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Bilateral brachial plexus neuritis following parvovirus B19 and cytomegalovirus infection.

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of the deficiency of P-dystroglycan, since P-dystroglycan is believed to be crucial for linking dystrophin and
a-dystroglycan to the sarcolemma. Although it is quite
unlikely that the normal molecular organization of the
complex is maintained without P-dystroglycan, the
present finding suggests that there may be another dystrophin-anchoring site, possibly in the sarcoglycan
complex. Also, there might be a direct interaction between a-dystroglycan and the sarcoglycan complex for
maintaining the membrane organization of a-dystroglycan without P-dystroglycan. These interesting possibilities should be studied by further biochemical experiments.
References
1. Ervasti JM, Ohlendieck K, Kahl SD, et a]. Deficiency of a
glycoprotein component of the dystrophin complex in dystrophic muscle. Nature 1990;345:315-319
2. Ibraghimov-Beskrovnaya 0, Ervasti JM, Leveille CJ, et al. Primary structure of dystrophin-associated glycoproteins linking
dystrophin to the extracellular matrix. Nature 1992;355:696702
3. Campbell KP. Three muscular dystrophies: loss of cytoskeleton-extracellular matrix linkage. Cell 1995;80:675-679
4. Ohlendieck K, Matsumura K, Ionasescu W, et al. Duchenne
muscular dystrophy: deficiency of dystrophin-associated proteins in the sarcolemma. Neurology 1993;43:795-800
5. Roberds SL, Leturcq F, Allamand V, et al. Missense mutations
in the adhalin gene linked to autosornal recessive muscular dystrophy. Cell 1994;78:625-633
6. Lim LE, Duclos F, Broux 0, et al. P-Sarcoglycan: characterization and role in limb-girdle muscular dystrophy linked to
4q12. Nature Genet 1995;11:257-265
7. Jung D, Leturcq F, Sunada Y, et al. Absence of y-sarcoglycan
(35DAG) in autosomal recessive muscular dystrophy linked to
chromosome 13q12. FEBS Lett 1996;381:15-20
8. Salih MAM. Unusual muscular dystrophy in an extended Sudanese kindred. Master of Pediatrics and Child Health thesis.
University of Khartoum, 1980
9. Ben Hamida M, Fardeau M. Severe autosomal recessive limbgirdle muscular dystrophies frequent in Tunisia. In: Angelini
C, Danieli GA, Fontanari D, eds. Muscular dystrophy research:
advances and new trends. Amsterdam: Excerpta Media, 1980:
143- 146
10. Tom; FMS, Evangelista T, Leclerc A, et al. Congenital muscular dystrophy with merosin deficiency. C R Acad Sci Paris
1994;317:351-357
11. Sunada Y, Bernier SM, Kozak CA, et al. Deficiency of merosin
in dystrophic dy mice and genetic linkage of laminin M chain
gene to 4
1 locus. J Biol Chem 1994;269:13729-13732
12. Yung D, Yang B, Meyer J, et al. Identification and characterization of the dystrophin anchoring site on P-dystroglycan. J Biol
Chem 1995;270:27305-27310
13. Cullen MJ, Walsh J, Nicholson LVB. Immunogold localization
of the 43-kDa dystroglycan at the plasma membrane in control
and dystrophic human muscle. Acta Neuropathol (Berl) 1994;
87:349-354
14. Dubowitz V. Workshop report; 22nd ENMC sponsored workshop on congenital muscular dystrophy held in Baarn, the Netherlands, 14-16 May 1993. Neuromusc Disord 1994;4:75-81
15. Salih MAM, Mahdi A, Al-Rikabi AC, et al. Clinical and molecular pathological features of childhood autosomal recessive
muscuiar dystrophy in Saudi Arabia. Dcv Med Child Neurol
1996;38:262-270
928
16. Gardner-Medwin D, Walton J. The muscular dystrophies. In:
Walton J, Karpati G, Hilton-Jones D , eds. Disorders of voluntary muscle. Edinburgh: Churchill Livingstone, 1974:543-594
17. Salih MAM, Omer M U , Bayoumi RA, et al. Severe autosomal
recessive muscular dystrophy in an extended Sudanese kindred.
