ltal. J. Neurol. Sci. 13:611-616, 1992

Chronic spastic atrophic hemiparesis
with benign course
Casmiro M.*, Sintini M.**, Martinelli P.*, Leonardi M.*, D'Alessandro R.*
* Istituto di Clinica Neurologica, Universitd di Bologna
** Dipartimento di Neuroradiologia, Ospedale di Cesena

We present the clinical and neuroimaging data of 4 patients (mean age: 62
years, range 54-67; mean follow-up: 19.5 years, range: 26 months-29 years)
with chronic spastic atrophic hemiparesis (CSAH). Neuroimaging findings
were non specific, CT scan and M R I showing white matter lesions located
within the centrum semiovale in two cases, parieto-occipital lobe and pons in
one case each. This study underlines that patients with CSAH may have a good
long-term prognosis. MRI studies may demonstrate associated lesions within
the white matter or brainstem and are advisable in all subjects with CSAH,
but further studies are necessary to clarify the nature of the observed lesions.

Key Words: Mills syndrome - - chronic spastic atrophic hemiparesis (CSAH) - - MRI - - CT scan.

Introduction

Report of cases

Chronic spastic atrophic hemiparesis (CSAH) may
result from several diseases such as a slowly evolving focal lesion within the cerebral hemisphere
involving parietal lobe (explaining so-called "parietal lobe atrophy"), unilateral amyotrophic lateral
sclerosis (ALS), idiopathic degeneration of pyramidal tracts (so-called Mills syndrome), multiple
sclerosis (especially in its early stages) and diseases of the cervical spinal cord. So many different etiologies imply very different therapeutic
strategies and prognosis (both quoad vitam and
quoad valetudinem), and this makes an accurate
diagnosis imperative.
In some cases, however, definite diagnosis is
prevented by a lack of pathological confirmation
and a descriptive formulation (CSAH) must be
used in these instances. We report 4 cases of
CSAH with a long follow-up and benign course
that, to our knowledge, have not been previously
described.

Case 1, - This man was first hospitalized in 1959,
when aged 38; he complained of L sided weakness since age 25, and reported a slow onset with
arm and leg involvement from the beginning, but
a predominant impairment of the leg. The course
was very slowly progressive, with minor functional impairment, N e u r o l o g i c a l e x a m i n a t i o n
showed L facial palsy of central type, mild L
spastic hemiparesis with extensor plantar response and absence of abdominal reflexes on the
L; muscle wasting was present, proximally predominant in the arm and distally in the leg, but
neither fasciculations or sensation defects. CSP
examination, EEG, skull x-ray and blood tests
were normal. Widening of subarachnoid spaces
over the L convexity was present at air pneumoencephalography.
He was re-examined 29 years later, in June 1988.
Resting tremor and rigidity in the L ann had appeared since age 62, but he complained only of

Received 24 January 1992 - Accepted 30 April 1992

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Fig. 1. Case 1. CT scan (A and B) and MR1 (T2-weighted image) (C and D) showing two lacunar
lesions at the R centrum semiovale and head of caudate.
moderate impairment of gait and object handling
with his L hand. Weakness and atrophy appeared
unchanged, and cogwheel phenomenon was present in the L arm. EMG examination was normal.
Cerebral CT scan and MRI (Fig. 1) showed two
R sided lesions in the centrum semiovale and head
of caudate. Muscle CT scan showed diffuse atrophy involving all the muscle groups in the L calf
and thigh.
Case 2. - A man presented in 1961, when aged
40, because of progressive L sided weakness dating back three years. He did not seek medical care
612

before coming to our Institute. His leg was first
involved. Clinical examination showed mild to
moderate L sided spastic hemiparesis, hyperreflexia with absent abdominal and cremasteric reflexes on the L and a proximally predominant,
non-segmental muscle atrophy on the same side.
A Babinski sign on the L and a questionable response on the R were found on plantar stimulation, but no sensation defects..CSF, EEG and
blood examination were normal.
At follow-up, in July 1987, the patient was still
independent. L hemiparesis appeared unchanged
while atrophy was slightly increased. EMG

Casmiro M.: Chronic spastic' atrophic hemiparesis

Fig. 2. Case 2. CT scan (A) showing a small lacunar lesion in tile anterior arm oj tile L internal
capsule (arrow). MRI (B) (T2-weighted image) showing multiple small periventricular hyperintense foci and an area of high-signal density in the R centrum semiovale, not detected by CT scan.

showed no alterations. Cerebral CT scan (Fig. 2a)
revealed a hypodense lesion in the anterior limb
of the L internal capsule; MRI showed multiple
hyperintense foci in periventricular areas and a
greater lesion in the R centrum semiovale (Fig.
2b). Muscle CT scan was not performed.
Case 3. - A 40-year-old man was hospitalized in
1967 because of a walking disorder which had
started two years earlier when he complained of

