in the confirmation of the diagnosis. Of the 10 cases in which sensory nerve conduction velocity measurements were performed, 7 had absent sensory action potentials, 2 had decreased potentials and one was normal. In our study, it is shown that in patients having ataxic gait, ataxia of limbs and tendon reflexes depression or loss, Friedreich's ataxia may be diagnosed with the help of electrophysiological studies.     

Neuropathy Associated With Anti-Chondroitin Sulfate C IgM Antibodies

Chondroitin Sulfate C (ChS-C), is a glycosaminoglycan present in the membranes of neurons and axons. Anti-ChS-C IgM antibodies have been reported in patients with predominantly sensory neuropathy (PN) often associated with IgM monoclonal gammopathy, but also in some neurological controls. In order to evaluate the frequency and clinical correlate of anti-ChS-C IgM antibodies, we tested them by a new Covalink ELISA technique in sera from 206 patients with IgM monoclonal gammopathy including 79 with PN (PN+IgM) with unknown IgM reactivity, 65 with PN with antibodies to the myelin-associated glycoprotein and 62 without PN, and from 33 patients with PN of other causes, 30 with other neurological and non-neurological diseases and 23 normal subjects. We only found high titers of anti-ChS-C IgM in two patients (1/128,000 and 1/256,000 respectively) with IgM monoclonal gammopathy: one had Waldenström Macroglobulinemia diagnosed seven years before and a 3 year history of slowly progressive limb weakness, finger paresthesias, unsteady gait and occasional nocturnal cramps. Neurological examination revealed a predominantly large-fiber sensory neuropathy with mild distal atrophy and weakness in upper and lower limbs. Electrophysiological and morphological studies were suggestive of a predominantly demyelinating neuropathy. The other patient had IgM MGUS without PN at the time of antibody testing but developed finger paresthesias seven years later, when he had decreased position sense and abnormal sensory nerve conduction studies. In conclusion high titers of anti-ChS-C IgM, though infrequent, were always associated with the presence or development of sensory PN in patients with IgM M-protein, supporting a possible role for these antibodies in the neuropathy.     

Cutaneous silent period in human immunodeficiency virus-related peripheral neuropathy

The aim of this work was first to determine whether the cutaneous silent period (CSP), a marker of small-nerve-fibre function, was altered in human immunodeficiency virus (HIV)-positive subjects with predominantly sensory symmetrical polyneuropathy and, second, to assess whether such alterations were predictive of an impairment in the largest calibre sensory and motor nerve fibres of the upper limb (UL) peripheral nerves. CSP was assessed in three groups of subjects: healthy control subjects, HIV-positive subjects with peripheral neuropathy (PN) of the lower limbs, and HIV-positive patients with clinical and neurophysiological involvement of the four limbs. CSP study showed a significant increase of the latency compared to the controls both in HIV-positive cases with no impairment in the UL (p=0.006) and in patients with four-limb neuropathy (p=0.002). CSP study in HIV-positive patients with mild lower limb distal sensory polyneuropathy can detect an early involvement of the UL peripheral nerves. CSP latency increase could therefore be addressed as the first sign of PN spreading to the UL.     

Motor chronic inflammatory demyelinating polyneuropathy (CIDP) in 17 patients: Clinical characteristics,electrophysiological study,and response to treatment

Motor chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare and poorly described subtype of CIDP. We aimed to study their clinical and electrophysiological characteristics and response to treatment. From a prospective database of CIDP patients, we included patients with definite or probable CIDP with motor signs and without sensory signs/symptoms at diagnosis. Patients were considered to have pure motor CIDP (PM‐CIDP) if sensory conductions were normal or to have motor predominant CIDP (MPred‐CIDP) if ≥2 sensory nerve action potential amplitudes were abnormal. Among the 700 patients with CIDP, 17 (2%) were included (PM‐CIDP n = 7, MPred‐CIDP n = 10); 71% were male, median age at onset was 48 years (range: 13‐76 years), 47% had an associated inflammatory or infectious disease or neoplasia. At the more severe disease stage, 94% of patients had upper and lower limb weakness, with distal and proximal weakness in 4 limbs for 56% of them. Three‐quarters (75%) responded to intravenous immunoglobulins (IVIg) and four of five patients to corticosteroids including three of three patients with MPred‐CIDP. The most frequent conduction abnormalities were conduction blocks (CB, 82%) and F‐wave abnormalities (88%). During follow up, 4 of 10 MPred‐CIDP patients developed mild sensory symptoms; none with PM‐CIDP did so. Patients with PM‐CIDP had poorer outcome (median ONLS: 4; range: 22‐5) compared to MPred‐CIDP (2, range: 0‐4; P = .03) at last follow up. This study found a progressive clinical course in the majority of patients with motor CIDP as well as frequent associated diseases, CB, and F‐wave abnormalities. Corticosteroids might be considered as a therapeutic option in resistant IVIg patients with MPred‐CIDP.     