Dev Med Child Neurol 1983;25:43-52
18. Hoffman EP, Fiachbeck KH, Brown RH, et al. Characterization of dystrophin in muscle-biopsy specimens from patients
with Duchenne's or Becker's muscular dystrophy. N Engl J
Med 1988;3 18:1363-1368
19. Williamson R, Ibraghimov-Beskrovnaya 0, Hrstka R, et al.
Inactivation of the murine dystroglycan gene produces an early
midgestation embryonic lethal. Am J Hum Genet 1995;
57(suppl):A12
20. Vater R, Harris JB, Anderson LVB, et al. The expression of
dystrophin-associated glycoproteins during skeletal muscle degeneration and regeneration. An immunofluorescence study. J
Neuropathol Exp Neurol 1995;54:557-569
Bilateral Brachial Plexus
Neuritis Following
Parvovirus B19 a i d
Cytomegalovirus Infection
J. J. Maas, M D , * t M. F. C. Beersma, MD, PhD,$
J. Haan, M D , P h D , * t G. J. P. M . Jonkers, MD,$
and A. C. M. Kroes, MD, PhDt
A man, 23 years of age, had a typical erythema infectiosum, complicated by a severe bilateral brachial plexus
neuritis. Motor function recovered slowly and only partially after 6 months. An infection by human parvovirus
B19 was demonstrated, with strongly positive and gradually declining I@ antibodies and viral DNA detectable
in serum for more than 3 months. There was also clear
evidence of a recent infection by cytomegalovirus. The
interaction between these two viruses could be responsible for this rare and severe complication of common infections in this patient.
Maas JJ, Beersma MFC, Haan J, Jonkers GJPM,
Kroes ACM. Bilateral brachial plexus neuritis
following parvovirus B 13 and cytomegalovirus
infection. Ann Neurol 1996;40:928-932
Human parvovirus B19 infection commonly causes erythema infectiosum (fifth disease) as a mild disease of
childhood. In adult infections, arthropathy may be
From the Departments of *Neurology and DInternal Medicine,
Rijnland Hospital Leiderdorp, Leiderdorp, and tNeurology and
$Virology, University Hospital Leiden, Leiden, The Netherlands.
Received Mar 18, 1996, and in revised form Jun 11. Accepted for
publication Jun 12, 1996.
Address correspondence to Dr Haan, Department of Neurology,
Leiden University Hospital, PO Box 9600, 2300 RC Leiden, The
Netherlands.
Copyright 0 1996 by the American Neurological Association
prominent and infection during pregnancy can lead to
fetal death d u e to a disorder of fetal erythropoiesis.
Parvovirus B 19 only replicates in erythroid precursor
cells, which is also evident from its severe effects in
patients with chronic hemolytic anemias, in whom
aplastic crisis may occur. A reliable diagnosis of h u m a n
parvovirus infection is possible since serological techniques became available in the late 1980s.
Neurological complications of parvovirus infection,
considered rare, include encephalopathy [ 1, 21 a n d disturbance of consciousness [ 3 ] . Brachial plexus neuritis
associated with parvovirus infection in adult patients
appears t o be the most frequent neurological complication, described in 5 cases [4-81. A fairly consiscent
clinical pattern is apparent, b u t its pathogenesis is unexplained. The prevalence is uncertain as the frequency
of search for parvovirus infection in brachial neuritis
is unknown.
We recently cared for a n immunologically healthy
m a n with severe bilateral brachial plexus neuritis, in
which parvovirus B 19 infection was demonstrated by
several techniques, including detection of viral DNA.
T h e r e was also evidence of a recent infection by cytomegalovirus (CMV). Brachial plexus neuritis has been
described before in association with CMV infection,
albeit less frequently and less convincingly documented
[9, 101. T h e relationship, between these combined viral
infections a n d the severe long-standing manifestations
in this patient, is discussed.
