Neuromuscular Disorders

Article	Citations
Newborn screening programs for spinal muscular atrophy worldwide: Where we stand and where to go	90
The 2021 version of the gene table of neuromuscular disorders (nuclear genome)	45
Fatigue in patients with myasthenia gravis. A systematic review of the literature	39
Randomized phase 2 trial and open-label extension of domagrozumab in Duchenne muscular dystrophy	39
Combining genetics, neuropsychology and neuroimaging to improve understanding of brain involvement in Duchenne muscular dystrophy - a narrative review	38
The 2022 version of the gene table of neuromuscular disorders (nuclear genome)	36
Recent advances in nemaline myopathy	36
Guillain-Barré-Strohl syndrome and COVID-19: Case report and literature review	31
X-linked myotubular myopathy	31
Age related treatment effect in type II Spinal Muscular Atrophy pediatric patients treated with nusinersen	28
Congenital muscular dystrophies: What is new?	26
Chronic pain is common in mitochondrial disease	25
Age and baseline values predict 12 and 24-month functional changes in type 2 SMA	24
Strength-training effectively alleviates skeletal muscle impairments in myotonic dystrophy type 1	23
The DMD gene and therapeutic approaches to restore dystrophin	23
Understanding European patient expectations towards current therapeutic development in spinal muscular atrophy	23
Clinical practice guidelines for glycogen storage disease V & VII (McArdle disease and Tarui disease) from an international study group	21
Gain and loss of abilities in type II SMA: A 12-month natural history study	21
Cognitive impairment appears progressive in the mdx mouse	20
Disease-modifying effects of edasalonexent, an NF-κB inhibitor, in young boys with Duchenne muscular dystrophy: Results of the MoveDMD phase 2 and open label extension trial	19
The importance of an integrated genotype-phenotype strategy to unravel the molecular bases of titinopathies	18
Two Japanese LGMDR25 patients with a biallelic recurrent nonsense variant of BVES	18
Miyoshi myopathy and limb girdle muscular dystrophy R2 are the same disease	18
Sarcoglycanopathies: an update	18
Congenital myasthenic syndromes: where do we go from here?	18

Nusinersen treatment of older children and adults with spinal muscular atrophy	17
Immune checkpoint inhibitors (ICIs)-related ocular myositis	17
Impact of a national population-based carrier-screening program on spinal muscular atrophy births	16
Long-term follow-up of patients with type 2 and non-ambulant type 3 spinal muscular atrophy (SMA) treated with olesoxime in the OLEOS trial	15
Efficacy and safety of mexiletine in non-dystrophic myotonias: A randomised, double-blind, placebo-controlled, cross-over study	14
Clinical and genomic characteristics of LAMA2 related congenital muscular dystrophy in a patients’ cohort from Qatar. A population specific founder variant	14
Mortality rates and causes of death in Swedish Myasthenia Gravis patients	14
SARS-CoV-2 vaccination and new-onset myasthenia gravis: A report of 7 cases and review of the literature	14
Facioscapulohumeral muscular dystrophy 1 patients participating in the UK FSHD registry can be subdivided into 4 patterns of self-reported symptoms	14
Clinical and histological features of immune-mediated necrotising myopathy: A multi-centre South Australian cohort study	14
Spinal muscular atrophy: from rags to riches	13
Prevalence and associated factors of fatigue in autoimmune myasthenia gravis	13
Bilateral facial palsy with paresthesias, variant of Guillain-Barré syndrome following COVID-19 vaccine: A case series of 9 patients	13
A review of core myopathy: central core disease, multiminicore disease, dusty core disease, and core-rod myopathy	13
249th ENMC International Workshop: The role of brain dystrophin in muscular dystrophy: Implications for clinical care and translational research, Hoofddorp, The Netherlands, November 29th–December 1st	13
Fatigue, physical activity and associated factors in 779 patients with myasthenia gravis	12
Next-generation sequencing application to investigate skeletal muscle channelopathies in a large cohort of Italian patients	12
A novel heterozygous mutation in the C-terminal region of HSPB8 leads to limb-girdle rimmed vacuolar myopathy	12
