PATIENTS WITH HYDROCEPHALUS PRESENTING
IN THE PSYCHIATRIC CLINIC.
R E P O R T O F T W O C A S E S
WOLFGANG W . M A Y , M . D .
The purpose of this communication is to call attention to some unusual cases of dementia caused by hydrocephalus and potentially amenable to treat-ment. H a k i m and A d a m s6
have described a syndrome of normal pressure hydrocephalus with free communication between the ventricles and basilar cysterns. T h e two cases presented here had normal pressure of the ventricular fluid but they were of the non-communicating type. T h e y were caused by an obstructive lesion a t the level of the aqueduct of Sylvius. Their symp-toms differed in several aspects not only from those reported b y H a k i m and Adams but also from other cases of "occult hydrocephalus" reported in the literature. Some of the clinical aspects shall be discussed.
C A S E 1 — A 60-year-old h o u s e w i f e w a s referred to the Psychiatric Clinic of the University of M i c h i g a n M e d i c a l Center for e v a l u a t i o n of personality changes and blackout spells. O v e r the last four years she h a d developed a feeling of tiredness and lost interest in h e r household. L a t e l y she h a d difficulty in getting around. H e r m e m o r y became impaired especially for recent events. Also, for four y e a r s she h a d occasional generalized convulsions a n d a t times brief episodes d u r i n g w h i c h she w o u l d look pale a n d stare a n d not understand w h a t w a s said. E v a l u a t i o n s over the last three years included electroencephalograms w h i c h w e r e a b n o r m a l and x - r a y studies of the skull suggestive of increased i n t r a c r a n i a l pressure b u t a brain scan w a s normal a n d she w a s considered to h a v e a depressive o r schizophrenic reaction and w a s treated w i t h electroshocks a n d medications. P a s t history r e v e a l e d that a t a g e 15 she h a d a n acute febrile illness a n d stayed in bed for t w o weeks, after w h i c h her mind w a s "not right" for some time and she w a s kept o u t of school for mine months. F a m i l y history indicated that of h e r six siblings, three died of earcinonja of the spine or of the throat. H e r h u s b a n d h a d a long history of drink-ing a n d h e r only son w a s servdrink-ing in the a r m e d forces overseas for the last year.
D u r i n g the i n t e r v i e w the patient w a s mildly depressed. She misinterpreted or misunderstood some questions a n d tended to get lost in details. She admitted to ruminations a n d w a s a w a r e that something w a s not r i g h t w i t h her. A n E E G w a s
F r o m t h e Y p s i l a n t i S t a t e H o s p i t a l ( C h i e f , S e c t i o n o f N e u r o l o g y a n d G e r o n t o l o g y R e s e a r c h ) a n d t h e N e u r o p s y c h i a t r i c I n s t i t u t e o f t h e U n i v e r s i t y o f M i c h i g a n ( C a s e 1, o b s e r v e d w h i l e a u t h o r s e r v e d as a r e s i d e n t in P s y c h i a t r y ) .
P a p e r p r e s e n t e d a t t h e T h i r d P a n a m e r i e a n C o n g r e s s o f N e u r o l o g y ( S ã o P a u l o , B r a z i l , O c t o b e r 10-14, 1 9 7 1 ) .
moderately a b n o r m a l , a b o r t i v e l y p a r o x y s m a l w i t h strong emphasis in the left tem-poral region. Psychometric tests r e v e a l e d a full scale I . Q . of 98. T h e r e w a s evidence of v i s u a l m o t o r difficulties a n d of impaired a b s t r a c t reasoning. O n e x a -mination by the neurologist several minor a b n o r m a l i t i e s w e r e found. C o n v e r g e n c e of g a z e w a s poor. J a w j e r k w a s brisk. F a c e w a s asymmetrical w i t h the left corner of the mouth slightly depressed a n d flattened. T o n g u e protruded to the left and moved slowly. R a p i d a l t e r n a t i n g movements w e r e p e r f o r m e d r a t h e r poorly w i t h both hands. W i t h passive movements there w a s sometimes a n i r r e g u l a r resis-tance in the upper extremities. D e e p tendon reflexes w e r e s o m e w h a t brisk b u t symmetrical in the upper extremities. T h e left p r e p a t e l l a r reflex w a s elicited more easily than the right. Both a n k l e j e r k s w e r e absent. B i l a t e r a l g r a s p reflexes w e r e present.
