Page 8 C ER E B R A L P A LSY - PART I AETIOLOGY (Aetiology and methods found useful in its treatment Part II) P H Y S I O T H E R A P Y JUNE, 1971 compiled by Physiotherapists o f the Pretoria School for Cerebral P alsy Cerebral palsy is a condition caused by a brain lesion to the immature brain. Signs and sym ptom s depend on the area o f the brain injured and the extent o f the injury. Cerebral palsy usually involves physical abnorm alities (e.g. increased or fluctuating m uscle tone, retarded developm ental patterns, late disappearance or pathological retention o f primitive reflexes, etc.) and is som etim es accom panied by m ental retardation. CaWo&u^ T V ©Ao.w> vxwctrifcU trttdAlc. CXrcbreA 1. Schematic representation o f a section through the cerebrum. Fig. A . Prenatal factors C A U SE S 1. T raum a: (a) B low o n the abdom en especially during 8th-9th m on th o f pregnancy with resultant occipital contusion. , (b) Car accidents, especially towards end o f pregnancy. (c) A ttem pted abortion— by chem icals or som e type o f instrument, e.g. knitting needle. 2. D iseases: (a) Virus infections (e.g. measles, influenza, mumps, chickenpox). These cause a slight m eningo­ encephalitis concurrent with the m other’s illness. It usually resolves as the m other recovers, but som etim es with a residual brain lesion caused by adhesion form ation o f the external fluid spaces. A cyst m ay form or there may be blockage o f the external or internal fluid spaces with resulting hydrocephalus. (b) Syphilis and tuberculosis have becom e very rare causes. (c) T oxoplasm osis— early in pregnancy this causes a m alform ation o f the foetus, but later infections cause chorioretinitis and hydrocephalus with calcification o f the subventricular areas o f the brain. (d) R u bella may cause heart lesions, cataract and deafness or m icrocephalus with hydrocephalus e xtem u s and internus. 3. Circulatory disorders: (a) Deficient oxygen especially in the first few weeks and months o f pregnancy w hen no bloodvessels are found in the embryo. (b) Haemorrhages during pregnancy, especially with threatening abortus. (c) Erythroblastosis causes interference with blood­ vessel permeability— due to the iso-immunization o f the Rh-negative m other by the Rh-positive foetus. It is accompanied by kernicterus (the basal nuclei are stained by bile, with toxic degeneration o f the nerve cells). Anti-Rh-agglutinins in the m other’s blood pass through the placenta and agglutinate the red corpuscles o f the foetus. It is treated by trans­ fusion o f O-Rh-negative b lood directly after birth. 4. Other: Effects o f Radiation—R on tgen rays may cause bloodvessel changes with serious brain parenchyme interference, especially during the second h alf of pregnancy. Even ultrasound waves have been thought to cause brain lesions. 5. M etabolic Disorders: (a) Vitamin deficiency. (b) Deficiency o f trace-elements (e.g. copper). (c) Diabetes mellitus. (d) Protein—deficiency dystrophy o f the mother. 6. Bloodfactors: (a) R h-factors— as described a b o v e (3c). (b) Incompatibility o f the bloodgroups o f the mother and father (e.g. mother group B and father group O—both Rli-positive). This is very seldom seen. B. N atal factors 1. Mechanical lesions: (a) Haemorrhage—subdural haemorrhages may be due to tears o f venous vessels (e.g. sinusses or basal veins below the Tentorium) which may be caused by the friction action o n the head in the birth canal, (b) Traumatic tissue damage is seldom seen e.g. contusion due to forceps delivery or very difficult birth through a narrow pelvic outlet. 2. Asphyxia: (a) Placenta praevia. (b) Prematurity. (c) Lengthened or difficult birth due to inadequate uterine contraction or a false rhythm o f the uterine contractions e.g. during hypertonic continuous contracture with no periods o f rest in between. The intra-uterine pressure during labour con­ tractions is much higher than the systolic blood- pressure o f the baby. The effect o f asphyxiation is increased by the usual delay before normal breathing begins. These lesions are mainly found in the cortex and basal ganglia with scar formation, so-called Plaques fibromyeliniques (in the cortex) and R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) JUNE, 1971 P H Y S I O T H E R A P Y Page 9 Status m armoratus (in the brainstem and basal ganglia). The outer layers o f the brain are more sensitive to circulatory dam age then the brainstem, thus the baby will survive in spite o f extensive cortical damage. D iffuse scar-tissue is formed as a result o f ischaem ia (Fig. 2). H ypoxaem ia causes the form ation o f m ore localized single areas o f scar-tissue and cyst form ation in the white matter o f the brain. Fig. 2. Diffuse brain damage due to asphyxia and resulting ischaemia (quadriplegia with athetosis). (d) V enous congestion leading to oedem a causes a typical conic lesion o f the brain, the base stretching from both sides o f the longitudinal sinus. The apex m ay reach d ow n to the brainstem ganglia. This causes different types o f spastic diplegia with possible athetosis o f the arms and face. Fig. 3. Venous congestion with typical conic lesion. I/////I Pure spastic diplegia. Area marked with dotted line: Spastic diplegia with athetosis o f arms and face. (e) M any o f the typical child hood hem iplegias arise from dam age during birth, causing the form ation o f porencephalia (the presence o f cysts in the surface o f the brain due