Case Report
A 23-year-old man was admitted to our outpatient clinic
with weakness and tingling in both arms. Ten days before
admission, he had a flu-like episode with a cold and a warm
shaky feeling, without fever, coughing, or headache. Three
days after onset he developed a maculopapular red rash on
the arms, back, and abdomen (sparing the legs and the face),
which disappeared after 1 day. One day later, pain in the
shoulders and arms developed, followed by tingling in the
right hand and the left thumb. Two days later, he was unable
to move the left thumb and to elevate the left wrist. Due to
severe shoulder pain, he was not able to elevate his arms
above the level of the shoulders. There was neither pain,
paresthesia, or loss of strength in the legs and no neck pain.
He took no medication. In 1988 he had an episode with
pain in the left arm, followed by a dropping hand, without
trauma. An electromyogram at that time was normal, and
the symptoms of this “radial palsy of unknown origin” disappeared spontaneously within 2 months. His history was uneventful otherwise and he was an active sportsman. The patient had been vaccinated against poliomyelitis in his youth.
O n admission, shoulder and upper-arm movements were
very painful, rendering determination of proximal muscle
strength in the arms impossible. There was no winging of
the scapulae. The extensors of the fingers and the musculus
opponens pollicis at the right side showed a moderate loss
of strength and the left finger extensors and wrist elevators
were paralyzed. Both biceps and triceps reflexes were absent.
Pain, temperature, and tactile sensation in the right hand
and left thumb were diminished. Abdominal reflexes were
normal. The legs showed normal strength, sensation, and
reflexes. General physical examination was normal, including
absence of lymphadenopathy and hepatosplenomegaly. There
was no supraclavicular tenderness. Temperature never exceeded 37.9”C. Fundoscopy showed no signs of retinitis.
The pain in the shoulders decreased slowly within 2 weeks,
but serratus-type winging of the right scapula, moderate loss
of strength in both deltoidei, biceps, triceps, infra-, and supraspinate muscles, and bilateral paralysis of all muscles of
lower arms and hands developed. A numb feeling appeared
in both hands and lower arms, with diminished pain, temperature, and tactile sensation. After several weeks, pronounced atrophy of all muscles of both lower arms and
hands developed. Sensation, strength, and reflexes of the legs
remained normal.
Chest x-ray and magnetic resonance imaging of the cervical spinal cord were normal. Laboratory investigation revealed an elevated alkaline phosphate (up to 151 UIL), ?Iglutamyltransferase (223 U/L), alanine aminotransferase
(116 U/L), and creatinine phosphokinase (443 U/L), all
normal within 2 weeks. Other chemical and hematological
results were normal (including protein spectrum, glucose,
tests for rheumatoid arthritis, cryoglobulins, thyroid function, and antinuclear factor). Cerebrospinal fluid at admission showed normal cell count and protein content (42 mg/
dl), and after 9 days there was a slight pleocytosis (23 mononuclear cells/mm3) and elevated protein content (50 mg/
dl). No oligoclonal bands were present. Albumin ratio and
IgG index were normal. The first electromyogram (4 days
after admission) showed positive sharp waves in the left deltoid and right abductor digiti minimi (of 11 arm muscles
tested). Conduction velocities and compound motor activity
potential (CMAP) amplitudes of several arm and leg nerves
were normal. The second electromyogram (22 days after the
first one) showed positive sharp waves in all arm muscles
tested (both arms, proximal and distal). Motor units were
decreased in number with rapid firing rates at low level of
activation. Motor conduction velocity still was normal in
both arms, without conduction block. CMAP amplitudes,
however, were abnormally low (approximately 0.75 mV).
Human immunodeficiency virus antibodies were absent.
Serological tests for influenza A and B, hepatitis A, B, and
C , adenovirus, Mycoplasma pneumoniae, Cblamydia, Coxiella,
mumps, measles, herpes simplex, and varicella zoster were
negative and showed no changes after 14 days. Epstein-Barr
virus IgM was negative and IgG positive. IgM and IgG antibodies against Borrelia burgdorferi were negative. Treponema
pallidum hemagglutination assay and antistreptolysin test
were negative. A throat swab culture was negative for poliomyelitis virus.
