Keywords:
Nervous System Diseases - Conversion Disorder - Movement Disorders - Rehabilitation
Palavras-chave:
Doenças do Sistema Nervoso - Transtorno Conversivo - Transtornos do Movimento - Reabilitação
Over time, disorders that cause unstable body motion and control, inconsistent and
incongruent with any recognizable neurological disease, have received a variety of
names, such as hysterical paraplegia, psychogenic movement disorder and conversion
disorder[1]. In this paper, we use the term functional neurological disorder (FND)[2].
FND is heterogeneous. The present report mainly focuses on FND presenting with motor
symptoms (mFND), such as dystonia, paralysis, tremor, imbalance, etc. Usually, it
is accompanied by a wide range of other somatic features, such as dizziness, headaches
and non-epileptic seizures[3].
Previously, this diagnosis of mFND was made only in the absence of a medical explanation
for the symptoms, on the basis of history features alone. Currently, the diagnosis
relies in the presence of positive signs: multiple symptoms and other functional symptoms
(chronic fatigue, fibromyalgia, and asthma), clear evidence of physical signs of internal
inconsistency such as Hoover's sign, gait changes, fluctuation of symptoms, presentations
incompatible with scientific knowledge, and lack of positive findings in imaging and
laboratory studies[4].
By definition, mFND is a condition that can resemble other neurological conditions,
so the accuracy of diagnosis must be reassured. Studies conducted in the 1960s reported
a 29% rate of misdiagnosis. Currently, after substantial scientific advances and the
development of more accurate criteria, the rate of false positives has dropped to
4%[1],[5].
While the complex relations between mind and body motion remain obscure, the burden
of mFND is high; FND represents an important socioeconomic challenge accounting for
between 1 in 20 and 1 in 10 neurologic presentations. Several studies have reported
a high rate of unemployed patients, ranging from 43 to 89%[6],[7]. Symptoms persistence may alter quality of life to the same degree of a severe neurological
condition such as Parkinson’s disease[8],[9].
Studies indicate that mFND disturbances require a multidisciplinary treatment approach.
The importance of how to communicate the problem and implement a highly integrated
treatment has been emphasized. Overall, studies indicate that the evolution of motor
alterations secondary to functional neurological disorder has a poor prognosis. A
recent review of 22 studies on the subject suggests that 33 to 66% of patients evolved
without modification or worsening of symptoms[4],[10]. Factors correlated with better prognosis are related to good physical health, negative
psychiatric history, and a history of acute onset and short duration, triggered by
a marked stress factor[6].
In this paper we describe the epidemiological profile, symptom pattern, and outcome
of patients with motor alterations resulting from functional neurological disorder
admitted to the Belo Horizonte unit of the SARAH Network of Rehabilitation Hospitals
from January 1997 to June 2018.
METHODS
This is a retrospective chart review based on electronic records of patients admitted
to the SARAH Rehabilitation Hospital in Belo Horizonte from January 1997 to June 2018.
The search for electronic records was performed from the records of the rehabilitation
team: neurologists, clinicians, psychiatrists, psychologists, and physiotherapists,
among others. Somatization disorder (F-45 ICD10) and the terms in Portuguese "conversão" (conversion), "psicogênico" (psychogenic), "somático" (somatic) and "somatoforme" (somatoform) were searched. This search resulted in 1,015 records.
Exclusion criteria were patients who came for just an evaluation and dropped out of
care. Patients with sudden and transient conditions, usually blackouts, vertigo and
paralysis; patients with painful disorders, in which pain was the main symptom without
any change in movement, and patients with known neurological diseases whose symptoms
are exacerbated by psychic issues. Additionally, 10% of the patients who had mFND
as one of the diagnostic hypotheses in their first evaluation at the hospital, ultimately
had the diagnosis discarded throughout medical evaluation, they were also eliminated
from the sample.
In the end, 185 patients were selected. All of them had motor changes in the lower
and/or upper limbs and/or trunk with and without accompanying gait changes. The diagnosis
was not a diagnosis of exclusion. All patients underwent neurological exams and 86%
were examined by more than three experienced rehabilitation professionals. All patients
met the diagnostic criteria for mFND: historical background, usually with multiple
somatizations, variability of severity and distribution of symptoms; clinical examination
presenting atypical, incongruous and inconsistent motor patterns; laboratory and image
findings incompatible with organic disease[2].
Clinical characteristics, risk factors, psychiatric comorbidities, treatment and prognoses
of the selected patients were delineated.
The age of patients was considered as the age at which symptoms appeared and not age
upon admission for rehabilitation care. The endpoint was the date when patients had
the last visit or the end of follow-up in June 2018, whichever occurred first. All
clinical diagnostic and physical evaluation criteria followed standardized international
guidelines.
