severely ill patients – Page 8 – American ME and CFS Society

Adolescents with severe chronic fatigue syndrome can make a full recovery

Abstract:

The needs of children and adolescents severely affected by chronic fatigue syndrome, myalgic encephalomyelitis (CFS/ME) are currently inadequately addressed in the UK. Sadly, there are few specialists addressing the needs of these patients who are primarily bed-bound, wheelchair users or who can only leave home on an infrequent basis. Uncertainty about what to offer as well of a lack of funding may play a part. Action for Young people with ME (AYME) suggests that at least 350 severely affected children/adolescents are receiving little or inadequate care to help them overcome this debilitating illness. This case report illustrates how recovery can occur with pragmatic rehabilitation combined with a committed compassionate family based approach.

Source: Burgess M, Chalder T. Adolescents with severe chronic fatigue syndrome can make a full recovery. BMJ Case Rep. 2011 May 10;2011. pii: bcr0120113716. doi: 10.1136/bcr.01.2011.3716. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3091076/ (Full article)

Cervical spine stenosis as a cause of severe ME/CFS and orthostatic intolerance symptoms

Background: Comparatively little has been published on the clinical features and management of severe forms of ME/CFS.

Objectives: To describe the presenting symptoms and neurological examination findings in three young adult women whose disabling ME/CFS symptoms and orthostatic intolerance improved after the recognition and surgical management of cervical spine stenosis (CSS).

Methods: This retrospective case series includes three consecutive individuals who (1) met the Fukuda and criteria for CFS, (2) had evidence of refractory orthostatic intolerance, (3) were unable to work or attend school, and (4) were minimally responsive to medical and psychiatric management. To investigate pathological reflex findings, all underwent MRI evaluations. CSS was considered present if the AP cervical spinal canal diameter (SCD) was less than 10 mm at any level. Overall function was assessed before and after cervical disc replacement surgery using (1) a clinician-assigned Karnofsky score (range 0 to 100) and (2) the SF-36 physical function (PF) subscale score (range 10-30). Higher scores indicate better function on both measures.

Results: Age at onset of symptoms was 12, 29, and 29 years. The onset of ME/CFS was acute in all three. Neurological exam findings included > 3+ (brisk) deep tendon reflexes (DTR) in 2/3, positive Hoffman sign in 2/3, tremor in 2/3, and absent gag reflex in 1/3. Diagnosis was delayed for 6-9 years after the onset of symptoms. Brain MRIs were normal. The youngest patient had congenital CSS with a single level disc protrusion at C5-6 that caused further ventral cord compression and a SCD of 7 mm. Her mother also has cervical stenosis. A second
patient had two disc protrusions at C5-6 and C6-7 with SCD of 7 and 9 mm, and myelomalacia (this patient has a sibling with Chiari I malformation). The third had acquired CSS due to a single level disc bulge at C5-6 (SCD = 8.5 mm).

Improvements were evident within 2 months of single-level cervical disc replacement surgery (one patient also had fusion at an adjacent level). After 16-40 months of follow-up, all reported improved fatigue, cognitive dysfunction, PEM, lightheadedness, and anxiety. The pre- to post-op SF-36 PF scores improved from 13 to 30, 18 to 30, and 16 to 26, respectively, and the Karnofsky scores improved from 40 to 90, 40 to 90, and 50 to 100, respectively. Standing tests conducted at variable intervals from pre- to post-op showed a reduction in the maximal heart rate (HR) change during 5 minutes of standing from 64 to 22 bpm, 42 to 29 bpm, and 34 to 27 bpm, respectively.

Conclusion: This case series draws attention to the potential for CSS to contribute to ME/CFS and orthostatic symptoms, extending work by Heffez in fibromyalgia (Eur Spine J 2004;13:516). Further work is needed to define indications for surgery. However, the improvements in HR and function following surgery emphasize the importance of detecting and treating CSS, especially in the subset of those with ME/CFS whose severe symptoms are refractory to other interventions.

Peter C. Rowe, M.D.
Professor of Pediatrics
Johns Hopkins University School of Medicine/200 N. Wolfe Street/Room 2077
Baltimore, MD 21287
prowe@jhmi.edu

Dr. Rowe is supported by the Sunshine Natural Wellbeing Foundation Professorship in Chronic Fatigue and Related Disorders. No author has a conflict of interest.