Novel TNNT1 mutation and mild nemaline myopathy phenotype in an Italian patient	12
The 2023 version of the gene table of neuromuscular disorders (nuclear genome)	12
Real-world and natural history data for drug evaluation in Duchenne muscular dystrophy: suitability of the North Star Ambulatory Assessment for comparisons with external controls	12
Dysphagia in adult myopathies	12
Revised upper limb module in type II and III spinal muscular atrophy: 24-month changes	12
Early safety and tolerability profile of the BNT162b2 COVID-19 vaccine in myasthenia gravis	11
Facioscapulohumeral muscular dystrophy type 2: an update on the clinical, genetic, and molecular findings	11
256th ENMC international workshop: Myositis specific and associated autoantibodies (MSA-ab): Amsterdam, The Netherlands, 8-10 October 2021	11
Expanding the disease phenotype of ADSSL1-associated myopathy in non-Korean patients	11
247th ENMC International Workshop: Muscle magnetic resonance imaging - Implementing muscle MRI as a diagnostic tool for rare genetic myopathy cohorts. Hoofddorp, The Netherlands, September 2019	11
Physical training and exercise in myasthenia gravis	11
Electrophysiological study of neuromuscular junction in congenital myasthenic syndromes, congenital myopathies, and chronic progressive external ophthalmoplegia	11
Beyond ambulation: Measuring physical activity in youth with Duchenne muscular dystrophy	11
Evaluation of real-life outcome data of patients with spinal muscular atrophy treated with nusinersen in Switzerland	11
Rituximab in myasthenia gravis: efficacy, associated infections and risk of induced hypogammaglobulinemia	11
Pre-operative exercise and pyrexia as modifying factors in malignant hyperthermia (MH)	11
COVID-19 in advanced Duchenne/Becker muscular dystrophy patients	11
Glucose and lipid metabolism disorders in children and adolescents with spinal muscular atrophy types 2 and 3	11
Longitudinal data of neuropsychological profile in a cohort of Duchenne muscular dystrophy boys without cognitive impairment	11
Effectiveness of thymectomy in juvenile myasthenia gravis and clinical characteristics associated with better outcomes	11
242nd ENMC International Workshop: Diagnosis and management of juvenile myasthenia gravis Hoofddorp, the Netherlands, 1–3 March 2019	11
The endocrine manifestations of spinal muscular atrophy, a real-life observational study	10
Feeding difficulties in children and adolescents with spinal muscular atrophy type 2	10
Pig models for Duchenne muscular dystrophy – from disease mechanisms to validation of new diagnostic and therapeutic concepts	10
The glycomic sialylation profile of GNE Myopathy muscle cells does not point to consistent hyposialylation of individual glycoconjugates	10
Phenotypic convergence in Charcot-Marie-Tooth 2Y with novel VCP mutation	10
Presynaptic congenital myasthenic syndrome due to three novel mutations in SLC5A7 encoding the sodium-dependant high-affinity choline transporter	10
Respiratory muscle training in late-onset Pompe disease: Results of a sham-controlled clinical trial	10
Accuracy of patient-reported data for an online patient registry of autoimmune myasthenia gravis and Lambert-Eaton myasthenic syndrome	10
Clinical outcome of SARS-CoV-2 infection in 7 adults with Duchenne muscular dystrophy attending a specialist neuromuscular centre	10
Patient and parent oriented tools to assess health-related quality of life, activity of daily living and caregiver burden in SMA. Rome, 13 July 2019	10
Insulin-like growth factor-1 status is associated with insulin resistance in young patients with spinal muscular atrophy	10

Corrigendum to “Randomized phase 2 trial and open-label extension of domagrozumab in Duchenne muscular dystrophy” [Neuromuscular Disorders, Vol. 30 (6) 2020, 492-502]	9
Population pharmacokinetics-based recommendations for a single delayed or missed dose of nusinersen	9
Musculoskeletal magnetic resonance imaging in the DE50-MD dog model of Duchenne muscular dystrophy	9
Cardiovascular disease in non-classic Pompe disease: A systematic review	9
HyperCKemia and rhabdomyolysis in the neuroleptic malignant and serotonin syndromes: A literature review	9
Pathogenic variants in COL6A3 cause Ullrich-like congenital muscular dystrophy in young Labrador Retriever dogs	9
