Spinal Neurofibromas Research Articles

Late-Onset Presentation of Spastic Paraplegia 3A (ATL1-HSP) and Its Rare Occurrence with Multiple Spinal Neurofibromas

Late-Onset Presentation of Spastic Paraplegia 3A (ATL1-HSP) and Its Rare Occurrence with Multiple Spinal Neurofibromas

The rare association of congenital glaucoma, giant melanocytic nevus, alopecia, and hypospadias in an Egyptian child with neurofibromatosis type 1: a case report

BackgroundNeurofibromatosis type 1 (NF1) is a multisystem genetic disorder that commonly involves skin, nerves, and skeletal system with increased neoplastic predisposition. This disease has been rarely associated with multiple congenital anomalies. Herein, we describe an Egyptian child with NF1 and coexistent bilateral congenital glaucoma, giant congenital melanocytic nevi (GCMN), alopecia, and hypospadias.Case presentationA 2.5-year-old boy presented with developmental delay, back swelling, and multiple congenital anomalies. His father and two sisters were known to have NF1. The child was diagnosed with bilateral primary congenital glaucoma at the age of 3.5 months and underwent trabeculectomy with mitomycin C therapy. Examination at the age of 5 months revealed marked hypotonia, multiple GCMN, scanty café-au-lait macules, left upper eyelid plexiform neuroma and trichomegaly, hypertrichosis of left eyebrow, hypertelorism, depressed nasal bridge, left frontal scalp alopecia, and distal penile hypospadias. At the age of 8 months, brain imaging depicted a markedly dilated left lateral ventricle, and he underwent ventriculoperitoneal shunt surgery. The child developed back swelling at the age of 2.5 years, and a spinal magnetic resonance image showed bilateral multiple spinal neurofibromas in the paraspinal region with intraspinal extensions. A whole exome sequencing identified a heterozygous missense variant NM_001042492.3:c.1466A > G (NP_001035957.1:p.Tyr489Cys) in NF1 gene.ConclusionsThe present case report adds to the knowledge of the phenotypic spectrum and variability of NF1 by reporting the association of multiple unusual congenital anomalies. Importantly, such congenital anomalies could be the first presenting features in patients with NF1 since cafe´-au-lait macules and other typical diagnostic criteria may not be apparent in the neonatal period and early infancy. Accordingly, NF1 should be considered in newborns with congenital glaucoma, GCMN, scalp alopecia, and hypospadias.

Genotype-Phenotype Correlation in Neurofibromatosis Type 1: Evidence for a Mild Phenotype Associated with Splicing Variants Leading to In-Frame Skipping of NF1 Exon 24 [19a

Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disorder caused by loss-of-function variants in the NF1 gene. As of 20 November 2023, over 5000 distinct pathogenic or likely pathogenic variants have been reported in public databases. However, only a few NF1 genotype-phenotype correlations have been established so far. In this study, we present findings on 40 individuals with NF1, comprising 26 unrelated probands and 14 affected relatives, who carry one of nine NF1 heterozygous pathogenic splicing variants, all of which result in the in-frame skipping of exon 24 [19a] (NM_000267.3:r.3114_3197del, p.Asn1039_Arg1066del). These variants include c.3114-2A>G, c.3114-1G>A, c.3196A>G, c.3197G>A, c.3197G>T, c.3197+1G>A, c.3197+1G>T, c.3197+2T>C, and c.3197+3A>T. Among individuals with these variants, none exhibit externally visible plexiform neurofibromas, histopathologically confirmed cutaneous or subcutaneous neurofibromas, symptomatic spinal neurofibromas, or symptomatic optic pathway gliomas. The most prevalent, and sometimes sole, clinical feature observed in this cohort is multiple café-au-lait macules, with or without skinfold freckles: 85% and 60.5% of the individuals display six or more café-au-lait macules and freckles, respectively. In comparison to established NF1 genotype-phenotype correlations, these patients demonstrate highly similar clinical presentations to those associated with the NF1 pathogenic variant c.2970_2972del (p.Met992del), known for resulting in the mildest clinical features. Despite the generally mild phenotype, cognitive impairment, developmental delay, and/or learning difficulties are still observed in 33.3% of these patients, suggesting that learning challenges remain a prominent aspect of the phenotypic presentation in these individuals and necessitate specialized care. This newly established genotype-phenotype correlation will assist clinicians in improving the management of patients harboring NF1 exon 24 [19a] skipping variants and provide a new therapeutic target for NF1 treatment.

