31.8c Tourette’s Disorder
1201
Table 31.8c-2
Differential Diagnosis of Tic Disorders
Disease or
Syndrome
Age at Onset
Associated Features
Course
Predominant Type of
Movement
Hallervorden-Spatz Childhood-
adolescence
May be associated with optic
atrophy, club feet, retinitis
pigmentosa, dysarthria,
dementia, ataxia, emotional
lability, spasticity, autosomal
recessive inheritance
Progressive to
death in 5 to
20 years
Choreic, athetoid,
myoclonic
Dystonia
musculorum
deformans
Childhood-
adolescence
Autosomal recessive
inheritance commonly,
primarily among Ashkenazi
Jews; a more benign
autosomal dominant form
also occurs
Variable course,
often progressive
but with rare
remissions
Dystonia
Sydenham’s chorea Childhood, usually
5 to 15 yrs
More common in females,
usually associated with
rheumatic fever (carditis
elevated ASLO titers)
Usually self-limited Choreiform
Huntington’s disease Usually 30 to 50
yrs, but childhood
forms are known
Autosomal dominant
inheritance, dementia,
caudate atrophy on CT scan
Progressive to death
in 10 to 15 years
after onset
Choreiform
Wilson’s disease
(hepatolenticular
degeneration)
Usually 10 to 25 yrs Kayser-Fleischer rings, liver
dysfunction, inborn error
of copper metabolism;
autosomal recessive
inheritance
Progressive to
death without
chelating therapy
Wing-beating tremor,
dystonia
Hyperreflexias
(including latah,
myriachit, jumper
disease of Maine)
Generally in
childhood
(dominant
inheritance)
Familial; may have generalized
rigidity and autosomal
inheritance
Nonprogressive
Excessive startle response;
may have echolalia,
coprolalia, and forced
obedience
Myoclonic disorders Any age
Numerous causes, some
familial, usually no
vocalizations
Variable,
depending on
cause
Myoclonus
Myoclonic dystonia 5 to 47 yrs
Nonfamilial, no vocalizations
Nonprogressive
Torsion dystonia with
myoclonic jerks
Paroxysmal
myoclonic
dystonia with
vocalization
Childhood
Attention, hyperactive,
and learning disorders;
movements interfere with
ongoing activity
Nonprogressive
Bursts of regular,
repetitive clonic (less
tonic) movements and
vocalizations
Tardive Tourette’s
disorder
syndromes
Variable (after
antipsychotic
medication use)
Reported to be precipitated by
discontinuation or reduction
of medication
May terminate
after increase
or decrease of
dosage
Orofacial dyskinesias,
choreoathetosis, tics,
vocalization
Neuroacanthocytosis Third or fourth
decade
Acanthocytosis, muscle
wasting, parkinsonism,
autosomal recessive
inheritance
Variable
Orofacial dyskinesia
and limb chorea, tics,
vocalization
Encephalitis
lethargica
Variable
Shouting fits, bizarre behavior,
psychosis, Parkinson’s
disease
Variable
Simple and complex motor
and vocal tics, coprolalia,
echolalia, echopraxia,
palilalia
Gasoline inhalation Variable
Abnormal EEG; symmetrical
theta and theta bursts
frontocentrally
Variable
Simple motor and vocal tics
Postangiographic
complications
Variable
Emotional lability, amnestic
syndrome
Variable
Simple motor and complex
vocal tics, palilalia
Postinfectious
Variable
EEG: occasional asymmetrical
theta bursts before
movements, elevated ASLO
titers
Variable
Simple motor and vocal tics,
echopraxia
Posttraumatic
Variable
Asymmetrical tic distribution Variable
Complex motor tics
Carbon monoxide
poisoning
Variable
Inappropriate sexual behavior
Variable
Simple and complex motor
and vocal tics, coprolalia,
echolalia, palilalia
(
continued
)