She w a s hospitalized for further studies. X - r a y s of the skull showed demi-neralization of the dorsum sellae a n d evidence of e n l a r g e d sella turcica. A t o m o g r a m confirmed intra and e x t r a s e l l a r type erosion. A left carotid a n g i o g r a m demonstrated m a r k e d v e n t r i c u l a r dilatation. A p n e u m o e n c e p h a l o g r a m failed to fill the p r o x i m a l portion of the S y l v i a n a q u e d u c t and the ventricles a b o v e . A P a n t o p a q u e ventriculo-g r a m showed obstruction a t the mid-rostral portion of the S y l v i a n a q u e d u c t w i t h resultant dilatation of the lateral a n d third ventricles ( f i g u r e 1 ) . O p e n i n g ven-t r i c u l a r pressure w a s 120 m m of w a ven-t e r . A r i g h ven-t venven-triculo-aven-trial shunven-t w a s per-formed a n d the patient h a d a n uneventful post-operative course.
W h e n seen t w o a n d a h a l f years after h e r operation, the patient felt she w a s back to w h a t she h a d been before the onset of h e r illness. N e u r o l o g i c a l e x a m i n -ation still revealed some minor abnormalities, b u t an E E G w a s within n o r m a l limits.
Disruss'.on — This patient resembles some cases of "occult
hydrocepha-lus" reported by M c H u g h8
. Those becoming symptomatic in adult life pre-sented w i t h : (1) a stiff, unsteady gait appearing insidiously in middle life;
(2) listlessness, apathy, inattentiveness followed by decline of memory, of
slowness; (4) brief a t t a c k s of loss of consciousness often preceded or fol-lowed b y headaches. These were more common than typical epileptic seizures. In none of the cases w a s there mention of papilledema. T h e clinical mani-festations appeared to depend rather on the character of change in the ven-tricular pressure than on the location of the obstruction.
T h e present case differs by the predominance of mental symptoms dur-ing a rather lengthy course without a distinct gait disturbance and without the a t t a c k s of unconsciousness mentioned by M c H u g h . T h e mental picture led to the consideration of a presenile dementing process and the epilepto-genic E E G focus to the suspicion of a brain tumor before further studies established the definite diagnosis.
C A S E 2 — A sixteen-year-old boy w a s admitted to Ypsilanti State Hospital w i t h a o n e - a n d - a - h a l f - y e a r history of w i t h d r a w a l , declining school performance, con-tinuous h e a d s h a k i n g a n d , m o r e recently, occasional a g g r e s s i v e behavior, n o c t u r n a l enuresis, a n d i n a p p r o p r i a t e sexual b e h a v i o r . Before being transferred he h a d been hospitalized e l s e w h e r e on t w o occasions. T h e diagnosis h a d been Schizophrenic Reaction a n d d u r i n g his first admission he seemed to benefit f r o m phenothiazines but, because of some a g g r e s s i v e behavior, he could not be kept in school. H e acted hostile and sometimes talked in a s t r a n g e w a y at home and w h e n he w a s r e -hospitalized he m a d e occasional sexual a d v a n c e s t o w a r d f e m a l e staff, and demonstr a t e d othedemonstr sexual disinhibitions. A n E E G a f e w months befodemonstre admission to Y p s i l -anti State Hospital w a s moderately a b n o r m a l a n d p a r o x y s m a l a n d focal to the r i g h t temporoparietal region. H e w a s placed on D i l a n t i n a n d P h e n o b a r b i t a l .
Past medical history w a s noncontributory. Social history r e v e a l e d conflits at h o m e because of his father's a n d later his step-father's drinking. O n e x a m i n a t i o n he w a s found to be a short, stocky boy, a p p e a r i n g y o u n g e r than his stated a g e . T h e only positive findings on physical e x a m i n a t i o n w e r e a slightly ataxic g a i t a n d continuous side-to-side s h a k i n g of his h e a d . D u r i n g psychiatric e x a m i n a t i o n he w a s cooperative. Speech w a s spontaneous a n d coherent and his r a n g e of affect f a i r l y broad. H e could smile a p p r o p r i a t e l y a n d m a k e jokes. T h o u g h t content w a s not overtly disturbed. Response to p r o v e r b s w a s concrete. Psychiatric diagnosis w a s acute schizophrenic episode. R o u t i n e l a b o r a t o r y studies w e r e n o r m a l except for white blood count of 21,500 cells per cubic millimeter w i t h neutrophilia.
