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Conjugal amyotrophic lateral sclerosis.

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does not, however, say if extraocular function was totally or
partially absent. Two of Jefferson’s [l) patients (cases 4 and
9) had partial extraocular muscle paralysis and apparently
normal pupils.
Department of Ophthalmology
University of Floridz College of Medicine
J . Hillis Miller Health Center
Gainesvzlle. FL 32610
1. Jefferson G: On the saccular aneurysms of the internal carotid
artery in the cavernous sinus. Br J Surg 26:267-302, 1938
2. Meadows SP: Intracavernous aneurysms of the internal carotid
artery. Arch Ophthalmol 651566-574, 1959
3. Trobe JD, Glaser JS, Post JD: Meningiomas and aneurysms of the
cavernous sinus. Arch Ophthalmol 96:457-467, 1978
Conjugal Amyotrophic
Lateral Sclerosis
E. Paolino, MD, E. Granieri, MD,
M. R. Tola, MD, and G . Rosati, M D
Recently, Chad and co-workers [3] described the first known
example of amyotrophic lateral sclerosis (ALS) occurring in
both a husband and wife. We describe a similar occurrence in
a Sardinian couple.
In 1976, at 62 years of age, a man noted diffuse muscle
twitching, wasting, and weakness in both hands. These symptoms worsened, and some months later he was admitted. The
medical record indicated a normal medical history. The patient was alert and fully oriented, and cranial nerves were
intact. Slight atrophy and weakness of the intrinsic hand muscles were present bilaterally. Muscle fasciculations were
noted in the upper trunk. Generalized hyperreflexia and a
bilateral Hoffmann’s sign were present, plantar responses
were flexor, and abdominal reflexes were absent bilaterally.
Sensation was normal, as were the rest of the findings from
neurological examination and results of routine laboratory
tests. A roentgenogram of the cervical spine showed moderate spondylosis; a myelogram showed no abnormalities.
Electromyography (EMG) performed in the shoulder,
forearm, and hand muscles revealed a denervation pattern
with fasciculation potentials, positive sharp waves, and largeamplitude motor unit potentials. Sensory nerve conduction
velocities were normal, while motor nerve conduction velocities showed a slight slowing. In the ensuing months,
weakness and wasting of the hands progressed and voice impairment and difficulty in swallowing appeared. Repeat
neurological examination disclosed a worsening of the previously mentioned signs and a slight weakness of the lower
facial muscles; the tongue was weak and showed atrophy and
fasciculations. Paralysis of the palate and dysarthria were
present. EMG showed a pattern consistent with anterior horn
cell damage. The patient was considered to have ALS. He
deteriorated and died of respiratory insufficiency after 3
years. No postmortem examination was performed.
In 1979 the wife, at 61 years of age, noticed progressive
weakness in both lower limbs, with difficulty in walking. In
the following months, weakness progressed to involve the
upper limbs, and dysphonia and dysphagia appeared. On examination, the patient was alert and oriented. Speech was
slow and dysphonic with a nasal quality. Facial weakness was
noted, and she could not smile well. There was bilateral mild
weakness of the masticatory and pharyngeal muscles with
moderate dysphagia for both liquids and solids. The tongue
was weak and atrophic. The sternocleidomastoid and trapezius muscles were atrophic and weak bilaterally, as were
the intrinsic hand and leg muscles. Coarse fasciculations were
noted in the tongue and in weakened muscles. Deep tendon
reflexes were brisk. Abdominal reflexes were present and no
pathological pyramidal sign was observed. Sensation was normal. The remainder of the neurological examination, as well
as routine laboratory tests and skull and cervical spine roentgenograms, showed normal results. EMG showed a denervation pattern. The motor unit potentials were decreased in
number and increased in amplitude; widespread fibrillations
and fasciculations were also present. The diagnosis of ALS
was made. Subsequently, the disorder has progressed, and
the patient now has partial paralysis of the intercostal muscles
with mild difficulty in breathing.
Although we did not examine the husband, the detailed
clinical record leaves little doubt as to the diagnosis of ALS.
Clinical and electrophysiological findings in the wife were
typical. The low prevalence of ALS and the occurrence in
two people closely sharing the same environment for several
years suggest that both husband and wife could have been
exposed to a common causative environmental agent. The
husband was a sheep breeder; his wife occasionally helped
him in his work. A significantly higher incidence of ALS
among agricultural workers has been reported { 1,6]. Physical
work load El, 21, exposure to transmissible slow virus diseases [ 5 ] , exposure to toxic farm substances [l], and increased milk ingestion [4,61 have all been speculated upon,
but at present no definite clues for specific environmental
agents have emerged.
Neurological Clinic
University of Fevraru
Corso dellu Giovecca, 203
44100 Ferraru, Itub
1. Breland AE, Currier R D Multiple sclerosis and amyotrophic lateral sclerosis in Mississippi. Neurology (Minneap) 17:lOll1016, 1967
2. Cendrowski W, Wender M, Owsianowski M: Analyse epidemiologique de la sclerose laterale amyotrophique sur ie territoire
de la Grande-Pologne. Acta Neurol Scand 46:609-617, 1970
3. Chad D, Mitsumoto H, Adelman LS, et al: Conjugal motor
neuron disease. Neurology (NY) 32:306-307, 1982
4. Felmus MT, Patten BM, Swanke L Antecedent events in amyotrophic lateral sclerosis. Neurology (Minneap) 26: 167- 172, 1976
5. Hirano A: Pathology of ALS. In Gajdusek DC, Gibbs CJ (eds):
Slow, Latent and Temperate Virus Infections. Washington, DC,
National Institutes of Health, 1965, pp 23-27
6. Rosati G, Pinna L, Granieri E, et al: Studies o n epidemiological,
clinical and aetiological aspects of ALS disease in Sardinia, Southern Italy. Acta Neurol Scand 55:231-244, 1977
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conjugal, lateral, sclerosis, amyotrophic
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