Laparoscopic-assisted Resection of a Retroperitoneal Lumbar Nerve Root Neurofibroma: A Case Report.

We present a case of a 59-year-old patient with chronic low back pain, caused by a retroperitoneal intraneural tumour. Laparoscopic excision was performed and histology revealed a spinal nerve root neurofibroma. Post-operatively, the patient developed partial motor and sensitive deficits due to tumoral nerve entrapment, with progressive recovery with rehabilitation. This report reviews the literature on this sparsely reported condition, highlighting the utility of laparoscopy in its management.

Genotype-phenotype correlations of neurofibromatosis type 1: a cross-sectional study from a large Chinese cohort.

Neurofibromatosis type 1 (NF1) is a highly heterogeneous autosomal genetic disorder characterized by a broad spectrum of clinical and molecular manifestations. The correlations between genotype and phenotype in NF1 remain elusive. This study aimed to elucidate genotype-phenotype associations in a large Chinese cohort of NF1 patients. We included NF1 patients from our center who underwent genetic testing for NF1 variants and systemic examination. Genotype-phenotype correlation analyses were performed, focusing on variation types and involved neurofibromin domains. A total of 195 patients were enrolled, comprising 105 males and 90 females, with a median age of 18years. Truncating variants, single amino acid variations, and splicing variants accounted for 139/195 (71.3%), 23/195 (11.8%), and 33/195 (16.9%), respectively. Patients with splicing variants exhibited a significantly higher prevalence of spinal plexiform neurofibromas (spinal PNF) than those with truncating variants (76.4% vs. 51.8%; p = 0.022). Variations affecting the PKC domain were associated with higher rates of cutaneous neurofibromas (CNF) (100% vs. 64.9%, p < 0.001), Lisch nodules (100% vs. 61.2%, p < 0.001), plexiform neurofibromas (PNF) (100% vs. 95.7%, p = 0.009), and psychiatric disorders (11.8% vs. 1.6%, p = 0.042). Patients with mutations in the CSRD had an elevated risk of secondary primary malignancies (11.6% vs. 2.8%, p = 0.015). GRD involvement might enhance the risk of Lisch nodules (76.9% vs. 53.7%, p = 0.044). Variations in the Sec14-PH domain were correlated with a higher rate of CNF (76.8% vs. 58.6%, p = 0.014). Additionally, we found that the p.R1748* variants carry a high risk of malignancy. Our study suggested some novel genotype-phenotype correlations within a Chinese cohort, providing innovative insights into this complex field that may contribute to genetic counseling, risk stratification, and clinical management for the NF1 population.

N°321 – A rare case of isolated cervical spinal nerve neurofibromas in a patient with a type 1 neurofibromatosis

N°321 – A rare case of isolated cervical spinal nerve neurofibromas in a patient with a type 1 neurofibromatosis

A case of cutis marmorata telangiectasia congenita

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A case of langerhans cell histiocytosis with cholangitis

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The Role of Magnetic Resonance Imaging in the Diagnosis of Spinal Dumbell Neurofibromatosis: A Case Report from a Specialist Hospital in Port Harcourt

Neurofibromatosis occurring in the spine also known as spinal neurofibromatosis (SNF) is a rare disease entity and they occur either unilaterally or bilaterally at the spinal roots. The low prevalence rate of the disease and scarcity of previous literatures is the main aim for this report.&#x0D; Our case study is a 29-years-old lady who presented to the Radiology department for a lumbosacral spine MRI, on account of both lower limb weakness, paresthesia, unsteady gait and finally inability to walk. The symptoms have been there since she was 14 years (15 years of progressive symptoms). She also presented with multiple macules on the skin (café au lait spots). Magnetic Resonance Imaging (MRI) of the lumbosacral spine revealed bilateral spinal neurofibromas at the spinal roots of the fourth lumbar vertebra to the sacral/coccygeal vertebrae with destruction of the sacro-coccygeal bones. MRI of the brain revealed no abnormalities in the brain and optic tract. The patient fulfilled the National Institutes of Health (NIH) criteria and that of revised diagnostic criteria for Neurofibromatosis (NF1).&#x0D; The patient also presented with lack of pain which could be due to impairment in sensory neurons. We describe a case of spinal neurofibromatosis diagnosed by MRI in a young lady with progressive lower limb weakness, paresthesia, sensory loss, café-au- lait spots and paraplegia.