H e w a s referred to the medical clinic w h e r e he w a s found to h a v e bilateral lymphadenopathy. I n g u i n a l node biopsy r e v e a l e d Hodgkin's disease. H e w a s trans-ferred to the University of M i c h i g a n M e d i c a l Center for e v a l u a t i o n a n d treatment. B e c a u s e of his h e a d s h a k i n g he w a s e v a l u a t e d by a neurologist. H e talked little and reluctantly. H e could arrest his h e a d s h a k i n g briefly, at request. N o nevv findings w e r e elicited. A n E E G w a s a g a i n focally a b n o r m a l b u t there w a s also s l o w i n g of the b a c k g r o u n d . C e r e b r o s p i n a l fluid e x a m i n a t i o n r e v e a l e d a protein of 63 m g m . % a n d a colloidal gold c u r v e of 5553210000; glucose a n d cell count w e r e n o r m a l . Skull films w e r e n o r m a l . A r i g h t carotid a r t e r i o g r a m showed evidence of dilated l a t e r a l ventricles w i t h o u t midline shift. A n a i r v e n t r i c u l o g r a m s h o w e d symmetrically dilated l a t e r a l ventricles, third ventricle a n d p r o x i m a l portion of the a q u e d u c t w h i c h g r a d u a l l y tapered off. T h e r e w a s no a i r in the fourth ventricle. P n e u m o e n c e p h a l o g r a m showed a i r in the fourth ventricle w i t h o u t gross deformity or displacement. T h e v e n t r i c u l a r pressure w a s between 50 a n d 70 millimeters of w a t e r . I t w a s decided not to p e r f o r m a shunting operation.
134 ARQ. NEURO-PSIQUIAT. (SAO PAULO J VOL. 30, N.o 2, JUNHO, 1972
pride in his products. A V i n e l a n d social m a t u r i t y test r e v e a l e d a level of 8 y e a r s , 6 months, for a n a c t u a l a g e of 16 years a n d 7 1/2 months. H e w a s released to r e t u r n to school b u t he soon a g a i n got into fights. His h e a d s h a k i n g b e c a m e w o r s e and he w a s rehospitalized. T h e last nine months of his life he deteriorated m e n t -ally a n d physic-ally. H e w a n d e r e d a r o u n d and seemed confused. H e w o u l d say a w o r d or t w o if h e needed something b a d l y enough. E v e n t u a l l y he b e c a m e l e t h a r g i c and w e a k a n d he died three years after onset of his first symptoms.
P o s t - m o r t e m e x a m i n a t i o n r e v e a l e d massive e n l a r g e m e n t of mesenteric, pelvic and retroperitoneal lymph nodes a n d implants of Hodgkin's s a r c o m a in v a r i o u s o r g a n s including liver, left kidney a n d left p l e u r a b u t none in the b r a i n or meninges. T h e b r a i n w e i g h e d 1,350 g r a m s . A t the base, both temporal lobes w e r e b u l b o u s and the cortex w a s m a r k e d l y thinned, especially o v e r the t e m p o r a l poles due to anterior extension a n d dilatation of the temporal horns of the l a t e r a l ventricles ( f i g u r e 2 ) . Both l a t e r a l ventricles a n d third ventricle w e r e g r e a t l y dilated w i t h m a r k e d thinning of the floor of the third ventricle. A thin m e m b r a n o u s septum completely blocked the c a u d a l end of the a q u e d u c t ; the a q u e d u c t p r o x i m a l to t h e m e m b r a n e w a s g r e a t l y dilated ( f i g u r e 3 ) . T h e septum pellucidum w a s fenestrated and the fornices thinned a n d flattened a g a i n s t the t h a l a m u s and roof of the third ventricle. T h e septal a r e a a n d d i a g o n a l b a n d of B r o c a w e r e p a r t l y cystic a n d gliotic. T h e choroid p l e x u s w a s atrophic. Microscopically, the m e m b r a n e blocking the a q u e d u c t w a s composed of astrocytes and g l i a l tissue devoid of ependyma on its surfaces. T h e r e w a s b i l a t e r a l atrophy of the fimbrias of the hippocampi; the for-nices w e r e devoid of myelin a n d showed gliosis.