腓骨肌萎缩症2F型1家系临床分析

目的探讨腓骨肌萎缩症(CMT)2F型的临床特点。方法对1家系内3例患者的临床表现和神经电生理资料进行回顾性分析。结果该家系为晚发(37~60岁),感觉障碍轻,1例伴右侧听力下降;神经传导速度检查示下肢感觉和运动传导速度减慢,甚至引不出反应波,但上肢正常或接近正常;躯体感觉诱发电位示中枢神经和周围神经均受累,运动诱发电位示运动通路周围段传导减慢,脑干听觉诱发电位示一侧听通路周围段严重受累。结论CMT2F型患者的临床特征有助于CMT患者的诊断和分型。     

Phenotypic heterogeneity in hereditary motor neuropathy type V: a new case report series

Previous studies have revealed a wide phenotypic heterogeneity in hereditary motor neuropathy type V in which upper and lower motor neurons and peripheral motor axons are variously affected, even within the same family. In this case series, we describe the genetic, clinical and electrophysiological features of patients belonging to a four-generation Italian family. Because of a possible anticipation phenomenon, the disorder became apparent at an earlier age as it passed to the next generation, with a median age of onset of 65?years for the first 2 generations, 32 for the third, and 13.5 for the fourth. The symptoms at onset varied considerably among the sufferers, with a predominant impairment of the hands in seven cases, the impairment of the four limbs in one patient and only of the lower limbs in another. Also muscle atrophy was variable, from very mild to severe (wasting of the distal muscles of the limbs). Moreover, electrophysiological results were heterogeneous, including cases with isolated and with diffuse axonal motor neuropathy, and one case of motor sensory polyneuropathy. A novel polymorphism G→T was also found in the Berardinelli-Seip congenital lipodystrophy 2 gene on intron 4. This broad phenotypic and genotypic spectrum calls the clinician attention to this rare and still insufficiently known disease.     

老年Miller-Fisher综合征患者的临床和神经电生理特点分析

目的研究老年Miller-Fisher综合征(MFS)患者的临床和电生理特征,并分析其预后。方法对9例老年MFS患者的临床表现、辅助检查、治疗和预后进行回顾性总结分析。结果本组男女比例4:5,患病年龄(68.3±4.1)岁,病程(19.2±9.9)d,发病主要在春冬季节,有呼吸道感染前驱史者3例,首发症状主要为头晕、头痛、眼睑下垂、复视、行走不稳和肢体麻木无力,起病至达峰平均(2.7±2.8)d;患者均有眼肌麻痹、共济失调和腱反射减弱或消失,眼胀痛5例,眼内肌受累2例,面神经受累4例,舌咽、迷走神经受累3例,四肢肌力减退3例,远端浅感觉减退或消失6例,自主神经受累3例;血清抗GQ1b抗体阳性5例,脑脊液示蛋白细胞分离6例,四肢肌电图异常7例,主要表现为感觉神经传导受累5例;患者应用激素和(或)免疫球蛋白治疗,头痛、眼胀痛、肢体麻木症状最早改善,眼外肌麻痹、共济失调分别在(2.5±1.5)d、(6.3±3.8)d时开始改善,腱反射异常在2~3周出院时无明显恢复;患者住院(15.0±4.6)d,出院时均好转。结论老年MFS临床表现多样,肌电图主要表现为感觉神经传导受累,血清抗GQ1b抗体有助于诊断,激素和(或)免疫球蛋白治疗后好转。     