Surgical outcome of spinal schwannoma and neurofibroma.

The aim of this study was to evaluate the outcome and surgical complications in patients with spinal schwannoma or neurofibroma surgically treated at the Hospital das Clínicas of the State University of Campinas. This was a retrospective cohort study, using medical records of patients operated between 2011 and 2021. The sample distribution was verified using the Kolmogorov-Smirnov test. The dynamics between qualitative variables were assessed using Fisher's exact test. We used means analysis to assess patient improvement based on Frankel scores. A total of 16 patients were evaluated, of whom 56.25% (9) were men and 43.75% (7) were women. There were 13 (81.25%) patients with schwannomas and 3 (18.75%) with neurofibromas. Patients with deficits had neurological improvement, such as walking or with at least Frankel D or E after surgery. Laminectomy, performed in 8 patients (50%), and laminoplasty, used in 9 patients (56.25%), were the main techniques. The surgical approach was proved to be an effective and safe alternative to the treatment of these tumors, with neurological improvement and minor surgical complications.

Atypical clinical manifestations and genotype-phenotype correlations of neurofibromatosis type 1

Purpose of the study: Analysis of available data on geno-phenotypic correlations and atypical forms of neurofibromatosis type 1. Material and methods. We searched for relevant sources in the Scopus, Web of Science, PubMed systems, including publications from May 1993 to October 2021. Of the 318 studies we identified, 59 were used to write a systematic review. Results. We found studies describing atypical forms of neurofibromatosis type 1 with an erased course without manifestation of a tumor syndrome, which are caused by specific mutations in the NF1 gene (causing substitutions of amino acids in neurofibromin: p.Arg1038, p.Met1149, p.Arg1809, or deletion of amino acids: p.Met990del, p.Met992del). NF1 patients with microdeletions are characterized by more severe disease symptoms (more often facial dysmorphism, skeletal and cardiovascular abnormalities, learning difficulties, and symptomatic spinal neurofibromas). mutations of splicing sites and extended deletions of the NF1 gene are associated with early manifestation of tumors, mutations at the 5’-end of the gene, causing a shortening of the protein product, are associated with optic nerve gliomas. the mutation c.3721C&gt;T (p.R1241*) correlated with structural brain damage, and c.6855C&gt;A (p.Y2285*) with endocrine disorders. the manifestations of NF1, similar to lipomatosis and Jaffe-Campanacci syndrome, not associated with a specific type of mutation are described. Conclusion. In spite of pronounced clinical variability of the disease, even among members of the same family, several studies have described genotype-phenotype correlations. Therefore, the role of modifier genes and epigenetic factors in the pathogenesis of NF1 is assumed, since the neurofibromin protein has a complex structure with several functional domains. It has been shown that the severity of the tumor syndrome is influenced by the methylation characteristics of NF1 gene and adjacent areas. in addition, NF1 gene is associated with a variety of microRNAs. therefore, targeted therapy aimed at specific non-coding RNAs to restore normal expression of NF1 gene can become a promising treatment for NF1.

Diagnostic and therapeutic approach of artificial intelligence in neuro-oncological diseases

Neuro-oncological diseases are rare and their fatality rate is increased in patients due to advance disease development despite of the recent outcomes on neuro-oncological therapies. Artificial intelligence (AI) approaches and the exponential expansion of computing algorithms are set to increase the precision of diagnostic and therapeutic approaches in medicine. Medical imaging is one of the common AI applications where it assists radiologists in diagnosis. Radiomics has been successfully applied in neuro-oncology and it will be at forefront of AI revolution. Various AI methods can define numerous infiltrating margins of neuro-oncological diseases and it differentiates pseudo-progression from real progression and envisage recurrence and survival better than the methods used in routine practice. The present review deliberates the common neuro-oncological diseases such as glioblastoma, meningioma, spinal cord tumor and neurofibroma (NF1) and its AI algorithms related to imaging techniques such as computed (MRI) and computed tomography (CT). Also, we have discussed the beneficial aspect of AI and recent trends in diagnosis. From the study, the management of neuro-oncological diseases using AI can be revolutionized and the need of omics analysis is essential in future.

