Ali, S

Firdaus, H

Sher, K.

English

eCommons@AKU

Pakistan Journal ofNeurological Sciences (PJNS)Volume 5 | Issue 1 Article 34-2010Spectrum of Chronic Acquired ImmuneNeuropathiesK. SherJinnah Postgraduate Medical CentreH FirdausJinnah Postgraduate Medical CentreS AliJinnah Postgraduate Medical CentreFollow this and additional works at: https://ecommons.aku.edu/pjnsPart of the Neurology CommonsRecommended CitationSher, K.; Firdaus, H; and Ali, S (2010) "Spectrum of Chronic Acquired Immune Neuropathies," Pakistan Journal of NeurologicalSciences (PJNS): Vol. 5 : Iss. 1 , Article 3.Available at: https://ecommons.aku.edu/pjns/vol5/iss1/3INTRODUCTIONNeuropathies are routinely classified as demyelinating oraxonal, based on the presence or absence ofdemyelinating abnormalities on electrodiagnostic testing.The presence of conduction block/temporal dispersionreflects acquired nature of immune neuropathies and hasbeen reported in patients with generalized, distal andasymmetric phenotypic pictures.    Chronic acquiredimmune polyneuropathies are heterogeneous group ofdisorders, which may present in a variety of ways, besidesthe typical symmetric pattern observed in ChronicInflammatory Demyelinating Polyneuropathy (CIDP). It istherefore important to appreciate different clinicalpresentations of underly ing immune mediatedneuropathies because majority of these are potentiallytreatable entities.MATERIAL AND METHODSPlace of studyThis study was carried out at Jinnah Post GraduateMedical Centre in Karachi over a period of 2 years fromJune 2006 to June 2008. This was a prospective clinicalstudy.Inclusion criteriaAll the adult male and female patients who presented withchronic proximal and/or distal weakness of more then onelimbs in symmetric or asymmetric fashion were included inthe study.Exclusion criteriaAny of the patients with one or more of the followingfeatures was excluded from the study:1) History of diabetes mellitus, uremia, chronic liver disease or alcoholism.2) Positive family history of similar illness.3) Any patient with foot deformities, sphincter impairment, mutilation of hands and feet, icthyosis, or retinitis pigmentosa.4) History of drug or toxic exposure likely to cause neuropathy.MethodsPatients with chronic acquired neuropathies withneurophysiological features of segmental demyelination inany combination, i.e. conduction block (CB), slowing ofconduction across the affected segment (proximal and/ordistal), prolonged distal latency and/or temporal dispersion(TD) were evaluated over a period of 2 years. Those whoV O L .  5 ( 1 )  J A N  -  M A R   2 0 1 0P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S  6O R I G I N A L  A R T I C L ESPECTRUM OF CHRONIC ACQUIRED IMMUNENEUROPATHIESKhalid Sher, H Firdaus and S AliDepartment of Neurology, Jinnah Postgraduate Medical Centre, Karachi, PakistanCorrespondence to: Dr Sher, Department of Neurology, Jinnah Postgraduate Medical Centre, Rafiqi Shaheed Road, Karachi, Pakistan.ABSTRACTObjective: To determine the different clinical presentations of patients with acquired immune mediated neuropathies.Methodology: This was a hospital based prospective study over a period of 2 years in a series of patients withelectrophysiological evidence of chronic acquired immune neuropathies. Results: Males out-numbered females by 7 (19 vs12). Majority of patients with chronic acquired immune neuropathies were found to have typical symmetric sensorimotorpattern with variable involvement of proximal and distal muscles. The sensory pattern was the next common patternfollowed by asymmetric pattern consistent with madsaM neuropathy. Conclusion: Because immune mediated neuropathiesare potentially treatable entities, it is essential to keep in mind their unusual presentations besides the typical symmetricpattern of weakness of limb girdle muscles.Pak J Neurol Sci 2010; 5(1): 6-9V O L .  5 ( 1 )  J A N  -  M A R   2 0 1 0P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S  7met revised neurophysiological criteria (Nicolas et al.) forCIDP were clinically categorized into different groups.The following segments were considered for analysis: tibal(ankle and popliteal fossa), peroneal (ankle and below thefibular head), ulnar (wrist and below elbow), and mediannerve (wrist and elbow). Electro diagnostic changes thatwere considered features of demyelination were similar tothose proposed by American Academy of Neurology (AAN)committee (AAN, 2001), but with more stringent criteriafor partial conduction block/temporal dispersion (NICOLASET AL). Revised neurophysiological criteria by Nicolas et al.were categorized into following four groups.GROUP 1:CB/TD