Independent variables were:
-
Socio-epidemiological: sex, age, marital status, and level of education.
-
Lifestyle: alcohol or tobacco consumption or drug use.
-
Clinical: presence of pain and comorbidities such as psychiatric disturbances, lower
urinary tract dysfunction, blackout episodes.; and
-
Physical signs: weakness or paralyses and the use of some type of support for locomotion.
Outcome was considered as: remission of symptoms, moderate improvement (50%) or marked
improvement (75%), patients who had periods of improvement and worsening, and patients
who got worse. Moderate and marked improvements were defined considering pain reduction,
increased mobility, participation in daily activities and working status.
Statistical analysis
After data collection, the records were entered into Excel spreadsheets. The description
of patients’ epidemiological, sociodemographic and clinical characteristics was made
in the same program. Next, an exploratory data analysis was performed with the Statistical
Package for the Social Sciences (SPSS) 20.0 to examine the characteristics of the
population studied. The chi-square and Fisher's exact tests were used to compare the
proportions of the categorical variables. For comparisons between means and medians,
the nonpaired and Mann-Whitney t tests, respectively, were used. Two-tailed significance
level considered was p<0.05.
RESULTS
In the SARAH unit of the Belo Horizonte, the number of patients diagnosed with mFND
has been growing. Although it is increasing, this diagnosis with no other neurological
condition represents less than 0.5% of all diagnoses made per year.
Among the 185 patients of our sample, there was a predominance of women (75.1%), 50.8%
were married, 92 patients (49.7%) had gone to school nine years or more, which is
a higher level of education than the average of the hospital population. The youngest
patient was 3 years old, and the oldest patient was 69 years old. The mean age of
symptoms onset was 28.8 years (SD±13.3 years). One-quarter of the subjects were less
than 18 years of age at the time of first symptom. Among those, mean age was 11.2
years (SD 3.6). 80% of them received inpatient treatment, always accompanied by at
least one of their parents.
Associated psychiatric conditions, mainly depression and anxiety, were identified
in 77.2% of the patients. There were no cases of alcoholism or drug addiction, and
only 7% of patients used tobacco ([Table 1]).
Table 1
Clinical and epidemiological profiles of 185 patients with mFND.
|
Features
|
n (%)
|
|
Sex
|
|
Female
|
139 (75.1%)
|
|
Male
|
46 (24.9%)
|
|
Age in years
|
|
Mean (standard variation)
|
28.8 (±13.3)
|
|
Minimum
|
3
|
|
Maximal
|
69
|
|
Less 18 years
|
47 (25.4%)
|
|
18+ years
|
138 (74.6%)
|
|
Schooling
|
|
In school
|
37 (20%)
|
|
1 to 8 years
|
56 (30.3%)
|
|
9+ years
|
92 (49.7%)
|
|
Symptoms
|
|
Depression/Anxiety
|
77.2%
|
|
Pain
|
84 (45.4%)
|
|
Limb weakness or paralysis Imbalance
|
185 (100%) 41 (22.2%)
|
|
Tremors
|
23 (18.9%)
|
|
Dystonia Sensitive changes
|
5 (2.7%) 101 (54.6%)
|
|
Dizziness
|
39(29%)
|
|
Blackout episodes
|
36 (19.5%)
|
|
Migraine
|
67 (36.2%)
|
|
Bladder complaints
|
54 (29.2%)
|
|
Onset disability
|
|
Onset less than 24 hours
|
108 (58.4%)
|
|
Onset less than 30 days
|
24 (13%)
|
|
Onset 30+ days
|
53 (28.6%)
|
|
Locomotion
|
|
Wheelchair-bounded
|
44 (23.8%)
|
|
Ambulatory only inside home
|
33 (17.8%)
|
|
Ambulatory
|
89 (48.1%)
|
|
Dependent of caregiver
|
33 (17.8%)
|
|
One walking stick
|
29 (15.7%)
|
|
Two walking sticks
|
10 (5.4%)
|
|
Walker
|
4 (2.2%)
|
In most cases (58.4%), symptoms started abruptly, in less than 24 hours, and in 45.4%
of the cases, the initial manifestation was pain. Upon physical evaluation, 81.1%
of patients showed weakness in the lower limbs, with 23.8% requiring the use of wheelchairs;
50% had upper limb involvement. In total, 22.2% of patients had balance impairment,
18.9% had tremors, 2,7% had dystonia and 54.6% had associated changes in sensitivity.
A total of 19.5% had blackout episodes, 29% had dizziness, 29.2% had abnormal functioning
of the lower urinary tract, manifesting as voiding dysfunction, storage dysfunction,
or both. In addition, 36.2% of the sample had migraines.