Spinal neurofibromas presenting as quadriparesis in a case of neurofibromatosis Type 2

Neurofibromatosis Type II (NF2) is a neurocutaneous syndrome with autosomal dominant inheritance caused by mutation in NF2 gene that codes for protein merlin. It is associated with various tumours such as vestibular schwannoma, meningiomas, ependymomas, etc. in central and peripheral nervous system. The Manchester criteria is used for its diagnosis. We report a case of a 9 year old girl with café au lait spots and plexiform neurofibromas who presented with gradual onset UMN type of progressive quadriparesis over 20 days. Magnetic resonance imaging brain and spine revealed bilateral vestibular schwannomas and multiple spinal neurofibromas which were confirmed on histopathologic examination, thereby confirming diagnosis of NF Type 2.

Spinal screening, malignancy, medical therapy, and surgical correction of deformity in pediatric patients with neurofibromatosis type 1: a systematic review.

The objective of this systematic review was to synthesize evidence regarding spinal screening recommendations, types of spinal and thoracic neurofibromatosis type 1 (NF1) tumors, medical therapy for NF1-associated neoplasms, and treatment with magnetically controlled growing rods (MCGRs) or cervical kyphosis correction in pediatric patients with NF1. We queried PubMed, Embase, Cochrane Library, Web of Science, Scopus, Clinicaltrials.gov, and medRxiv for studies reporting spinal screening recommendations, prognosis, and medical therapy for NF1-associated spinal tumors and MCGR use or cervical kyphosis correction in pediatric NF1 patients, yielding 758 publications, 33 of which were included. There is no consensus on spinal screening interval. Computed tomography is recommended for postoperative monitoring. Patients with gangliomas and spinal neurofibromas had nearly complete symptom resolution after resection. Plexiform neurofibromas were most commonly treated with resection and laminectomy; some patients reported tumor enlargement after intervention. Malignant nerve sheath tumors have high rates of metastasis even after chemoradiation and resection. MEK-inhibitors produced limited regression in tumor size. Sirolimus and thalidomide reduced tumor size but caused more severe adverse effects than MEK-inhibitors. Improvements in major curves and T1-T12 height gain were reported after MCGR intervention. Anteroposterior arthrodesis produced the greatest correction of dystrophic cervical kyphosis. There may be value in establishing standardized spinal screening protocols for pediatric NF1 patients. Surgical correction of NF1-associated spinal deformity is effective, though current medical therapies for spinal tumors have limited success. Areas for further investigation include determining appropriate screening intervals, choice of medical therapy for spinal tumors, and long-term outcomes of MCGRs. Level of Evidence: IV.

Segmental neurofibromatosis with compression of the spinal cord at the cervical level. Literature review and case report

Background. Neurofibromatosis is a fairly rare disease (1/3000). In 1992, V. Riccardi described seven types of neurofibromatosis. Segmental neurofibromatosis (sh), also known as type V neurofibromatosis, is an extremely rare variant characterized by the development of typical cutaneous manifestations or one body segment neurofibromas. Clinical case. Currently, the literature describes about 100 cases of sh and only one of them with compression of the spinal cord. We present our first case of this nosological form with spinal cord compression in a Russian patient. A 70-year-old patient, due to an increasing paresis in the left extremities, underwent mri of the cervical spine, which revealed solid tumors located extramedullary intra-extradurally at the level of c2-c3 vertebrae with pronounced compression of the spinal cord. At the time of hospitalization, clinical presentation was characterized by deep spastic tetraparesis (1–2 points), impairment of all types of sensitivity from the c4 level by the conductive type, and dysfunction of the pelvic organs by the type of delay. Karnofsky index was 50 %, 2 points on the Fim scale. Standard c2-c3 vertebrae laminectomy was performed. Spinal cord compression was eliminated due to the removal of intradural tumors. Subsequently, extradural tumors were removed step by step. On histological examination, tumors were represented by intertwining bundles of elongated schwann cells with wavy nuclei with pointed ends and ileogenic fibers. Mucin present in the stroma separated cells and fibers. Conclusion. Sn is a rare type of neurofibromatosis. However, from the point of view of genetics, it is most likely incorrect to attribute it to a separate type of neurofibromatosis, since the cause of its development is mosaicism of somatic cells due to mutation of the NF 1 gene. Sn is rarely manifested by the development of spinal nerves multiple neurofibromas, however, it can be accompanied by a gross neurological deficit caused by compression of the spinal cord such neurofibromas. Surgical treatment is based on basic and special surgical principles that determine the anatomical and morphological characteristics of the area of intervention, the compliance of which allows for good treatment results.