Fig. 3. Case 3. Transverse CT scan. R parietooccipital hypodense lesion (cortical-subcortical).

slowly progressive weakness in the L leg. Neurological examination revealed mild hyposthenia
and spasticity in the L leg, increased tendon reflexes, extensor plantar response and absence of
abdominal reflexes on the L. Mild L sided distally predominant muscle wasting was observed, together with lowered skin temperature. Fasciculations and sensation defects were otherwise absent. CSF, EEG, skull X-ray and routine blood
examination were normal. Pneumoencephalography revealed mild internal hydrocephalus at the R
hemisphere.
We evaluated the patient again in February 1988,
when he was 61: mild distal hyposthenia in the L
arm was also present and muscle wasting in the
L leg was slightly increased. Spontaneous movements on the L were weaker but nowhere abolished, the patient still being independent; hypothermia persisted. EMG examination was normal.
CT scan (Fig. 3) showed R ventricular enlargement prominent in the trigone and the occipital
horn, and a hypodense area at the R parieto-occipital border zone. CT scan of the L calf and
thigh revealed atrophy of all muscle groups. The
patient refused MRI.
Case 4. - This 54 year old woman was referred
for neurological examination in September 1989
while hospitalized for gallstones. Clinical history
revealed a 10-year mild weakness with difficulty
in climbing stairs and transient pins and needleslike sensation in the R leg. When aged 53 she also complained of a slight difficulty in handling
small objects with the R hand. Neurological ex-

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Fig. 4. Case 4. MRI showing a L sided lesion within the pons, involving the pyramidal tract zone (A:
axial T2-weighted image; B: sagittal Tl-weighted image).
amination revealed R sided spastic hemiparesis
with facial palsy of the central type, hyperreflexia
and extensor plantar response. Abdominal reflexes were absent on both sides. Mild hypothermia,
with a sock-like distribution, was found at the R
foot and a slight distally predominant muscle
atrophy on the R. Normal findings were obtained
at EMG, EEG, skull and chest X-ray, routine
blood examination, visual and brainstem evoked
responses.
CT scan revealed only slight enlargement of the
subarachnoid spaces in the posterior fossa. MRI
(Fig. 4) showed a L sided hyperintense lesion
within the basis pontis, at the pontomesencephalic junction. The lesion slightly extended beyond
the midline.
At follow-up, 26 months later, both clinical and
MRI findings were unchanged.

Discussion

We have reported 4 patients with a syndrome of
chronic spastic atrophic hemiparesis (CSAH),
whose clinical features, listed in the table, may be
summarized as follows: 1) a strictly unilateral and
"pure" pyramidal disorder, without signs of lower
motor neuron involvement; 2) a slow onset and a
slow progression without major functional impairment and/or death; 3) associated muscle atrophy on the affected side; 4) an ascending course
(from lower to upper limb). Additional features
may be hypothermia of paretic limbs and facial
nerve palsy.
CSAH may be viewed as a syndrome with different underlying etiologies such as ALS, multiple
sclerosis, expanding lesions within the cerebral
hemispheres and rare cases of Mills syndrome.

TABLE I. Clinical features of CSAH patients.
Sex
Age at Follow Slow Spastic Exten- Absent Ascend- Muscle Facial HyAge (ys) onset up (ys) progr- hemipa- sor abdoming
atrophy palsy pothession resis plantar
inal
course
ermia
resreflex
ponse
Case 1
Case 2
Case 3
Case 4

+ Sign present
- Sign absent.