Source: Peter C. Rowe, M.D*, Colleen L. Marden, Scott Heinlein, PT, Charles Edwards II, M.D. Cervical spine stenosis as a cause of severe ME/CFS and orthostatic intolerance symptoms. Poster presentation, IACFS/ME 2016 conference.

Being homebound with chronic fatigue syndrome: A multidimensional comparison with outpatients.

Abstract:

Many patients with chronic fatigue syndrome (CFS) seem to experience periods in which they are homebound due to their symptomatology. Despite a growing body of research about CFS, little is known about patients who no longer feel able to leave their homes.

The purpose of the present study was to examine whether homebound patients differ from other CFS patients on illness-specific characteristics. Besides experiencing more impairment in daily functioning than participants of an outpatient intervention study, homebound patients were characterised by extremely high levels of daily fatigue, predominant somatic attributions, and pervasively passive activity patterns. The course of symptomatology was similarly stable in both groups.

Our findings suggest that homebound patients form a distinct subgroup of CFS patients who might profit from a treatment approach that is tailored to their specific needs. The exploratory nature of this first systematic investigation of homebound CFS patients is stressed, and suggestions for future research are made.

Source: Wiborg JF, van der Werf S, Prins JB, Bleijenberg G. Being homebound with chronic fatigue syndrome: A multidimensional comparison with outpatients. Psychiatry Res. 2010 May 15;177(1-2):246-9. doi: 10.1016/j.psychres.2010.02.010. Epub 2010 Mar 5. https://www.ncbi.nlm.nih.gov/pubmed/20207012

Severe versus Moderate criteria for the new pediatric case definition for ME/CFS

Abstract:

The new diagnostic criteria for pediatric ME/CFS are structurally based on the Canadian Clinical Adult case definition, and have more required specific symptoms than the (Fukuda et al. Ann Intern Med 121:953-959, 1994) adult case definition.

Physicians specializing in pediatric ME/CFS referred thirty-three pediatric patients with ME/CFS and 21 youth without the illness. Those who met ME/CFS criteria were separated into Severe and Moderate categories. Significant differences were found for symptoms within each of the six major categories: fatigue, post-exertional malaise, sleep, pain, neurocognitive difficulties, and autonomic/neuroendocrine/immune manifestations.

In general, the results showed participants who met the Severe ME/CFS criteria reported the highest scores, the Moderate ME/CFS group show scores that were a little lower, and the control group evidenced the lowest scores. Findings indicate that the Pediatric Case Definition for ME/CFS can distinguish between those with this illness and controls, and between those with Severe versus Moderate manifestations of the illness.

Source: Jason L, Porter N, Shelleby E, Till L, Bell DS, Lapp CW, Rowe K, De Meirleir K. Severe versus Moderate criteria for the new pediatric case definition for ME/CFS. Child Psychiatry Hum Dev. 2009 Dec;40(4):609-20. doi: 10.1007/s10578-009-0147-8. Epub 2009 Jun 10.https://www.ncbi.nlm.nih.gov/pubmed/19513826

The course of severe chronic fatigue syndrome in childhood

Abstract:

Little has been reported on prognostic indicators in children with chronic fatigue syndrome (CFS). We used interviews with children and parents, a mean of 45.5 months after illness onset, to follow up 25 cases of CFS referred to tertiary paediatric psychiatric clinics. At its worst, the illness had been markedly handicapping (prolonged bed-rest and school absence in two-thirds); mean time out of school was one academic year. Two-thirds, however, had recovered and resumed normal activities–mean duration of illness to recovery/assessment 38 months–and none had developed other medical conditions. Recovery was associated with specific physical triggers to the illness, with start of illness in the autumn school term and with higher socioeconomic status. Severe fatigue states in children can cause serious and longlasting handicap but most children recover.

Comment in: Chronic fatigue syndrome in mother and child. [J R Soc Med. 2000]

Source: Rangel L, Garralda ME, Levin M, Roberts H. The course of severe chronic fatigue syndrome in childhood. J R Soc Med. 2000 Mar;93(3):129-34. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1297949/ (Full article)

Can we predict recovery in chronic fatigue syndrome?

Abstract:

PURPOSE: To determine if selected demographic or clinical features of chronic fatigue syndrome (CFS) are associated with recovery.