Parvovirus B19 IgM antibodies, detected by both immunofluorescence and by enzyme-linked immunosorbent assay
(ELISA) [ 11, 121 were strongly positive and remained detectable until three months after admission (Fig A). Parvovirus
IgG titers were also positive and remained high during
follow-up. Parvovirus DNA was readily detectable in serum
until 3 months after admission and weakly positive in the
next 3 months (see Fig A), as demonstrated by polymerase
chain reaction (PCR) using primer sets and oligonucleotide
Brief Communication: Maas et al: Brachial Neuritis after Parvo and CMV Infection
929
Titer IFA
Ratio ELISA
12
10
10000
8
1000
6
100
4
10
Parvc
2
PCR
neg
9 Feb
20 Feb
3 Mar
5Apr
17 May
27 Jun
0
28Aug
1995
A
Ratio CMV-IgM EIA
015
9 Feb
1995
S/N Ratio CMV-IgG MElA
In
20 Feb
3 Mar
5 Apr
17 May
27 Jun
28 &g
1CMV isolation positive
(throat)
B
~~
(A) Parvovirus B19 seroloD and DNA detection. Serological jndings in specimens o f the patient, employing two techniques, ie,
enzyme-linked immunosorbent assay (ELISA) (EIA), with indication of ratio of specimen to cut08 and immunojuorescence assay
('FA), with indication o f titer. Parvovirus B19 DNA detection in specimens o f the patient, employing polymerase chain reaction
(PCR), with indication of qualitative results (? indicuting a weak borderline positive signal). (B) Cytomegalovirus (CMV) serology. Quantitative results are shown of specimens of the putient, obtuined by ELISA (EIA) technique with indication of ratio of
specimen to cutoff (fgM assay) and of signal-to-noise (S/N) ratio (positive result is > 15, f i r IgG ussay in Abbott Laboratories
IMx microparticle ELISA).
930 Annals of Neurology Vol 40
No 6
December 1996
Clinical Features o f Paruouirus-associated Brachial Plexus Neuritis Reported in Literature
Case No.
Sex
Age (yr)
Period Between Rash and
Onset of Neuritis
1
2
3
Male
Female
Female
26
23
20-40?”
Same day
2 days
Same day
4
5
Male
Female
23
38
0-5 days
Same day
6
Male
23
1 day
Clinical Outcome
ND
Recovery after 6 months
2 years: recovery of motor weakness; recurrences of paresthesia
ND
Complete resolution after
steroid therapy
6 months: partial recovery
Parvovirus DNA
in Serum
Reference
ND
ND
4 years
(intermittently)
4
5
ND
ND
7
>3 months
This report
6
8
‘No age documented in report, but patient was pregnant during course of disease.
ND
=
not documented or not done.
probe hybridization as described [13]. This PCR method
does not quantitate detectable virus DNA, but a decreasing
concentration of parvovirus DNA was considered likely because intensity of the hybridization signal became lower and
was intermittently positive and negative in the last two determinations (indicated as i in Fig A).
CMV-IgM antibodies were positive on admission and became negative 4 months later, while CMV-IgG titers raised
in this period from borderline to strongly positive (Fig B).
CMV-IgM antibodies were also found both with ELISA and
immunofluorescence techniques. CMV was cultured from a
throat swab, confirming the presence of this virus (although
this procedure is not to be considered evidence of clinically
relevant infection).
The patient was treated with nonsteroidal anti-inflammatory drugs, morphine, amitriptyline, and ganciclovir (from
the 14th to the 21st day of admission). H e was discharged
from the hospital after 1 month, to be treated in a rehabilitation center. At follow-up 6 months after the onset of the
symptoms, the patient had no pain or paresthesias. H e had
a numb feeling and diminished pain, temperature, and tactile
sensation in the right lower arm, palm of the hand, thumb,
index finger, and top of the middle finger, and in the left
thumb. There was severe atrophy of all muscles of both lower
arms and hands. The strength of the proximal arm and
shoulder muscles had recovered slightly, bur wrist elevation
and finger extension still was fully impossible. Some grasp
function had returned in both hands. The patient was able
to feed himself and to take a shower but was still unable to
dress himself.