MILD CARPAL TUNNEL SYNDROME: EARLY ELECTROPHYSIOLOGICAL DIAGNOSIS AND POST SURGICAL FOLLOW UP

We studied 476 patients affected by diabetes: 166 male (mean age 61.6 ± 10 years, range 27–91) and 310 female (mean age 61.5 ± 8.4 years, range 25–82). Mean disease duration was 11.3 ± 7.6 years, range 0.3–37).
All patients underwent surface motor and sensory nerve conduction along median, popliteal, and sural nerve.
Results. Median nerve: in 3.1% of subjects sensory action potentials (SAP) was absent; sensory nerve conduction velocity (SNCV) was reduced in 41.8% in distal segment and in 27.5% in the proximal segment. Motor nerve conduction (MNCV) was reduced in 29.9% of the subjects.
Sural nerve: SAP was absent in 24.4% and SNCV was reduced in 32.7%.
Popliteal nerve: MNCV was abnormal in 30.4% of the subjects. Combining electrophysiological data we observed that:
1. 28.6% of the subjects resulted normal
2. 12.8% were affected by a lower limbs sensory neuropathy
3. 0.2% had a lower limbs motor neuropathy
4. 5.9% had a lower limbs sensory-motor neuropathy
5. 6.1% had a diffused sensory neuropathy
6. 30.2% had a diffused sensory-motor neuropathy
7. 16.2% had a carpal tunnel syndrome.
Patients were divided in 2 groups: patients with and patients without neuropahy: the latter showed a significantly shorter disease duration (12.7 ± 8.1 vs 9.0 ± 6.3; p < 0.0001).
In addition, we observed a significant correlation between disease duration and distal latency, median and popliteal MNCV, and SNCV in median and sural nerve (Regression test; p < 0.0001).
Patients on insulin showed a longer disease duration and more severe electrophysiological abnormalities.     

Peripheral nerve involvement in primary systemic AL amyloidosis: a clinical and electrophysiological study

Background: Involvement of visceral organs usually dominates the clinical picture of primary systemic AL amyloidosis, but some patients suffer from serious peripheral neuropathy. The aim of this study is to clinically and electrophysiologically investigate peripheral nerve involvement in AL amyloidosis patients. Patients and methods: We reviewed clinical manifestations, electrophysiological findings including nerve conduction velocities and treatments in 43 consecutive patients. Twenty age‐matched healthy subjects were employed as controls. Results: Fifteen patients (34.9%) showed apparent neuropathic symptoms, which consisted of polyneuropathy in 11 (25.6%), bilateral carpal tunnel syndrome in 4 (9.3%), and autonomic dysfunction in 8 (18.6%). Polyneuropathy in this disease was characterized by symmetrical and sensory‐dominant impairment, early involvement of the lower limbs, loss of all sensations, rarity of motor weakness, and painful paresthesia in the legs predominant at an early stage. Autonomic dysfunction including orthostatic hypotension was frequently associated with polyneuropathy at an advanced stage. On electrophysiological studies, motor conduction velocity and compound muscle action potential of both median and tibial nerves were significantly decreased in the patients with polyneuropathy but also in those without any signs of neuropathy. Only four of 15 patients with neuropathy were able to receive intensive but promising chemotherapy with a large dose of melphalan for plasma cell dyscrasia. Conclusions: Peripheral nerves in primary systemic AL amyloidosis patients seem to be involved more extensively than clinical manifestations might suggest. The clinical picture of polyneuropathy in this disease closely resembles that in transthyretin‐type familial amyloid polyneuropathy patients with a late age at onset, particularly those originating from sporadic kindreds.     