Predictors of outcome in patients with myasthenic crisis undergoing non-invasive mechanical ventilation: A retrospective 20 year longitudinal cohort study from a single Italian center	9
Myasthenia gravis: do not forget the patient perspective	9
Safety and clinical outcome of tamoxifen in Duchenne muscular dystrophy	9
Biomarkers of disease progression in adolescents and adults with 5q spinal muscular atrophy: a systematic review and meta-analysis	9
Lack of effect on ambulation of dalfampridine-ER (4-AP) treatment in adult SMA patients	9
Newborn screening for spinal muscular atrophy: The Wisconsin first year experience	9
Congenital asymmetric distal myopathy with hemifacial weakness caused by a heterozygous large de novo mosaic deletion in nebulin	8
1st FSHD European Trial Network workshop:Working towards trial readiness across Europe	8
Genitourinary and lower gastrointestinal conditions in patients with myotonic dystrophy type 1: A systematic review of evidence and implications for clinical practice	8
Tasks and interfaces in primary and specialized palliative care for Duchenne muscular dystrophy – A patients’ perspective	8
252nd ENMC international workshop: Developing best practice guidelines for management of mouthpiece ventilation in neuromuscular disorders. March 6th to 8th 2020, Amsterdam, the Netherlands	8
Clinical and molecular evidence of possible digenic inheritance for MFN2/GDAP1 genes in Charcot-Marie-Tooth disease	8
Exploring the efficacy of the expiratory muscle strength trainer to improve swallowing in inclusion body myositis: A pilot study	8
Transportin 3 (TNPO3) and related proteins in limb girdle muscular dystrophy D2 muscle biopsies: A morphological study and pathogenetic hypothesis	8
Managing pregnancy and anaesthetics in patients with skeletal muscle channelopathies	8
Oral bisphosphonate treatment in patients with Duchenne muscular dystrophy on long term glucocorticoid therapy	8
Screening for early symptoms of respiratory involvement in myotonic dystrophy type 1 using the Respicheck questionnaire	8
Utility of maximum inspiratory and expiratory pressures as a screening method for respiratory insufficiency in slowly progressive neuromuscular disorders	8
Mutation spectrum and health status in skeletal muscle channelopathies in Japan	8
Characteristics of VCP mutation-associated cardiomyopathy	8
The functional and structural evaluation of small fibers in asymptomatic carriers of TTR p.Val50Met (Val30Met) mutation	8
A rare case of adult onset LPIN1 associated rhabdomyolysis	8
Rituximab in chronic immune mediated neuropathies: a systematic review	8
Efficacy and safety of tacrolimus as long-term monotherapy for myasthenia gravis	7
A new respiratory scoring system for evaluation of respiratory outcomes in children with spinal muscular atrophy type1 (SMA1) on SMN enhancing drugs	7
Epidemiology and treatment of myasthenia gravis: a retrospective study using a large insurance claims dataset in Germany	7
Spontaneous symptomatic improvement in a pediatric patient with anti-3-hydroxy-3-methylglutraryl-coenzyme A reductase myopathy	7
AChR myasthenia gravis switching to MuSK or double antibody positive myasthenia gravis in two children and literature review	7
Hereditary myotonia in American Bulldog associated with a novel frameshift mutation in the CLCN1 gene	7
Respiratory muscle function in patients with nemaline myopathy	7
Prevalence of atrial fibrillation in myotonic dystrophy type 1: A systematic review	7
The effectiveness and side effects of pyridostigmine in the treatment of myasthenia gravis: a cross-sectional study	7
Identification of novel SMN1 subtle mutations using an allelic-specific RT-PCR	7
Expiratory dysfunction in young dogs with golden retriever muscular dystrophy	7
Effect of long term enzyme replacement therapy in late onset Pompe disease: A single-centre experience	7
The Spinal Muscular Atrophy Health Index: Italian validation of a disease-specific outcome measure	7
Newborn screening of neuromuscular diseases	7
A hospital based epidemiological study of genetically determined muscle disease in south western Norway	7
Subcutaneous immunoglobulin for maintenance therapy in stiff-person syndrome: One-year follow-up in two patients	7
Hereditary polyneuropathy with optic atrophy due to PDXK variant leading to impaired Vitamin B6 metabolism	7