614

M/67
M/66
M/61
F/54

25
37
38
44

29
26
21
2.2

+
+
+
+

+
ยง
+
+

+
+
+
+

+
+
+
+

+
+
+

+
+
+
+

+
+

+
+

Casmiro M.: Chronic spastic atrophic hemiparesis

The neurologist confronted with CSAH has therefore to address differential diagnosis among theseheterogeneous diseases with very different
prognosis and treatment.
In our cases expanding cerebral lesions were ruled
out by neuroimaging studies and clinical followup. A diagnosis of ALS was unlikely because extensive studies failed to demonstrate any clinical
a n d / o r EMG sign of lower motor neuron involvement; furthermore, both autonomy in daily
living and mean survival from onset were much
greater than expected for ALS. Multiple sclerosis
was unlikely in our patients because of the absence of a relapsing-remitting course, absence of
multifocality and temporal disc pallor at fundus
examination, normal findings at cerebrospinal fluid
examination (patients 1 through 3) and visual and
brainstem evoked potentials (patient 4).
We also considered a diagnosis of primary degeneration of the pyramidal tracts or Mills Syndrome, the diagnostic criteria of which include: 1)
a slowly progressive spastic hemiparesis with ascending course (distal to proximal, and lower to
upper limb), associated on the affected side with:
2) vasomotor disturbances (hypothermia a n d / o r
cyanosis), 3) muscle atrophy without a segmental
distribution, no fasciculations or signs of denervation on electrical examination, 4) absence of
objective sensory loss, cerebellar, extrapyramidal
signs and sphincter disturbances [15]. Our cases
fulfil these criteria.
Mills Syndrome is referred to as a "spinal form"
of unilateral ascending paralysis on the basis of
only post mortem examination showing degeneration of direct and crossed pyramidal tracts in the
spinal cord, extending into the pons [10, 13].
However, the hypothesis that Mills Syndrome may
be also due to hemispheric lesions was suggested
by Cossa et al [3, 4] on the basis of several findings pointing to intracranial involvement in some
cases, such as facial palsy and wasting of the tongue, and cortical atrophy involving mainly the
motor cortex at air pneumoencephalography and
biopsy, Because of the absence of further pathological and neuroimaging data the question of the
lesion site (spinal, cortical or both) is unsettled at
present.
Mills Syndrome has been distinguished into primary (or idiopathic) forms and secondary forms
[15]. The latter may result from multiple sclerosis
[12], ALS [2, 8, 9, 14], diseases of cervical spinal
cord due to spondylosis [ 1], anomalies of cervical

spine [6] and atlanto-occipital joint [7]. Given the
hypothesis that idiopathic Mills Syndrome is the
result of primary degeneration of pyramidal tracts,
the question of its relationship to ALS remains
unanswered. Indeed, idiopathic Mills Syndrome is
sometimes simply considered a variant of ALS
("hemiparetic or Mills variant of ALS") [2, 8, 9,
14, 17]. In a recent report [5] it has been described in association with multiple lacunar infarcts of the periventricular and subcortical white
matter. So, a multiple etiology for idiopathic forms
of Mills Syndrome may be hypothesized.
In our cases the nature of the observed white
matter lesions (WMLs) remains uncertain, since
they are not specific and pathological confirmation is not available; moreover, some of them may
be chance findings in aged patients. Even the
causal relationship between W M L s and CSAH
cannot be demonstrated beyond doubt, due to the
long time interval between onset and neuroimaging studies. Such a relationship, however, is
strongly suggested by the concordance between
side of lesions and clinical findings.
The clinical course and the young age at onset in
our cases makes a vascular e t i o l o g y unlikely
(though this statement may well be untrue for individual cases), and the long slowly progressive
course without other signs of central nervous system involvement makes a diagnosis of multiple
sclerosis improbable.
Explanation of muscle atrophy in CSAH is based
on damage to the parietal lobe or fibers descending from it, since atrophy is a well-known feature
of such lesions, even in the absence of sensory
defects [16]. Vegetative disturbances such as hypothermia and cyanosis may result from lesions
within the supranuclear regulation apparatus of the
autonomic nervous system descending from the
frontal lobe and limbic system together with corticospinal fibers.
In conclusion, our cases of CSAH lack pathological confirmation so that diagnosis remains undefined; they could be cases of idiopathic Mills
Syndrome with associated cerebral lesions, but this
hypothesis remains unproven. Our study shows
that MRI is useful in making a differential diagnosis between CSAH and ALS or allied disorders
which may mimic CSAH. At all events, patients
with WMLs and CSAH may have a good prognosis and are to be kept distinct from ALS patients. Further studies are necessary in order to
clarify the nature of the WMLs observed.

Acknowledgements:We thank R. Daidone, MD and V. Poli, MD for their help in performing this study, and A. Pollini
for expert secretarial assistance.

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The Italian Journal of Neurological Sciences

Sommario

Vengono presentati i dati clinici e neuroradiologici relativi a quattro pazienti (eta media 152 anni, range 54-67; follow-up medio 19,5 anni, range 26 mesi-29 anni) affetti da emiparesi cronica spastica ed
atrofica (CSAH). La TAC e la R M N cerebrale hanno evidenziato la presenza di lesioni localizzate nel
centro semiovale in due casi, in regione parieto-occipitale e a livello del ponte in un caso ciascuno.
Questo studio sottolinea il fatto che i pazienti con C S A H possono presentare una prognosi favorevole
a lungo termine. Lo studio con R M N cerebrale pub evidenziare lesioni a livello della sostanza bianca
emi~ferica o del tronco cerebrale ed b pertanto indicato in tutti i soggetti con CSAH. Tuttavia, ulteriori studi sono necessari al fine di chiarire la natura delle lesioni osservate.

A d d r e s s reprint r e q u e s t s to:
Dr. R o b e r t o D ' A l e s s a n d r o
Clinica Neurologica
V i a U. F o s c o l o , 7 - 40123 B o l o g n a

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