Sporadic spinal schwannomas and neurofibromas - a review.

Tumours arising from the spinal nerve roots are more common in men between the third and fourth decades of life, located mainly in the intradural space of the lumbar region. The clinical symptoms of spinal schwannomas and neurofibromas are similar and depend on the location of the tumour and its relationship to the spinal cord and nerve roots. The pain (radiculalgia and/or vertebralgia) is the earliest symptom, followed by sensory deficit, while motor deficits and sphincter disorders are late symptoms. Magnetic resonance imaging is the diagnostic tool of choice and should be performed when the disease is suspected, as early and accurate diagnosis is essential for treatment outcome. The treatment of these lesions is surgical aiming at total tumour resection that promotes neurological recovery and prevents from recurrences. Subtotal removal also provides favourable outcome but at higher recurrence rates. The aim of this literature review was to study some important characteristics of sporadic spinal schwannomas (SSS) and neurofibromas (SSN), i.e. those notassociated with neurofibromatosis type I and II.

Severe Phenotype in Patients with Large Deletions of NF1.

Simple SummaryNeurofibromatosis type 1 (NF1) is a genetic disorder caused by pathogenic variants in the NF1 tumor suppressor gene. In 5–10% of NF1 patients, a large heterozygous deletion of the whole NF1 gene is identified, leading to the commonly called “NF1 microdeletion syndrome”. NF1-deleted patients were previously reported to develop a particularly severe form of the disease with frequent cognitive impairment and an increased risk of benign and malignant tumors. Here, we performed a comprehensive clinical assessment of the largest NF1-deleted cohort to date, including 126 NF1 patients with a deletion of the NF1 gene. This work provides new insights into the specific phenotype associated with NF1 deletions and may contribute to improve the follow-up care of NF1 patients. Complete deletion of the NF1 gene is identified in 5–10% of patients with neurofibromatosis type 1 (NF1). Several studies have previously described particularly severe forms of the disease in NF1 patients with deletion of the NF1 locus, but comprehensive descriptions of large cohorts are still missing to fully characterize this contiguous gene syndrome. NF1-deleted patients were enrolled and phenotypically characterized with a standardized questionnaire between 2005 and 2020 from a large French NF1 cohort. Statistical analyses for main NF1-associated symptoms were performed versus an NF1 reference population. A deletion of the NF1 gene was detected in 4% (139/3479) of molecularly confirmed NF1 index cases. The median age of the group at clinical investigations was 21 years old. A comprehensive clinical assessment showed that 93% (116/126) of NF1-deleted patients fulfilled the NIH criteria for NF1. More than half had café-au-lait spots, skinfold freckling, Lisch nodules, neurofibromas, neurological abnormalities, and cognitive impairment or learning disabilities. Comparison with previously described “classic” NF1 cohorts showed a significantly higher proportion of symptomatic spinal neurofibromas, dysmorphism, learning disabilities, malignancies, and skeletal and cardiovascular abnormalities in the NF1-deleted group. We described the largest NF1-deleted cohort to date and clarified the more severe phenotype observed in these patients.

RARE-16. A PATIENT WITH MOSAIC POST-ZYGOTIC KRAS-G12D PATHOGENIC VARIANT PRESENTING WITH A SYMPTOMATIC SPINAL NEUROFIBROMA AND LARGE SEGMENTAL TRUNCAL CAFÉ AU LAIT SPOT