A novel, pathogenic dinucleotide deletion in the mitochondrial MT-TY gene causing myasthenia-like features	7
A web-based questionnaire survey on the influence of coronavirus disease-19 on the care of patients with muscular dystrophy	7
Genetic neuropathies presenting with CIDP-like features in childhood	6
Psychological parameters impact health-related quality of life in mental and physical domains in adults with muscular dystrophy	6
Late onset Sandhoff disease presenting with lower motor neuron disease and stuttering	6
Pyrostigmine therapy in a patient with VAMP1-related congenital myasthenic syndrome	6
Non-dystrophic myotonia Chilean cohort with predominance of the SCN4A Gly1306Glu variant	6
Intracranial hemorrhage secondary to vitamin K deficiency in X-linked myotubular myopathy	6
A novel nonsense variant in MT-CO3 causes MELAS syndrome	6
259th ENMC international workshop: Anaesthesia and neuromuscular disorders 11 December, 2020 and 28–29 May, 2021	6
Association of fingerprint bodies with rods in a case with mutations in the LMOD3 gene	6
Myasthenia gravis after etanercept and ustekinumab treatment for psoriatic arthritis: A case report	6
mRNA intramuscular vaccination produces a robust IgG antibody response in advanced neuromuscular disease	6
Sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance: Report of four patients	6
Muscular dystrophy-dystroglycanopathy in a family of Labrador retrievers with a LARGE1 mutation	6
Congenital myasthenic syndromes in the Thai population: Clinical findings and novel mutations	6
Inflammatory myopathies in childhood	6
LATE BREAKING NEWS ORAL PRESENTATION	6
Late onset Pompe Disease in India – Beyond the Caucasian phenotype	6
Natural history of 10-meter walk/run test performance in spinal muscular atrophy: A longitudinal analysis	6
Novel compound heterozygous TTN variants as a cause of severe neonatal congenital contracture syndrome without cardiac involvement diagnosed with rapid trio exome sequencing	6
Idiopathic eosinophilic myositis: a systematic literature review	5
Cardiac involvement in two rare neuromuscular diseases: LAMA2-related muscular dystrophy and SELENON-related myopathy	5
Clinical and muscle MRI features in a family with tubular aggregate myopathy and novel STIM1 mutation	5
Risk factors and future directions for preventing and diagnosing exertional rhabdomyolysis	5
Acute severe sensory ganglionopathy in systemic lupus erythematous	5
A novel MT-CO2 variant causing cerebellar ataxia and neuropathy: The role of muscle biopsy in diagnosis and defining pathogenicity	5
Expiratory muscle strength training improves measures of pressure generation and cough strength in a patient with myotonic dystrophy type 1	5
LATE BREAKING NEWS E-POSTER PRESENTATION	5
Clinical characteristics and outcome in muscular sarcoidosis: a retrospective cohort study and literature review	5
A late-onset congenital myasthenic syndrome due to a heterozygous DOK7 mutation	5
Factors associated with delayed diagnosis of spinal muscular atrophy in China and changes in diagnostic delay	5
Serum miRNAs as biomarkers for the rare types of muscular dystrophy	5
Infant with early onset bilateral facial and bulbar weakness: Successful treatment of riboflavin in multiple acyl-CoA dehydrogenase deficiency caused by biallelic nonsense FLAD1 variants	5

Late-onset camptocormia caused by a heterozygous in-frame CAPN3 deletion	5
Safety, tolerability and pharmacokinetics of eteplirsen in young boys aged 6–48 months with Duchenne muscular dystrophy amenable to exon 51 skipping	5
Tandem duplication within the DMD gene in Labrador retrievers with a mild clinical phenotype	5
SMA – THERAPY	5
Home-based exercise in autoimmune myasthenia gravis: A randomized controlled trial	5
Mutations in the SIGMAR1 gene cause a distal hereditary motor neuropathy phenotype mimicking ALS: Report of two novel variants	5
Impaired secretion of platelet granules in patients with Duchenne muscular dystrophy – results of a prospective diagnostic study	5
TNNT1 myopathy with novel compound heterozygous mutations	5
A rare mutation in the COLQ gene causing congenital myasthenic syndrome with remarkable improvement to fluoxetine: A case report	5
GDAP1 mutations are frequent among Brazilian patients with autosomal recessive axonal Charcot-Marie-Tooth disease	5