Discussion — This second patient seems to fall between some of the
cases reported by M c H u g h
8and the juvenile type of hydrocephalus as
des-cribed by P e n n y b a c k e r
1 0but disturbance of gait, clumsiness or large head
were not observed. The dementia was more pronounced than in the cases
described by M c H u g h and it was progressive. In addition there were several
striking features not usually observed in cases of occult hydrocephalus and
which for their very unusualness led to the diagosis of a functional psychosis.
T h e y were: (1) occasional aggressive and sexually disinhibited behavior;
(2) incomplete mutism; ( 3 ) to and fro shaking of the head.
T h e aggressive and sexually disinhibited behavior might be related to
disturbance of the postero-medial nuclei of the hypothalamus or their
con-nections
5. Such hypothalamic symptoms have been noticed in tumors
adjacent to the aqueduct and causing increased cerebrospinal fluid pressure
in the third v e n t r i c l e
7.
Mutism due to an organic lesion has usually been described in
associa-tion with akinesis
2»
4. Cairns and associates
3, however, have observed an
incomplete and reversible mutism after ventriculogram in chronic
hydroce-phalus due to stricture of the aqueduct and other lesions of the midbrain.
The nearly continuous headshaking represented the most intriguing
symptom. Recently Benton and associates
1, and subsequently, N e l l h a u s
9S U M M A R Y
T w o p a t i e n t s w i t h m a j o r m e n t a l s y m p t o m a t o l o g y a n d f e w n e u r o l o g i c a l
s i g n s w e r e p r e s e n t e d w h o o n f u r t h e r s t u d i e s w e r e r e v e a l e d t o h a v e u n s u s
-p e c t e d h y d r o c e -p h a l u s . T h i s w a s d u e t o a n o b s t r u c t i v e l e s i o n a t t h e l e v e l
of t h e a q u e d u c t p r e s e n t s i n c e b i r t h o r f o r m a n y y e a r s . B o t h p r e s e n t e d w i t h
a r a t h e r u n u s u a l c l i n i c a l p i c t u r e s o t h a t t h e y w e r e f i r s t c o n s i d e r e d t o s u f f e r
f r o m a p s y c h i a t r i c i l l n e s s . R a d i o g r a p h i c c o n t r a s t s t u d i e s e s t a b l i s h e d t h e
d i a g n o s i s i n b o t h c a s e s a n d l e d t o s u c c e s s f u l t r e a t m e n t i n o n e o f t h e m .
T h e f i r s t p a t i e n t m a d e a n u n e v e n t f u l r e c o v e r y a f t e r a s h u n t i n g o p e r a t i o n .
T h e o t h e r h a d n o s u c h o p e r a t i o n a n d d i e d o f a n u n r e l a t e d d i s e a s e s o m e t i m e
l a t e r .
A c o n s t e l l a t i o n o f d e c l i n i n g m e n t a l f u n c t i o n s w i t h o r w i t h o u t b e h a v i o r a l
d i s t u r b a n c e a n d f e w o r m i n o r n e u r o l o g i c s i g n s a n d a n a b n o r m a l e l e c t r o
e n c e p h a l o g r a m s h o u l d i n c l u d e in t h e d i f f e r e n t i a l d i a g n o s i s " o c c u l t " h y d r o
-c e p h a l u s w h i -c h is a p o t e n t i a l l y -c o r r e -c t a b l e -c o n d i t i o n . T h e p s y -c h i a t r i s t m i g h t
b e t h e f i r s t o n e t o s e e t h i s t y p e o f p a t i e n t a n d t o i n i t i a t e t h e a p p r o p r i a t e
e v a l u a t i o n .
R E S U M O
Pacientes com hidrocéfalo e predominyncia de distúrbios psíquicos.