PATIENTS AND METHODS: A follow-up questionnaire was mailed to 341 patients who had been ill on average for nine years to ascertain “recovery” rate (defined as self-reported recovery on a visual analog scale). Baseline demographic and clinical features (functional status and psychological status) recorded at the time of the initial (baseline) clinical visit were analyzed for their association with recovery at the time of follow-up.

RESULTS: Of the 177 patients who responded to the follow-up questionnaire, only 21 (12%) reported “recovery.” Patients with higher levels of physical and social functioning and lower levels of anxiety and obsessive-compulsiveness at baseline were more likely to report recovery at follow-up (p < 0.05). No specific demographic characteristics were associated with recovery.

CONCLUSION: These findings support previous research that complete recovery from CFS is rare and that patients with less severe illness at the initial clinic visit are more likely to have a positive prognosis for recovery. However, considerable overlap in illness severity was observed between the recovered and nonrecovered groups, suggesting that accurate prediction of recovery in individual CFS patients is not currently feasible.

Source: Pheley AM, Melby D, Schenck C, Mandel J, Peterson PK. Can we predict recovery in chronic fatigue syndrome? Minn Med. 1999 Nov;82(11):52-6. http://www.ncbi.nlm.nih.gov/pubmed/10589213

Absence of evidence of Borna disease virus infection in Swedish patients with Chronic Fatigue Syndrome

Abstract:

Chronic Fatigue Syndrome (CFS) is characterized by debilitating fatigue, somatic symptoms and cognitive impairment. An infectious basis has been proposed; candidate agents include enteroviruses, herpesviruses, retroviruses and Borna disease virus (BDV), a novel neurotropic virus associated with neuropsychiatric disorders.

Sera and peripheral blood mononuclear cells (PBMC) from Swedish CFS patients were assayed for evidence of infection using ELISA and Western immunoblot for detection of antibodies to BDV proteins N, P and gp18; and using nested reverse transcriptase polymerase chain reaction (RT-PCR) for detection of BDV N- and P-gene transcripts. No specific immunoreactivity to BDV proteins was found in sera from 169 patients or 62 controls.

No BDV N- or P-gene transcripts were found through RT-PCR analysis of PBMC from 18 patients with severe CFS. These results do not support a role for BDV in pathogenesis of CFS.

Source: Evengård B, Briese T, Lindh G, Lee S, Lipkin WI. Absence of evidence of Borna disease virus infection in Swedish patients with Chronic Fatigue Syndrome. J Neurovirol. 1999 Oct;5(5):495-9. http://www.ncbi.nlm.nih.gov/pubmed/10568886

Natural history of severe chronic fatigue syndrome

Abstract:

OBJECTIVE: To evaluate the natural history of chronic fatigue syndrome (CFS) in a severely ill group of patients at three points in time.

DESIGN: Patients were enrolled from April 1992 to February 1994 and were evaluated three times. Time 1 (at enrollment): history, physical evaluation, and psychiatric evaluation; Time 2 (median = 1.6yrs after initial evaluation): postal questionnaire to assess current condition; Time 3 (median = 1.8 yrs after Time 2): medical and psychiatric evaluations.

SETTING: The New Jersey CFS Cooperative Research Center, an ambulatory setting.

PATIENTS: Twenty-three patients fulfilled the 1988 case definition for CFS and had symptom complaints that were substantial or worse in severity. All patients were ill less than 4.5 years; and none had a DSM-III-R psychiatric disorder in the 5 years before illness onset; none had substance abuse in the 10 years before enrollment.

MAIN OUTCOME MEASURES: Severity of CFS symptoms was assessed by self-report questionnaires, laboratory tests, and medical examination. Psychological status was assessed using the Q-D15 and the Centers for Epidemiological Study-Depression Scale. At each time of evaluation, patients were categorized as severe, slightly improved, improved, and recovered.

RESULTS: Over the 4 years of the study, 13 patients remained severely ill, 9 improved but still fulfilled the 1994 case definition for CFS, and 1 recovered. Illness duration, mode of onset, psychiatric status or depressed mood at intake, or chemical sensitivity did not predict illness outcome. One patient was diagnosed with an alternate illness, but it probably did not explain her CFS symptoms. Mood improved for those patients whose illness lessened.