All patients were communicated about the diagnosis and 30.3% of them do not participate
in the rehabilitation program. Those were followed up for a period that ranged from
one month to two years, with median of six months.
Those who did undergo rehabilitation were followed up for a period ranging from one
month to 12 years, with median of 13 months (DP 46). Among them, 53.4% did so on an
inpatient basis. SARAH receives patients from different cities and states of Brazil.
Being in or outpatient is a decision made mainly based on the distance of patient´s
home and it was not a significant outcome variable.
The rehabilitation package may include physical and occupational activities, nursing
and nutrition guidance, psychiatric and psychological support. The program is developed
according to the demands and availability of each patient. In our sample, all patients
received physical therapy and 140 patients underwent at least one mental health evaluation
(psychiatrist and/or psychologist. Family participation is always encouraged).
In the follow-up, 35.9% did not have any changes in status, and 8.2% continued to
use a wheelchair. A total of 21.2% of patients had complete remission of their symptoms,
and 3.2% of them had spontaneous remission. 33,7% had moderate or marked improvement,
6.5% had periods of improvement and worsening, and 2.7% had worsening. There were
no demographic differences between those who improved and did not improved.
In addition to the 45.5% of patients who had pain as initial symptom, 39.5% complained
about pain during follow-up. Another etiology for the symptoms was not found in any
of the 185 patients.
Patients with acute evolution of symptoms (<24 hours) seemed 12x more likely to improve
than those with a slow evolution of symptoms (chi-square 12.1). Mental health monitoring
appeared to be a relevant variable in the improvement process (p=0.02). Patients who
participated in a rehabilitation program were five times more likely to be cured than
patients who did not participate in a rehabilitation program ([Table 2]).
Table 2
Outcome of 185 patients with mFND.
|
n (%)
|
|
|
|
|
No improvement
|
83 (44.9%)
|
|
|
|
|
Improvement
|
101 (55.1%) [Complete recovery of all symptoms n=39 (21.2%)]
|
|
|
|
|
Rehabilitation
|
|
No
|
56 (30.3%)
|
|
|
|
|
Yes
|
129 (69.7%)
|
|
|
|
|
Improvement
|
Rehabilitation No
|
Rehabilitation Yes
|
95%CI
|
chi-square p-value
|
|
No
|
46
|
38
|
11.01(4.9–23.5)
|
<0.0001
|
|
Yes
|
10
|
91
|
|
|
|
56
|
129
|
|
|
|
Improvement
|
Age under 18 years
|
Age 18+
|
95%CI
|
chi-square p-value
|
|
No
|
7
|
77
|
7.2 (3.0–17.7)
|
<0.0001
|
|
Yes
|
40
|
61
|
|
|
95%CI: 95% confidence interval.
Among 129 patients who received rehabilitation, 70.5% had partial (50%) or marked
(75%) improvement and, from those, 36,2% had complete remission of symptoms ([Table 3]).
Table 3
Outcome of 129 patients with mFND after rehabilitation.
|
n (%)
|
|
No improvement
|
38 (29.5%)
|
|
Improvement
|
91 (70.5%)
[Completely recovered from all symptoms n=33 (36.2%)]
|
|
Improvement after rehab
|
Age under 18 years
|
Age 18+
|
chi-square
|
p-value
|
|
No
|
1
|
37
|
16.3
|
<0.0001
|
|
Yes
|
34
|
57
|
Of the 47 patients younger than 18 years of age, 35 participated in rehabilitation,
and, of these, 26 had complete remission of symptoms. Only one younger patient did
not improve after rehabilitation ([Table 3]). Children younger than 18 years old improved significantly more than adults and
chronic mFND patients (p<0.001).
Children and adults who improved and healed did so primarily in the first six months
of rehabilitation, with younger patients tending to improve faster than adults. Among
these, the remission of symptoms was achieved within 1.6 month of treatment on average,
while for adults it happened within 4.4 months of treatment on average (p=0.04).
Patients with history of blackouts, altered sensitivity (p=0.02) and associated imbalance
(p<0.01), with evolution of symptoms lasting more than a year (p<0.01) and with lower
urinary tract dysfunction (p=0.02) had a worse prognosis.
DISCUSSION
Over the last two decades, we have seen increasing recognition of mFND[11]. In the SARAH unit of Belo Horizonte, although growing, the number of patients remain
small, but representative. Those patients challenge knowledge in various stages of
the rehabilitation process: diagnostic investigation, problem communication, and treatment.
This study presents a large number of patients from the same unit who were followed
up by a multidisciplinary team, some for years. The sample is heterogeneous with respect
to age, which allows for comparison between children and adults.