Discussion
Brachial plexus neuritis has been associated with several
viral infections and with vaccinations with viral antigens. Infection with poliomyelitis virus was considered
unlikely in our patient, because this occurs seldomly
after vaccination, sensory symptoms were prominent in
our patient, and headache and meningeal signs were
absent. Besides, poliomyelitis most frequently affects
the lower extremities and seldomly both arms, and a
throat swab was negative for poliomyelitis virus. Parvovirus [4-81 and CMV infection [9, 101 have been
implicated in brachial plexus neuritis before, but a
combined infection of parvovirus B 19 and CMV causing brachial plexus neuritis has not previously been
documented.
The prognosis of plexus neuritis after viral disease is
considered to be excellent and poor outcome is rare.
Brachial plexus neuritis after parvovirus infection has
a variable prognosis and recovery is generally delayed
(Table). A case of bilateral brachial plexus neuritis with
severe atrophy showed “improvement of weakness”
after 6 months [5], while another case with chronic
parvovirus infection causing unilateral brachial plexus
neuritis had complete recovery in the course of several
years [6]. From the even more limited data on CMV
and brachial plexus neuritis, it appeared that outcome
was also variable, described as “resolution of symptoms” [9] and “remaining paralysis” [lo].
Parvovirus infection that persists for several months
is uncommon in an immunologically normal host but
has been documented [14]. In the cases of brachial
plexus neuritis in which this feature has been analyzed
(the patient described by Faden and colleagues [GI, and
our patient), parvovirus could be detected for an unusually long period.
The pathological mechanism of brachial plexus neuritis after parvovirus B19 infection is not known. Human parvovirus is generally not regarded to be neurotropic. However, clinical characteristics of brachial
plexus neuritis following parvovirus infection show
some striking similarities, as can be concluded by reviewing the 6 described cases (including the present
case, see the Table). All cases concerned adults (2338 years old), while the majority of parvovirus infections occur in children. The patients all presented
symptoms of brachial plexus neuritis coincident or immediately after appearance of a rash, which is interest-
Brief Communication: Maas et al: Brachial Neuritis after Parvo and CMV Infection
931
ing because the rash of erythema infectiosum is believed to be immune mediated and generally coincides
with the appearance of viral antibodies. It is likely that
autoantibodies are involved in parvovirus infection
[151, and consequently these antibodies may play a role
in the pathophysiology of plexus neuritis. In 1 case of
brachial plexus neuritis with parvovirus infection [8],
treatment with methylprednisolone resulted in complete resolution of symptoms, further supporting an
immunopathogenic mechanism of the plexus neuritis,
which is also compatible with its occurrence after some
vaccinations.
Our patient showed, in addition to parvovirus B19
infection, evidence of a recent CMV infection, which
explains the hepatitis. The strong serological response
is most typical of a primary infection, although a reactivation of this virus is theoretically also possible. CMV
infections are known to influence immunological responses of the host and to cause impairment of cellular
immunological reactivity [ 161. The severe brachial
plexus neuritis in this patient might have been the result of the interaction of the two viruses. A transient
immunosuppressive effect by CMV could then have
facilitated the persistence of parvovirus infection in our
patient, which in turn led to neurological symptoms by
autoimmunological reactivity. Whether CMV infection
can indeed lead to the development of chronic or protracted parvovirus infections is not yet proven. Both
infections are widespread, affecting the majority of the
human population. Although probably rare, their simultaneous occurrence could enhance the development
of specific manifestations. Careful serological analysis
of brachial plexus neuritis, including the search for parvovirus as well as CMV, may provide evidence for viral
interactions that could be relevant in the pathogenesis
of this rare complication of common viral infections.
