1 -  Heller and Landau-Kleffner disorders
Childhood disintegrative disorder is defined in the
DSM-IV manual as a marked regression following at
least 2 years of normal postnatal development within
the first decade of life, with loss of previously acquired
language, social interaction, play, and even motor
skills including bladder and bowel control in some
cases.  According to DSM-IV this is the disorder
referred to by Heller (1908) and DeSanctis (1908) as
dementia infantilis, or the earliest manifestation of
dementia praecox, later renamed schizophrenia [1-3].

  Landau-Kleffner syndrome is one of the
disintegrative disorders in which autistic behaviors are
prominent.  Landau-Kleffner syndrome develops within
the first three years of life; seizures, cessation of
language development with loss of existing language
skills, and autistic behaviors are characteristic of this
disorder (Landau & Kleffner 1957, Rapin 1995) [4-6].  
DaSilva et al. (1997) examined 17 children with
Landau-Kleffner syndrome and found bilateral
hypometabolism in the temporal lobes in 15 using PET
scanning; focal areas of hypermetabolism in the
temporal lobes were found in the other two children
[7].  These abnormalities could perhaps be
investigated as possible results of chronic depression
of energy metabolism within brainstem auditory nuclei
affecting postnatal development of metabolic function
within the temporal lobes.

  Chugani et al. (1996) used PET scanning of children
with infantile spasms to determine which might be
helped by surgery to eliminate the epileptic focus [8].  
Of 110 children, bilateral hypometabolism within the
temporal lobes was revealed in 18.  Uptake of the
deoxy-fluoro-glucose tracer used was at the same
time increased bilaterally in the lenticular nuclei
(putamen and globus pallidus of the basal ganglia),
confirming findings of other investigators.  Chugani et
al. suggested that the seizure activity might be
initiated in the temporal lobes then propagated to the
lenticular nuclei.  Possibly, metabolic rate in the
lenticular nuclei was elevated because responses
were not received from the impaired neural circuits in
the temporal lobes.

  Chugani et al. diagnosed autistic disorder in 10 of
14 children they followed for up to 10 years.  The
majority of autistic children are not in any way as
impaired as the children with seizure disorder and
bilateral involvement of temporal lobes.  But failure to
develop language, lack of social or emotional
reciprocity, motor mannerisms, and preoccupation with
parts of objects led Chugani et al. to this diagnosis,
which they did not encounter in the majority of children
with infantile spasms.  The bilateral involvement of
temporal lobes appeared to be related to
manifestation of autistic disorder.  The bilateral
involvement of temporal lobes and structures of the
basal ganglia seen by Chugani et al. can possibly be
compared to the subcortical damage found by
Malamud (1959) in one pair of siblings with Heller
disintegrative disorder [9]. Malamud's paper is
discussed below.
Full References
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References
  1. Heller T (1908) Ueber
    Dementia infantilis.
  2. Heller T (1930) Ueber
    Dementia infantilis.    
    Translation by Hulse WC
    (1954)
  3. DeSanctis S (1908)
    Dementia pracocissima
    catatonica oder Katatonie des
    frueheren Kindesalters?  
  4. Landau WM, Kleffner FR
    (1957) Syndrome of acquired
    aphasia with convulsive
    disorder in children.
  5. Rapin I (1965) Dementia
    infantilis (Heller’s disease).
  6. Rapin I (1995) Autistic
    regression and disintegrative
    disorder: how important the
    role of epilepsy?
  7. daSilva EA et al. (1997)
    Landau-Kleffner syndrome:
    metabolic abnormalities in
    temporal lobe are a common
    feature.
  8. Chugani HT (1998) A critical
    period of brain development:
    studies of cerebral glucose
    utilization with PET.
  9. Malamud N (1959) Heller's
    disease and childhood
    schizophrenia.
  1. Heller T (1908) Ueber Dementia infantilis.  Zeitschrift für die Erforschung und Behandlung
    des Jugendlichen Schwachsinns 2:17-28
  2. Heller T (1930) Ueber Dementia infantilis.  Zeitschrift für Kinderforschung 37:661-667.  
    Translation by Hulse WC (1954) in Journal of Nervous and Mental Diseases 119:471-477.
  3. DeSanctis S (1908) Dementia pracocissima catatonica oder Katatonie des frueheren
    Kindesalters?  Folia Neuro-Biologica, (Leipzig) 2:9-12.
  4. Landau WM, Kleffner FR (1957) Syndrome of acquired aphasia with convulsive disorder in
    children. Neurology 7:523-30.
  5. Rapin I (1965) Dementia infantilis (Heller’s disease). In Carter CH, ed, Medical Aspects of
    Mental Retardation. Charles C Thomas, Springfield IL, chapter 23, pp760-767.
  6. Rapin I (1995) Autistic regression and disintegrative disorder: how important the role of
    epilepsy?   Seminars in Pediatric Neurology. 2:278-85.
  7. daSilva EA, Chugani DC, Muzik O, Chugani HT (1997) Landau-Kleffner syndrome: metabolic
    abnormalities in temporal lobe are a common feature. Journal of Child Neurology 12:489-495.
  8. Chugani HT (1998) A critical period of brain development: studies of cerebral glucose
    utilization with PET.  Preventative Medicine 27:184-188.
  9. Malamud N (1959) Heller's disease and childhood schizophrenia.  American Journal of
    Psychiatry 116:215-218.