Clinical and electrophysiological evaluation of myasthenic features in an alpha-dystroglycanopathy cohort (FKRP-predominant)	5
Muscle “islands”: An MRI signature distinguishing neurogenic from myopathic causes of early onset distal weakness	5
Longer-term follow-up of nusinersen efficacy and safety in adult patients with spinal muscular atrophy types 2 and 3	5
Troponin-T type 1 (TNNT1)-related nemaline myopathy: unique respiratory phenotype and muscle pathology findings	5
246th ENMC International Workshop: Protein aggregate myopathies 24–26 May 2019, Hoofddorp, The Netherlands	5
A mutation in MTM1 causes X-Linked myotubular myopathy in Boykin spaniels	5
Reliability and validity of the FSHD-composite outcome measure in childhood facioscapulohumeral dystrophy	5
250th ENMC International Workshop: Clinical trial readiness in nemaline myopathy 6–8 September 2019, Hoofdorp, the Netherlands	5
Language, speech, and oromotor function in children with Pompe disease	5
Cerebral involvement and related aspects in myotonic dystrophy type 2	5
Cognitive profiles and clinical factors in type III spinal muscular atrophy: a preliminary study	5
Primary mitochondrial myopathies in childhood	4
Lack of muscle stem cell proliferation and myocellular hypertrophy in sIBM patients following blood-flow restricted resistance training	4
Fatal disseminated Anncaliia algerae myositis mimicking polymyositis in an immunocompromised patient	4
“Status myotonicus” in Nav1.4-M1592V channelopathy	4
Long-term effects of enzyme replacement therapy in an elderly cohort of late-onset Pompe disease	4
High prevalence of sporadic late-onset nemaline myopathy in a cohort of whole-exome sequencing negative myopathy patients	4
Quantitative vs qualitative muscle MRI: Imaging biomarker in patients with Oculopharyngeal Muscular Dystrophy (OPMD)	4
Intellectual disability in paediatric patients with genetic muscle diseases	4
Response to “Autosomal recessive axonal neuropathy caused by HINT1 mutation: New association of a psychiatric disorder to the neurological phenotype”	4
A novel mutation in NEB causing foetal nemaline myopathy with arthrogryposis during early gestation	4
Is it Pompe Disease? Australian diagnostic considerations	4
SMA – THERAPY	4
Diaphragmatic dysfunction in neuromuscular disease, an MRI study	4
Health related quality of life in young, steroid-naïve boys with Duchenne muscular dystrophy	4
Long term follow-up of scoliosis progression in type II SMA patients	4
The impact of testosterone therapy on quality of life in adolescents with Duchenne muscular dystrophy	4
Optimizing hand-function patient outcome measures for inclusion body myositis	4
BMI-z scores of boys with Duchenne muscular dystrophy already begin to increase before losing ambulation: a longitudinal exploration of BMI, corticosteroids and caloric intake	4
Cardiac MRI identifies valvular and myocardial disease in a subset of ANO5-related muscular dystrophy patients	4
Respiratory decline in adult patients with Becker muscular dystrophy: A longitudinal study	4
Gene therapy and other novel treatment approaches for Charcot-Marie-Tooth disease	4
Randomisation versus prioritisation in a managed access programme: Lessons from spinal muscular atrophy	4
Frontal lobe-dominant cerebral blood flow reduction and atrophy can be progressive in Duchenne muscular dystrophy	4
Congenital myopathy and epidermolysis bullosa due to PLEC variant	4
Early pathological signs in young dysf mice are improved by halofuginone	4
Progressive external ophthalmoplegia due to a recurrent de novo m.15990C>T MT-TP (mt-tRNAPro) gene variant	4
The phenotypic and genotypic features of a Scottish cohort with McArdle disease	4
SMA – THERAPY	4
264th ENMC International Workshop: Multi-system involvement in spinal muscular atrophy Hoofddorp, the Netherlands, November 19th – 21st 2021	4
Homozygous intronic variants in TPM2 cause recessively inherited Escobar variant of multiple pterygium syndrome and congenital myopathy	4
Safety and effectiveness of resistance training in patients with late onset Pompe disease - a pilot study	4
DMD – THERAPY	4
Changes in walking velocity and stride parameters with age in children with Charcot-Marie-Tooth disease	4
Adalimumab and myositis: A case report and review of the French and international Pharmacovigilance Databases	4