Chronic distal spinal amyotrophy or spinal forms of Charcot-Marie disease. A report on six sporadic adult cases (author's transl)

Based on a personal series of 6 cases, and about 20 cases reported in the published literature, the authors describe the principal characteristics of chronic distal amyotrophy of spinal origin: early onset or in young adults, initial disorder in lower limbs, especially anterolateral region of the legs, with secondary lesions in small muscles of the hands, absence of sensory disturbance, usually very slow progression, electrophysiological appearance of chronic denervation with normal conduction velocities, and neurogenic muscle lesions with unaffected peripheral nerve. These forms can be compared to the "spinal" forms of Charcot-Marie-Tooth disease and appear to be degenerative in origin even though proof by autopsy is lacking. In spite of their common characteristics, however, their genetic heterogenicity has to be noted: sporadic cases are seen most frequently but some cases are related to recessive autosomic transmission and others to dominant autosomic transmission. From the clinical point of view, some cases are distinctive in that there is early or predominant atrophy in the lower limbs, while others have lesions in muscles of bulbar innervation, which is against their origin from spinal amyotrophy. Finally, electrical signs at a distance from the atrophied muscles suggest extension of the pathological process. In spite of these comments, the authors suggest that the clinical concept of the distal form of spinal amyotrophy should be retained together with the proximal and scapuloperoneal forms.     

The diagnosis of chronic axonal polyneuropathy: the poorly understood chronic polyradiculoneuritides

Chronic inflammatory demyelinating polyradiculoneuropathy is an autoimmune disease that target myelin sheats of peripheral nerves. Its diagnosis is often difficult to make, and a number of cases are probably not identified because of the clinical and electrophysiological heterogeneity. Typical cases associate progressive or relapsing-remitting motor and sensory deficit with increased CSF protein content and electrophysiological features of demyelination. In some cases electrophysiological studies fail to show evidence of demyelination, conventional electrophysiological diagnostic criteria are not filled yet the patient may respond to immunomodulatory treatments. In such cases, presence of clinical characteristics suggestive of CIDP (that means not compatible with a length-dependent axonal process) are critical justifying fully investigations including sural nerve biopsy. The main clinical characteristic are: a symmetric proximal and distal motor weakness predominantly affecting the lower limbs, a diffuse areflexia, a sensory deficit characterized by a preferential involvement of large fibers, an evolution which may be either chronic progressive or recurrent. Usual therapeutic agents (corticosteroids, intravenous immunoglobulins, plasma exchanges) seem to have the same efficacy whatever the electrophysiologic profile.     

Peripheral neuropathy in patients with beta-thalassaemia.

As some patients with beta-thalassaemia manifested neurological signs, clinical and electrophysiological investigations were carried out on 53 thalassaemic patients and 29 healthy control subjects. Twenty per cent of the patients showed clinical and electrophysiological findings of a mild peripheral sensorimotor neuropathy, mainly of the lower limbs. The clinical symptoms were numbness, pins and needles sensations, muscular cramps, myalgia and muscle weakness. The electrophysiological abnormalities were manifested by decreased motor conduction velocity (MCV) and prolonged F-wave latencies of the tibial and the peroneal nerves. Borderline increase in the latencies of the sensory potentials of the median nerve was also observed. The electromyographic findings of the patients with diminished MCVs were compatible with a predominantly motor peripheral neuropathy. This neuropathy appears during the second and third decade of life.     

Pain as the presenting symptom of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

Numerous clinical forms of CIDP have been described, but pain is generally considered a rare or secondary sign. We describe here the clinical, electrophysiological and neuropathological characteristics of five patients with CIDP and pain as the main presenting symptom, and their course with treatment. Between January 2003 and December 2004, we selected five patients with prominent or isolated pain among 27 patients diagnosed with CIDP. All patients were subjected to clinical and electrophysiological examinations, and had a complete laboratory work up to exclude other causes of neuropathy. In view of the atypical clinical presentation, all five patients underwent nerve biopsy. There were two men and three women. The mean age at onset of neuropathy was 70+/-7.39 years. All patients initially presented with pain in the lower limbs associated with modest motor impairment (1 case), distal paresthesia (4 cases), cramps (1 case) and fatigue (2 cases). CSF was normal in three cases. On electrophysiological examination, three patients had nerve conduction abnormalities with subtle or clear signs of demyelination: three (case 1, 2 and 4) fulfilled the criteria of Rotta et al. and two (case 2 and 4) the criteria of both Nicolas et al and the INCAT group. Patients were all given symptomatic treatment and four patients received an immunomodulatory treatment, which was constantly effective. Pain may be a major and disabling symptom in patients with CIDP, so this diagnosis has to be considered in patients referred for a painful polyneuropathy. Moreover, immunomodulatory treatment has to be considered in such patients as symptomatic therapy may be ineffective.     