must be present in one nerve and abnormalconduction values in at least two other nerves.GROUP 2:CB/TD must be present in two different nerves andabnormal conduction values in one other nerve.GROUP 3:CB/TD present in at least 3 different nerves with abnormalconduction values suggesting demyelination in at leastone nerve including one of the nerves with CB/TD.GROUP 4:No CB/TD but abnormal conduction values must bepresent in three different nerves.CSF Study was also performed in all the patients andthose with CSF content of more than 100 mg/dl werecategorized as having high protein content.The muscle strength was measured manually in abductorsof shoulders, flexors and extensors of neck, elbows, wrists,fingers, hips, ankles and toes by means of the MedicalResearch Council (MRC) scale. Functional impairment was assessed with modified RankinDisability scale:0) Asymptomatic.1) Non-Disabling symptoms that don’t interfere with activities of daily living..2) Slight Disability (unable to carry out all activities, such as running, but still able to look after themselves).3) Moderate disability (but able to walk without assistance).4) Moderately severe disability (unable to walk without assistance).5) Severe Disability (Dependant)For purpose of analysis patients with generalized (pattern)neuropathy were defined as having either symmetricproximal weakness or proximal plus distal weakness ofmajor muscle groups.Patients with distal pattern were defined as havingsymmetric distal large fiber sensory loss (Romberg’spositive) or glove stocking numbness with or without distalweakness.The asymmetric neuropathy was defined as having motorasymmetry, which differs by at least one grade in aminimum of 2 muscle groups, based on the MRC scale.RESULTS1) Age and SexOut of total 31 patients, 19 were males and 12 werefemales. Patients were categorized into different agegroups where in majority were found between 40-60 yearsof age.2)  Clinical VariantOf the 31 patients, majority of patients were found to havegeneralized pattern followed by distal pattern.3)  CSF AnalysisCSF analysis revealed protein concentration of <100mg/dl in majority of patients. Four out of 7 patients withdistal pattern had CSF protein concentration >100 mg/dl.4)    Weakness pattern in different VariantsOut of 21 patients with generalized pattern, 10 hasequally symmetric proximal as well as distal weaknessfollowed by proximal > distal pattern. Patients with distalTABLE 1. Demographic details and distribution of various clinicalvariants of neuropathy in the sample population.Male Female Totaln=19(%) n=12(%) n=31(%)Under 30 2(10.5%) 1(8.3%) 3(9.7%)30-39 5(26.3%) 2(16.7%) 7(22.6%)40-49 1(5.3%) 6(50.0%) 7(22.6%)50-59 8(42.1%) 2(16.7%) 10(32.3%)60 & above 3(15.8%) 1(8.3%) 4(12.9%)CIDP 12(63.2%) 9(75.0%) 21(67.7%)MADSAM 1(5.3%) 1(8.3%) 2 (6.5)DADS 5(26.3%) 2(16.7%) 7(2.3%)MMCB 1 (2.3%) 0 1(13.2%)AgeClinicalVariantpattern were found to be more ataxic rather than havingglove and stocking sensory pattern.5)    Functional stateMajority of patients with generalized pattern were found tohave moderate disability compared to distal pattern whowere mainly found to have slight disability.6)    Electro-diagnostic CriteriaPatients with generalized pattern were found to haveCB/TD in majority of cases (group 2 & 3). CB/TD wasinfrequent with distal pattern of CIDP.DISCUSSIONCIDP is a cl inical ly heterogeneous group ofpolyneuropathies united by their presumed immunemediated etiopathogenesis. In clinical practice, it isunusual to take into consideration different clinicalphenotypes of CIDP while coming across patients withneuropathic symptoms. However distinction betweenthese subgroups is of immediate practical relevance topatient management.2 The disorders, which have somecharacteristics unique but otherwise have clinical,electrophysiologic, laboratory and therapeutic aspectssimilar to CIDP, are considered variants.1Since the first consensus criteria proposed by theAmerican Academy of Neurology in 1991, numerousauthors have proposed alternative ones.10 However asthere is no gold standard parameter for diagnosis of CIDP,we selected the proposed revised electrophysiologicalcriteria by Nicolas et al.  for diagnostic purpose.5The disease is believed to more likely affect all age groupsbut is more common in older males.7 Our study foundsimilar pattern wherein 21/31 patients were above 40 atthe time of diagnosis. CIDP is traditionally distinguishedfrom chronic length dependent polyneuropathies byuniform muscle weakness (proximal as well as distal) ofupper and lower limb with generalized areflexia.10Similar pattern of global weakness of upper and lowerlimbs