Relato de dois casos
E m d o i s p a c i e n t e s c o m s i n t o m a t o l o g i a m e n t a l p r e d o m i n a n t e e p o u c o s
s i n a i s n e u r o l ó g i c o s , e x a m e s c o m p l e m e n t a r e s m o s t r a r a m t r a t a r s e d e h i d r o
-c é f a l o p o r l e s ã o o b s t r u t i v a a o n í v e l d o a q u e d u t o d e S y l v i u s , e x i s t e n t e d e s d e
o n a s c i m e n t o o u p o r m u i t o s a n o s . A m b o s f o r a m i n t e r n a d o s p o r a p r e s e n t a r
u m q u a d r o c l í n i c o n ã o u s u a l q u e a p r i n c í p i o l e v o u a p e n s a r e m d o e n ç a p s i
-q u i á t r i c a . E x a m e s r a d i o l ó g i c o s c o n t r a s t a d o s e s t a b e l e c e r a m o d i a g n ó s t i c o e m
a m b o s o s c a s o s e p e r m i t i r a m o t r a t a m e n t o e f i c a z d e u m deles. O p r i m e i r o
p a c i e n t e t e v e g r a n d e m e l h o r a a p ó s i n t e r v e n ç ã o c i r ú r g i c a n a q u a l f o i f e i t a
d e r i v a ç ã o v e n t r í c u l o - a t r i a l . O o u t r o p a c i e n t e n ã o p ô d e s e r o p e r a d o e f a l e c e u
d e a f e c ç ã o i n t e r c o r r e n t e . D i s c u t i n d o o s c a s o s , o a u t o r p r o c u r a c h a m a r a a t e n
-ç ã o p a r a a i m p o r t â n c i a d a a v a l i a -ç ã o d i a g n ó s t i c a d a s d e m ê n c i a s o r g â n i c a s
v i s a n d o a o d e s p i s t a m e n t o d e u m a c o n d i ç ã o p o t e n c i a l m e n t e c o r r i g í v e l .
R E F E R Ê N C I A S
1 . B E N T O N , J. W . ; N E L L H A U S , G . ; H U T T E N L O C H E R , P . R . ; O J E M A N , R . G. & D O D G E , P . R . — T h e b o b b l e - h e a d d o l l s y n d r o m e . R e p o r t o f a u n i q u e t r u n c a l t r e m o r a s s o c i a t e d w i t h t h i r d v e n t r i c l e c y s t a n d h y d r o c e p h a l u s in c h i l d r e n . N e u r o l o g y ( M i n n e a p o l i s ) 16:725, 1966.
2 . C A I R N S , H . — D i s t u r b a n c e s o f consciousness w i t h l e s i o n s o f t h e b r a i n s t e m a n d d i e n c e p h a l o n . B r a i n 75:109, 1952.
4 . C R A V I O T O , H . ; S I L B E R M A N , J. & F E I G I N , I . — A c l i n i c a l a n d p a t h o l o g i c s t u d y o f a k i n e t i c m u t i s m . N e u r o l o g y ( M i n n e a p o l i s ) 10:10, 1960.
5 . G R I N K E R , R . R . & S A H S , A . L . — N e u r o l o g y . 6th Ed.. C h a r l e s C. T h o m a s , S p r i n g f i e l d ( I l l i n o i s ) , 1966, p p . 498-521, 375-378.
6 . H A K I M , S. & A D A M S , R . D . — T h e s p e c i a l c l i n i c a l p r o o l e m o f s y m p t o m a t i c h y d r o c e p h a l u s w i t h n o r m a l c e r e b r o s p i n a l f l u i d p r e s s u r e . J. N e u r o l . Sci. 2, 307, 1965.
7 . K U N T Z , A . — T h e A u t o n o m i c N e r v o u s S y s t e m . 4th ed.. L e a & F e b i g e r , P h i l a -d e l p h i a , 1953, p p . 89-99.
8 . M c H U G H , P . R . — O c c u l t h y d r o c e p h a l u s . Q u a r t . J. M e d . 33, 297, 1964. 9 . N E L L H A U S , G . — T h e b o b b l e h e a d d o l l s y n d r o m e : a " t i c " w i t h a n e u r o
-p a t h o l o g y basis. P e d i a t r i c s 40:250, 1967.
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