It is noteworthy that, after initiation of rehabilitation, an organic etiology for
the symptoms was not found in any of our patients. The literature points out that
4% of patients usually has the wrong diagnosis after an average of five years of follow-up[5],[12]. Our data is certainly consequence of scientific advances and accurate criteria.
As described in the literature, movement disorders in our population are characterized
by a mixed pattern of weakness and sensory disturbance. Many patients also complain
of other functional disorders, fatigue, dizziness, and so on. The severity of symptoms
is seen as a great disability and renders patients unable to work. The number of people
receiving social security benefits reflects the functional and social damage caused
by this disease.
Many of our findings confirm the results of other studies. The predominance of females
with the disorder is one of them. This might suggest hormonal or genetic specificity
related to sex; however, the 25% prevalence in male patients questions this line of
reasoning.
Many patients in our population were in the third and fourth decade of life. The literature
indicates that the mean age of onset of symptoms is between 35 and 50 years of age[11]. The cases that involve very young children (7% in children less than 10 years of
age), as well as the almost absence of cases in elderly patients are noteworthy.
For the onset of symptoms, almost 60% of our subjects developed them suddenly, which
is within the range described in the literature, between 54 and 92%.
The literature shows a strong association between FND and psychiatric disorders: rates
of depression between 20 and 40% and a rate of anxiety of 38%[3]. In our sample, this prevalence was even greater, providing clear support for the
notion that emotional factors are a significant risk factor. All of our patients were
referred for mental health services, and those who underwent such treatment improved
more than those who underwent only medical and physical therapy treatments. Those
who received mental health treatment by psychiatrists and psychologists who were members
of the rehabilitation team improved more than patients receiving care from an external
service.
Overall, almost 70% of our patients who participated in the rehabilitation program
showed improvements. This result is better than most data presented in the literature,
where 66 to 100% of patients had the same or worse symptoms at follow-up (n=135)[11].
The favorable evolution of patients who participated in rehabilitation, all having
physical therapy treatment, is similar to the result presented by Nielsen et al.[13]. In their study 72% of patients with Functional Motor Symptoms who submitted to
physical therapy rated their symptoms as improved. Other studies also demonstrate
the feasibility of physical therapy as intervention for patients with mFND[14].
The better outcome of acute and young patients in relation to chronic patients also
confirms the data available in the literature[11],[15], but there are cases where patients with a long-term evolution of symptoms, after
beginning rehabilitation, improved rapidly. In our sample, patients who did not improve
during the first year of treatment had poorer outcomes compared to those who did.
There is only one published study on lower urinary tract dysfunction in patients with
FND. It was a retrospective review of 150 patients diagnosed with definite or probable
functional movement disorders between 2006 and 2014 from the National Hospital of
Neurology and Neurosurgery in London. We found no studies associating urinary symptoms
with the outcomes of FND, but the dysfunction is known to negatively affect quality
of life in other neurological disorders[15]. Our study suggests that mFND patients with lower urinary tract syndrome also have
worse prognosis than patients without it. Further studies are required to clarify
the association between urinary dysfunction and functional neurological disorder.
In contrast to what was previously reported by Nielsen et al.[16], in our sample, whether patients used locomotion aids was not significant for their
outcome. Interestingly, patients who were treated on an inpatient basis, viewing many
patients with neurological disorders, did not worsen or present with new symptoms[17].
The fact that better outcomes were obtained for children compared to adults is an
important point. Among children under 10 years old, 80% had remission of symptoms,
and all of them improved. Perhaps the good outcome of our young patients is related
to the fact that most of them had close family interventions associated with their
psychological and physical treatments. The only patient under 18 who did not improve
has been followed up for ten years and is now 27 years old, so far still using a wheelchair.
Motor FND is a highly disabling, daunting public health condition. Treating symptoms
early, both in relation to age and onset time, favors positive outcomes. It is assumed
that, over time, the mechanisms that underlie the process cause a dysfunctional relationship
between mind and body. Good prognosis is related to acute cases, which occur in young
individuals who are treated at the beginning of the disease course in a rehabilitation
program.
We acknowledge a number of limitations to this work. This study is retrospective,
so the variables recorded did not use the some proforma. There is no scale that defines
what is "moderate" or "marked" improvement. Also, we have a shorter period of follow-up
of those who did not participate in the rehabilitation program; this could have strongly
influenced prognostic factors. No participation may suggest patient’s refusal in accepting
diagnosis, which is known as a main barrier for the treatment of patients with FND[18], but may also reflect the “hard to treat” beliefs of the staff. Follow-up was naturalistic,
and this may also have influenced prognostic factors. Furthermore, as a rehabilitation
hospital, we may have an over-representation of severe cases, and the bias of the
over-representation of those who agreed to the treatment. Prospective analysis of
rehabilitation interventions and the determinants of their benefits is required to
target better outcomes for these patients.