was noticed in majority of our patients (32%)followed by proximal more than distal (22.6%) and thendistal more than proximal (12%) patterns. Thus it isimportant to keep in mind that predominant proximal ordistal weakness with hyporeflexia or areflexia with orwithout sensory symptoms may be presenting patterns ofCIDP.As CIDP is heterogeneous in presentation with unusualpatterns like ataxic and distal asymmetric variants,8 wealso came across such patterns in our series of patients.Typically a pure or predominant sensory presentation havealso been reported to occur in presence of marked motornerve slowing with relative preservation of motor function.Our patients with DADS pattern were ataxic in 42% (3/7)of case whereas pure distal numbness was found in 28%(2/7) of cases .One patient in this group was found tohave more distal sensory motor involvement of hands thanfeet.Two out of thirty patients were found to have distalasymmetric pattern, proportion corresponding to series ofpatients studied by M. Bushby et al.2V O L .  5 ( 1 )  J A N  -  M A R   2 0 1 0P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S  8TABLE 2. Clinical spectrum of various neuropathiesCIDP MADSAM DADS MMCBn=21 n=2 n=7 n=1Distributionof WeaknessFunctionalStatusCSFProtein(mg/l)ElectroDiagnosticCriteria(Nicolas et al.)Proximal=Distal(P=D)10(47.6%)Proximal>Distal(P>D)Distal>Proximal(D>P)Slightdisability(grade 2)Moderatedisability(grade 3)DistalAtaxicDistalNumbnessDistal(UL)Grade 1Under 5051-100>100Grade 2Grade 3Grade 4Asymmetric(D/P)SevereDisability(grade 4)6(28.6%)5(23.8%)9(42.9%)2(2.5%)1(50%)1(50%)6(28.6%)6(28.6%)3(14.3%)5(23.8%)10(47.6%)6(28.6%)2(29%)4(57%)1(100%)1(50%)1(50%)1(14%)10(47.6%)1(50%)1(50%)5(71%)2(29%)6(86%)1(14%)1(100%)1(100%)1(14%)3(43%)2(29%)1(14%) 1(100%)2(100%)CSF study was carried out in all patients and interestingly,the proportion of patient with protein content of more than100 mg/dl was higher in those with DADS than in thosewith typical CIDP pattern. This needs further elucidation.Because conduction block and temporal dispersion arehighly suggestive of segmental demyelination, thereforewe selected the proposed revised neurophysiologicalcriteria by Nioles et al. which gives special emphasis tomarkers of demyelination.5It came to our notice that patients with diffuse weaknesswere found to have increased proportion of patients withCB/TD than those with distal sensory or ataxic patterns.This observation suggests that all electrophysiologicfeatures of demyelination are important to look for indiagnostic neurophysiologic studies.The aim of this study was to enhance awareness aboutclinical profile of CIDP, which is usually considered uniformand symmetric neuropathic entity.REFERENCES1. Gorson KC, Alam G, Ropper AH. Chronic inflammatory demyelinating  polyneuropathy: Clinicalfeatures and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology 1997;48:321-28.2. Mark Busby, Michael Donaghy. Chronic dysimmune neuropathy. J Neurol 2003;250:714-724.3. Mainsonobe T, Chassanda B, Verin M, Jouni M, LergerGM, Bouche P. Chronic dysimmue demyelinatig polyneuropathy; a clinical and electrophysiological study of 93 patients. J Neurol Neurosurg Psychiatry 1996;61:36-42.4. Molenaar DS, Vermeulen M, de Haan RJ. Comparison of electrodiagnosiic criteria for demyelination in patients with chronic inflammatory demyelinating polyneuropathy. J Neurol 2002;249:400-3.5. Nicolas G, Maisonobe T, Le Forestier N, Léger JM, Bouche P. Proposed revised electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 2002;25:26-30.6. Oh SJ, Joy JL, Kuruoglu R. Chronic inflammatory demyelinating polyneuropathy presenting as pure sensory neuropathy. J Neurol Neurosurg Psychiatry 1992;55:677-80.7. Lewis RA. Chronic inflammatory demyelinating polyneuropathy. Neurol clin 2007;25:71-87.8. Saperstein DS, Katz JS, Amato AA, Barohn RJ. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve 2001 Mar;24(3):311-24.9. Sinnreich M, Klein CJ, Daube JR, Engelstad J, Spinner RJ, Dyck PJ. Chronic immune sensory polyradiculopathy: a possibly treatable sensory ataxia. Neurology 2004 Nov 9;63(9):1662-9.10. French CIDP Study Group, Vallat JM. Recommendations on diagnostic strategies for chronic inflammatory demyelinating polyradiculoneuropathy; Postgrad Med J 2008 Jul;84(993):378-81.V O L .  5 ( 1 )  J A N  -  M A R   2 0 1 0P A K I S T A N  J O U R N A L  O F  N E U R O L O G I C A L  S C I E N C E S  9

Spectrum of Chronic Acquired Immune Neuropathies

https://ecommons.aku.edu/cgi/viewcontent.cgi?article=1312&amp;context=pjns

Spectrum of Chronic Acquired Immune Neuropathies

Abstract

Similar works

Full text

Available Versions

eCommons@AKU