An 11 year old boy presented with a three month history of progressive bilateral lower extremity weakness associated with recent intermittent incontinence. Spine MRI showed a right-sided T11-T12 paraspinal mass extending through the neural foramina into the epidural space and causing severe spinal cord compression. Skin showed a large macular lightly-hyperpigmented café au lait spot with irregular borders along the T10-T12 dermatome extending from the spine to approximately the anterior-axillary line. He was suspected to have segmental neurofibromatosis type 1 (NF1) as no additional clinical findings, radiographic features or family history of NF1 were identified. Patient underwent T10-12 laminectomy for resection of the epidural tumor component. Post-operative MRI showed resolution of the mass effect on the thecal sac and cord, with expected tumor residual lateral to the neural foramen. His residual spinal tumor and mild scoliosis remained stable over the two years of follow up to date. Pathological and molecular analysis of the resected tumor revealed a neurofibroma harboring an activating KRAS c.35G>A, p.Gly12Asp (KRAS-G12D) pathogenic variant at 27% variant allele frequency. Melanocytes cultured from two hyperpigmented skin biopsies showed the same KRAS-G12D pathogenic variant. This KRAS-G12D pathogenic variant was not found in leukocytes, indicating a post-zygotic origin. No NF1 pathogenic variant was identified in tumor tissue, melanocytes or leukocytes. The clinical findings were consistent with a mosaic KRASopathy due to a post zygotic KRAS-G12D pathogenic variant. The presence of the KRAS variant in the spinal neurofibroma and overlaying café au lait spot without an NF1 etiology in associated tissues demonstrates overlapping variability of presentations of RAS-MAPK pathway disorders. This case highlights the need for full clinical and genetic evaluation of patients presenting with segmental neurocutaneous disorders.

Endoscopic spine surgery for cervicothoracic spine neurofibroma: a case report

Introduction: Spinal tumors are rare lesions. The incidence of spinal tumors is about 0.5-2.5 cases per 100,000 population per year.1 Thirty percent of spinal tumors are located intradural. Twenty to thirty percent of intradural tumors are schwannoma and spinal neurofibroma.2 Surgery is the leading choice for schwannomas and neurofibromas that grow progressively or cause neurological deficits.2,3,4 Currently, minimally invasive surgery is developing rapidly, including minimally invasive surgery on spine surgery.Better outcomes are expected in endoscopic spine surgery compared to conventional spine surgery techniques.This paper aims to provide an overview of endoscopic procedures for the surgical removal of spinal tumors.Case description: A 59-year-old man presented paraplegia of both lower limbs. The patient complained difficulty walking for nine months before admitted to the hospital. The patient also complained of difficulty moving both of his legs, burning pain sensation, pain and numbness sensation that radiates from chest region to both of his toes, he felt that his chest was tied, and he complained hard to breathe when he feels pain. Complaints are getting worse until the patient was unable to walk. We found intra-dural extramedullary lesion from the MRI that was suspicious of a nerve cell sheath tumor. The patient underwent surgical treatment for tumor removal with an endoscopic technique, the cancer was complete. From the histopathology, the tumor was a neurofibroma tumor.Conclusion: Neurofibroma is one of the most frequently intradural extramedullary spinal tumors. Good imaging can guide the diagnosis and a good plan for manage patients with spinal tumors. Complete tumor resection provides a better prognosis in patients with neurofibromas. With endoscopic techniques, total spinal tumor removal surgery can be performed. The surgical procedure for spinal tumors using an endoscopic approach may be an option for spinal neurofibromas patients.

Classical neurofibroma.

We are presenting to you an magnetic resonance imaging (MRI) finding of a 27-year-old male who presented to us with complains of gradually progressing pain over the neck along with radiation to the upper extremity with more involvement of the right side than the left. He also complained of insidious onset of weakness in the upper extremity. Magnetic resonance imaging findings revealed T2 hyperintense mass (red arrow) in the intradural extramedullary mass involving the C3-C4 cervical vertebrae and the intervertebral disc in between, highly suggestive of neurofibroma. Spinal neurofibromas are frequently observed over the cervical cord and are usually asymptomatic. Our patient showed mild symptoms, probable reasons might be the slow growth rate of neurofibroma. He was referred for physiotherapy for further opinion. On physical examination, he had weakness in upper limb movement (MRC grade 2+) and weak grip strength. Surgery was the treatment of choice for him and he underwent surgical excision of it along with pre-operative physiotherapy management for 5 days. Post-surgery, there was an enormous improvement in his symptoms i.e. residual weakness persisting without any pain. He was prescribed strengthening and gripping exercises. Before discharge, he had a grade of 4+ on the MRC grading scale. Early medical and surgical management along with pre- and post-operative rehabilitation is the road to recovery in such patients.