Novel ACTA1 mutation causes late-presenting nemaline myopathy with unusual dark cores	4
Clinical trajectory of a patient with filaminopathy who developed arrhythmogenic cardiomyopathy, myofibrillar myopathy, and multiorgan tumors	4
Analysis of complex structural variants in the DMD gene in one family	4
No effect of oral sucrose or IV glucose during exercise in phosphorylase b kinase deficiency	4
Refractory CIDP successfully treated with autologous haematopoietic stem cell transplantation in a patient with monoclonal gammopathy of undetermined significance (MGUS) and rheumatoid arthritis	4
Risk of malignant hyperthermia in patients carrying a variant in the skeletal muscle ryanodine receptor 1 gene	4
251st ENMC international workshop: Polyglucosan storage myopathies 13–15 December 2019, Hoofddorp, the Netherlands	4
Selected clinical and demographic factors and all-cause mortality among individuals with Duchenne muscular dystrophy in the Muscular Dystrophy Surveillance, Tracking, and Research Network	4
Novel autosomal dominant TPM3 mutation causes a combined congenital fibre type disproportion-cap disease histological pattern	4
Muscle cell differentiation and development pathway defects in Emery-Dreifuss muscular dystrophy	4
Characterization of cannabis use by patients with myotonic dystrophy type 1: A pilot study	4
MRI findings in SANDO variety of the ataxia-neuropathy spectrum with a novel mutation in POLG (c.3287G>T): A case report	4
Characterizing cognitive-motor impairments in patients with myotonic dystrophy type 1	3
Autoantibody profile in a Malaysian cohort of chronic inflammatory demyelinating polyneuropathy	3
Chronic mimics of myasthenia gravis: a retrospective case series	3
Early onset neutral lipid storage disease with myopathy presenting as congenital hypotonia and hepatomegaly	3
Correlations of disease severity outcome measures in inclusion body myositis	3
DMD & BMD – CLINICAL	3
FP.24 RAINBOWFISH: Preliminary efficacy and safety data in risdiplam-treated infants with presymptomatic spinal muscular atrophy (SMA)	3
SMA – THERAPY	3
An autopsied case of ADSSL1 myopathy	3
Prevalence of genetically confirmed skeletal muscle channelopathies in the era of next generation sequencing	3
Variant transthyretin amyloidosis (ATTRv) polyneuropathy in Greece: a broad overview with a focus on non-endemic unexplored regions of the country	3
Influence of β2 adrenergic receptor genotype on longitudinal measures of forced vital capacity in patients with Duchenne muscular dystrophy	3
Dystrophinopathy and the brain: A parent project muscular dystrophy (PPMD) meeting report November 11-12, 2021, New York City, NY	3
Whole-body muscle MRI in McArdle disease	3
Clinical and neuroradiological correlates of sleep in myotonic dystrophy type 1	3
265th ENMC International Workshop: Muscle imaging in Facioscapulohumeral Muscular Dystrophy (FSHD): relevance for clinical trials. 22–24 April 2022, Hoofddorp, The Netherlands	3
Clinical and genetic spectrum of a Chinese cohort with SCN4A gene mutations	3
Reducing body myopathy – A new pathogenic FHL1 variant and literature review	3
Myoneuropathic presentation of limb girdle muscular dystrophy R8 with a novel TRIM32 mutation	3
Results of an open label feasibility study of sodium valproate in people with McArdle disease	3
A family with adult-onset myofibrillar myopathy with BAG3 mutation (P470S) presenting with axonal polyneuropathy	3
A novel missense HNRNPA1 variant in the PY-NLS domain in a patient with late-onset distal myopathy	3
Motor unit number index (MUNIX) in children and adults with 5q-spinal muscular atrophy: Variability and clinical correlations	3
Filling the gaps in knowledge translation: Physical therapy recommendations for individuals with spinal muscular atrophy compared to standard of care guidelines	3
SMALED2 with BICD2 gene mutations: Report of two cases and portrayal of a classical phenotype	3
232nd ENMC international workshop: Recommendations for treatment of mitochondrial DNA maintenance disorders. 16 – 18 June 2017, Heemskerk, The Netherlands	3
Clinical characteristics and prognosis of very late-onset myasthenia gravis in China	3
Early and long-term effect of the treatment with pyridostigmine in patients with GMPPB-related congenital myasthenic syndrome	3