References
1. Balfour H H , Schiff GM, Bloom JE. Encephalitis associated
with erythema infectiosum. J Pediatr 1970;77:133-1 36
2. Hall CB, Horner FA. Encephalopathy with erythema infectiosum. Am J Dis Child 1977;131:655-671
3. Yoto Y, Kudoh T , Asanuma H, et al. Transient disturbance of
consciousness and hepatic dysfunction associated with human
parvovirus B19 infection. Lancet 1994;344:624-625
4. Denning DW, Amos A, Rudge P, Cohen BJ. Neuralgic amyotrophy due to parvovirus infection. J Neurol Neurosurg Psychiatry 1987;50:641-642
5. Walsh KJ, Armstrong RD, Turner AM. Brachial plexus neuropathy associated with human parvovirus infection. Br Med
J 1988;296:896 (Letter)
6. Faden H, Gary GW, Anderson LJ. Chronic parvovirus infection in a presumably immunologically healthy woman. Clin
Infect Dis 1392;15:595-597
7. Pellas F, Olivares JP, Zandorti C, Delarque A. Neuralgic
amyotrophy after parvovirus B19 infection. Lancet 1993;342:
503-504
8. Staud R, Davidson RA, Corman LC. Brachial plexitis in a
patient with acute parvovirus B19 infection. Br J Rheumatol
1995;34:480-481
932
9. Duchowny M, Caplan L, Siber G. Cytomegalovirus infection
of the adult nervous system. Ann Neurol 1979;5:458-461
10. Sundkvist T. Cytomegalovirus infection complicated by neuralgic amyotrophy. N Engl J Med 1983;308:461 (Letter)
11. Brown CS, Van Bussel MJAWM, Wassenaar ALM, et al. An
immunofluorescence assay for the detection of parvovirus B 19
IgG and IgM antibodies based on recombinant viral antigen.
J Virol Methods 1990;29:53-62
12. Salimans MMM, Van Bussel MJAWM, Browns CS, Spaan
WJM. Recombinant parvovirus B19 capsids as a new substrate
for detection of 819-specific IgG and IgM antibodies by an
enzyme-linked immunosorbent assay. J Virol Methods 1992;
39~247-258
13. Salimans MMM, Holsappel S, Van de Rijke FM, et al. Rapid
detection of human parvovirus B19 DNA by dot-hybridization
and polymerase chain reaction. J Virol Methods 1989;23:1928
14. Kerr JR, Curran MD, Moore JE, et al. Persistent parvovirus
B19 infection. Lancet 1995;345:118 (Letter)
15. Sasaki T, Takahashi Y, Yoshinaga K, et al. An assocation between human parovirus B19 infection and autoantibody production. J Rheumatol 1989;16:708-709
16. Beersma MFC, Bijlmakers MJE, Ploegh HL. Human cytomegalovirus down-regulates HLA class I expression by reducing the
stability of class I H chains. J Immunol 1994;151:4455-4464
Wide Range in Age
of Onset for Chromosome
1-related Familial
Alzheimer's Disease
Thomas D. Bird,*$$ Ephrat Levy-Lahad,$ll
Parvoneh Poorkaj,t Vikram Sharrna,t Ellen Nemens,$
Amnon Lahad,$S Thomas H. Lampe,?
and Gerard D. Schellenbergtg
Mutations in three different genes on chromosomes 1,
14, and 21 cause autosomal dominant forms of familial
Alzheimer's disease (FAD). Most result in an early-onset
phenotype. However, several kindreds of Volga German
ancestry have the same chromosome 1 gene mutation and
demonstrate a relatively older mean age of onset and include individuals with late age of onset. In these families,
the mean age of onset is 54.9 2 8.4 years (range, 40-75
years), mean age at death is 65.9 f 10-2 years (range,
From the *Department of Neurology and tGeriatric Research Education and Clinical Center, VA Medical Center, and Departments
of $Neurology and $Medicine, University of Washington Medical
School, Seattle, WA.
Received Jan 4, 1996, and in revised form Mar 6, May 8, and Jun
14. Accepted for publication Jun 18, 1996.
Address correspondence to Dr Bird, Neurology (127), VA Puget
Sound Health Care Center, 1660 S. Columbian Way, Seattle, WA
98108.
"Present address: Shaare Zedek Medical Center, Jerusalem, Israel.
SPresent address: Hebrew University, Hadassah Medical School, Jerusalem, Israel.
Copyright 0 1996 by the American Neurological Association
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