Minimal and asymptomatic chronic inflammatory demyelinating polyneuropathy.

OBJECTIVES: Show the chronic inflammatory demyelinating polyneuropathy (CIDP) is not only clinically heterogeneous but extremely variable in severity. METHODS: Three patients were referred for mild distal paresthesiae lasting more than 6 months and one for inguinal and thigh pain later ascribed to coxarthrosis. Strength was normal in all patients and tactile sensation reduced distally only in one. Tendon jerks were absent, except the knee jerks in one patient, reduced in lower limbs in two and normal in one. RESULTS: Electrophysiology showed a demyelinating neuropathy without motor conduction block. CSF protein content was increased in all patients. Nerve biopsies showed de-remyelination with varying degrees of axonal loss. Genetic studies excluded a demyelinating neuropathy associated with duplication or deletion of the 17p.11.2 segment. CONCLUSIONS: CIDP patients with pure sensory clinical presentation have been described but are generally more severely impaired. However, because of the mildness of symptoms and the unequivocal electrophysiological involvement of motor fibers, we think that in these cases the term minimal CIDP is more appropriate than sensory CIDP. These cases represent the most benign end of the CIDP spectrum. In our series minimal or even asymptomatic CIDP encompasses 8% of cases.     

Challenges in the identification of cobalamin-deficiency polyneuropathy

BACKGROUND: Diagnosing cobalamin (Cbl) deficiency as a cause of polyneuropathy (PN) is problematic, as the frequency of both disorders increases with age, and serum Cbl levels can be difficult to interpret. OBJECTIVES: To identify unique clinical or laboratory features among PN patients with Cbl deficiency and to examine the role of testing of serum metabolite levels in the identification of Cbl deficiency. DESIGN: Cohort survey comparing patients with Cbl deficiency and cryptogenic PN identified during a 2-year period. Cobalamin deficiency was diagnosed using low serum Cbl levels or elevated serum methylmalonic acid or homocysteine levels. SETTING: Academic neuromuscular clinic. RESULTS: Of 324 PN patients, 27 were diagnosed as having Cbl deficiency. Twelve had Cbl levels within the normal range, but elevated serum metabolite levels. Compared with patients with cryptogenic sensory/sensorimotor PN, those with Cbl deficiency were more likely to have concomitant involvement of the upper and lower extremities and experience symptom onset in the hands and a sudden onset of symptoms (P<.005). These differences were seen regardless of whether Cbl deficiency was defined using low Cbl levels or elevated serum metabolite levels. Autoimmune pernicious anemia was identified in 6 (50%) of 12 Cbl-deficient patients with normal serum Cbl levels. The patients with PN and Cbl deficiency showed little objective improvement after parenteral replacement therapy; however, progression occurred less often in these patients compared with those with cryptogenic sensory/sensorimotor PN (P =.02). CONCLUSIONS: This study highlights the challenges of proving that Cbl deficiency is the cause for PN and identifies clinical features that suggest Cbl-deficiency PN. Testing of serum metabolite levels may identify Cbl deficiency in some patients with normal serum Cbl levels.     

Acute small fibre sensory neuropathy: another variant of Guillain-Barré syndrome?