CONCLUSIONS: The prognosis for recovery was extremely poor for the severely ill subset of CFS patients. The majority showed no symptom improvement and only 4% of the patients recovered. Illness severity between Times 2 and 3 remained stable.

Source: Hill NF, Tiersky LA, Scavalla VR, Lavietes M, Natelson BH. Natural history of severe chronic fatigue syndrome. Arch Phys Med Rehabil. 1999 Sep;80(9):1090-4. http://www.ncbi.nlm.nih.gov/pubmed/10489014

Chronic fatigue syndrome. Role of psychological factors overemphasised

Comment in: Chronic fatigue syndrome and myalgic encephalomyelitis. [BMJ. 1994]

Comment on: Longitudinal study of outcome of chronic fatigue syndrome. [BMJ. 1994]

Editor,-In concluding that psychological factors are more important than immunological ones in determining the long term outcome of myalgic encephalomyelitis or the chronic fatigue syndrome Andrew Wilson and colleagues seem overconfident of the validity of their findings. Although the use of self rated measures of outcome is necessary, the validity of the investigators’ treatment of such data is questionable. For example, the five point self rated global illness outcome was dichotomised such that an original response of “not improved at all” was recorded to “worsened”-a decision the investigators fail to justify. It is also dubious whether patients’ recall of their own premorbid psychological state is accurate, given that the average onset was 9 years before recall and the finding that memory of an event is affected by subsequent events.

You can read the rest of this comment here: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2540179/pdf/bmj00440-0053a.pdf

Source: Blatch C, Blatt T. Chronic fatigue syndrome. Role of psychological factors overemphasised. BMJ. 1994 May 14;308(6939):1297. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2540179/

A chronic illness characterized by fatigue, neurologic and immunologic disorders, and active human herpesvirus type 6 infection

Abstract:

OBJECTIVE: To conduct neurologic, immunologic, and virologic studies in patients with a chronic debilitating illness of acute onset.

DESIGN: Cohort study with comparison to matched, healthy control subjects.

PATIENTS: We studied 259 patients who sought care in one medical practice; 29% of the patients were regularly bedridden or shut-in.

MAIN OUTCOME MEASURES: Detailed medical history, physical examination, conventional hematologic and chemistry testing, magnetic resonance imaging (MRI) studies, lymphocyte phenotyping studies, and assays for active infection of patients’ lymphocytes with human herpesvirus type 6 (HHV-6).

MAIN RESULTS: Patients had a higher mean (+/- SD) CD4/CD8 T-cell ratio than matched healthy controls (3.16 +/- 1.5 compared with 2.3 +/- 1.0, respectively; P less than 0.003). Magnetic resonance scans of the brain showed punctate, subcortical areas of high signal intensity consistent with edema or demyelination in 78% of patients (95% CI, 72% to 86%) and in 21% of controls (CI, 11% to 36%) (P less than 10(-9)). Primary cell culture of lymphocytes showed active replication of HHV-6 in 79 of 113 patients (70%; CI, 61% to 78%) and in 8 of 40 controls (20%; CI, 9% to 36%) (P less than 10(-8], a finding confirmed by assays using monoclonal antibodies specific for HHV-6 proteins and by polymerase chain reaction assays specific for HHV-6 DNA.

CONCLUSIONS: Neurologic symptoms, MRI findings, and lymphocyte phenotyping studies suggest that the patients may have been experiencing a chronic, immunologically mediated inflammatory process of the central nervous system. The active replication of HHV-6 most likely represents reactivation of latent infection, perhaps due to immunologic dysfunction. Our study did not directly address whether HHV-6, a lymphotropic and gliotropic virus, plays a role in producing the symptoms or the immunologic and neurologic dysfunction seen in this illness. Whether the findings in our patients, who came from a relatively small geographic area, will be generalizable to other patients with a similar syndrome remains to be seen.

Comment in:

The chronic fatigue syndrome controversy. [Ann Intern Med. 1992]

Source: Buchwald D, Cheney PR, Peterson DL, Henry B, Wormsley SB, Geiger A, Ablashi DV, Salahuddin SZ, Saxinger C, Biddle R, et al. A chronic illness characterized by fatigue, neurologic and immunologic disorders, and active human herpesvirus type 6 infection. Ann Intern Med. 1992 Jan 15;116(2):103-13. http://www.ncbi.nlm.nih.gov/pubmed/1309285