Six patients who presented with acute sensory neuropathy were studied. All patients underwent detailed clinical assessment along with electrophysiological tests and relevant laboratory investigations. All patients had acute onset numbness, reaching the peak deficit within 4 weeks. Four of them had associated burning dysaesthesia. An antecedent illness was reported in four; diarrhoea in three, and urinary tract infection in one. The neurological examination disclosed normal muscle strength, symmetric glove and stocking type sensory loss for pain and temperature, normal proprioception, and vibration senses with normal or brisk tendon reflexes. Analysis of CSF demonstrated albuminocytological dissociation in all. Routine motor and sensory nerve conduction studies were normal. Sympathetic skin responses were also normal except for the lower limbs in one patient. Stool cultures for Campylobacter jejuni were negative. The outcome was favourable. Burning dysaesthesia disappeared within 4 months. Numbness and objective sensory loss tended to persist longer. The clinical features and normal routine nerve conduction studies, which assess large diameter nerve fibre function, indicate small sensory fibre dysfunction in the group. Their presentation and CSF findings would fit into the diagnosis of sensory Guillain-Barré syndrome. The current study suggests that acute small fibre sensory neuropathy (ASFSN) is another clinical entity which could perhaps be included in the heterogeneous range of Guillain-Barré syndrome.     

Electrophysiological evaluation in 4 patients with diabetic pseudotabes

BACKGROUND: Researches on diabetic nervous system lesion are mainly focus on peripheral nerve and vegetative nerve, so there are few investigations on diabetic pseudotabes. OBJECTIVE: To investigate the electrophysiological examinations on the diagnosis of diabetic pseudotabes. DESIGN: Case study. SETTING: Department of Electrophysiology and Department of Neurology, Zhongshan Hospital Affiliated to Xiamen University. PARTICIPANTS: A total of 4 patients with type 2 diabetes mellitus, including 3 males and 1 female aged from 50 to 72 years, were selected from Department of Neurology, Zhongshan Hospital Affiliated to Xiamen University from March 2002 to February 2005. All accepted subjects met the modified diagnostic criteria of diabetes mellitus, which was set by American Diabetes Mellitus Association (ADA) in 1997. Otherwise, the subjects had typical symptoms and physical signs of spinal posterior funiculus damage. However, patients with spinal cord lesion which was caused by other factors were excluded. All accepted subjects provided the confirmed consent. METHODS: Nicolet NT electromyography (EMG)/evoked potential meter (made in the USA) was used to detect spinal cord conduction velocity (SCCV), somatosensory evoked potential (SEP) of lower limbs, motor nerve conduction velocity (MNCV) and sensory nerve conduction velocity (SNCV) of extremities. Determining criteria: Measurements were performed based on the laboratory standards. SCCV, which was less than lower limit of normal value (T2–12: 40–55 m/s, T12–L4: 20–41 m/s, T2–L4: 36–45 m/s), was regarded as abnormal. SEP value of lower limbs: P40, P60 and PF, which were more than standard deviation of normal value (x(—)+2.5), were regarded as the abnormality. Normal value of P40, P60 and PF latencies (x(—)±s) in this study: P40, P60 and PF in males were (37.6±1.9) ms, (59.8±3.9) ms and (7.6±0.9) ms, respectively; meanwhile, those in females were (35.5±1.7) ms, (55.2±2.7) ms and (6.3±0.7) ms, respectively. MNCV and SNCV, which were less than 50 m/s in upper limbs and 40 m/s in lower limbs, were regarded as the abnormality. MAIN OUTCOME MEASURES: Electrophysiological examinations. RESULTS: All 4 patients with type 2 diabetes mellitus were involved in the final analysis. ① SCCV: Among 4 patients, SCCV of three patients was decreased in T2–12, T12–L4 and T2–L4, and that of the other one was decreased in T2–12 and T2–L4; however, SCCV in T12–L4 was normal. There was significant difference as compared with normal value (P < 0.01). ② SEP of lower limbs: SEP values of lower limbs were abnormal in all 4 patients. Among them, P40, P60 and PF latencies of two patients were delayed; P40 of one patient was delayed and PF was not drained out; P40 and P60 of the last one were delayed and PF was normal. ③ MNCV and SNCV: The MNCV and SNCV were normal in one patient and abnormal in three patients. The results demonstrated that MNCV and SNCV of extremities decreased; especially, sensory nerve action potential (SNAP) of both lower extremities of one patient were not drained out. CONCLUSION: Detections of SCCV, SEP of lower limbs, MNCV and SNCV of extremities are helpful to investigate whether peripheral nerve and deep sensory passage are damaged or not and determine whether deep sensory damage is caused by peripheral nerve and spinal posterior funiculus.