to an arrest o f develop­ ment or to birth haem orrhage: B lack’s M edical D ictionary— W. A . R . T hom son, M .D .). The condition is generally associated with serious mental defect. T he lesion, usually found around the fissure o f Sylvius, is probably caused by a temporary ischaem ia in the area o f the carotid artery. Often the form ation o f poren­ cephalia is a limited tissue defect so that the rest o f the brain is relatively intact resulting in only slight mental defects. M ore diffuse dam age causes m ore serious mental defects. Fig. 4. Spastic hemiplegia. ( f ) Cord around the baby’s neck. C. Postnatal factors (up to fifth year o f life) 1. C irculatory disorders: (a) W hooping cough eclam psia— bloodvessels are weakened and remain m ore permeable or large adhesions are formed. (b) Continuous convulsions (“stuipe”) where the brain cannot cop e with the angio-spastic circula­ tory disturbance. (c) Cerebral em boli. 2. Infections: (a) Perivenous encephalitis and meningitis. (i) It m ay occur concurrently with influenza, mum ps, rubella and chickenpox. D iffuse lesions in the brain and spinal cord are produced. Fig*. 5. Diffuse , lesions due to perivenous encephalitis and meningitis. Various neuropathological pictures may result. (ii) It may also be caused by vaccinations, especially if done late (between 3rd-9th or up to 12th year o f life). The danger lessens after 17th year. (iii) Penicillin and sulphonam ide dam age o f the brain. R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) Page 10 P H Y S I O T H E R A P Y JUNE, 1971 (b) M eningism us m ay cause adhesions in the external or internal fluid spaces, which m ay cause o b ­ struction to the normal flow o f the meningeal fluid. 3. M e ta b o lic disorders: E.g. nutritional dystrophy o f the newborn. 4. Traum a: Car accidents mainly, but any kind o f trauma causing haemorrhage in the brain and skull fractures. Car accidents cause increasingly m ore brain lesions as speed and traffic increases. 5. B rain tum ors: E.g. N eurofibrom atosis. C L A SSIFIC A T IO N A. Spastic Type (Hypertonic) 1. Q uadriplegia—all four limbs, neck and head, m uscles o f articulation and m astication and eye m uscles are all affected. U sually there are perceptual problem s as well as m ental defects. 2. D iplegia— legs and lower trunk mainly affected. 3. D ouble hem iplegia— arms and headm uscles som etim es m ore spastic than legs. T he trunk m uscles are also affected. 4. H em iplegia— one side o f the body and the h alf o f the head and face are affected. 5. Triplegia— som etim es the two lower limbs, but only one arm is affected (the other arm seems norm al). It may be accom panied by spasticity o f som e head and face muscles. It is actually a quadriplegia with one arm fairly normal. 6. M onoplegia— o n ly one limb, usually an arm, is affected. It is actually a hem iplegia with the leg fairly normal. B . Athetoid T he trunk, lim bs and facial m uscles as well as those o f articulation and speech are affected. C. A taxia M ainly characterized by p o o r balance and inco-ordination. D . Hypotonic spastic The so-called “ floppy” child, is a quadriplegia with the head and face also affected. H yp oton ia usually develops into spasticity, ataxia, or athetosis in later life (e.g. about puberty). E . Hyperkinetic A hyperkinetic child with fairly m inim al brain damage, with little or n o physical disability (except awkwardness or stum bling or impaired fine co-ordination) but with mental retardation, often presents serious perceptual problem s and a very short concentration span. F. M ixed cases. CH A R A C TER ISTIC S A . Spastic Type 1. Quadriplegia: This is due to diffuse and extensive lesions o f the brain. It m ay be com bined with athetosis or rarely with ataxia, depending on the extent o f the lesion. Quadriplegia is characterised by increased m uscle tone, usually o f a rigid rather than clasp-knife type. One h alf o f the body is usually more affected than the other and spasticity is u su a lly w orse in th e low er extremities. Predom inant flexion o f the hips and adductor spasticity leads to scissoring o f the legs. There may be flexor spasticity or hyperextension o f the knees with plantarflexion o f the feet, which may be in equino-varus or -valgus position . The shoulders are protracted and the arms adducted and flexed; the elbow s are flexed and pronation o f the forearms, the hand in ulnar deviation, fingers flexed and the thumb in the palm o f the hand. There is often hyperextension at the m etacarpalphalangeal or proxim al or distal interphalangeal join ts (the typical sw an-neck deformity). T he face is rigid and slow to react, speech is usually affected and a tongue thrust and mandibular stretch reflex are present. There is difficulty with sw allow ing which results in dribbling. D ivergent or convergent squints are com m on arid often there is very little eye m ovem ent. N orm al developm ent is retarded and mental retardation is usually present. Abnormal tonic reflex activity is present. T he M oro reflex does not disappear and often is very marked. Certain primitive reflexes, norm ally present in the new b o m , do n o t disappear at the norm al tim e (e.g. Asym m etric T on ic N eck Reflex and others). Other reflexes like the Sprungbereitschaft reflex appear late or are absent. T he severity depends on the extent o f the brain lesion. 2. D iplegia. T he severity depends on the extent o f the brain lesion . In diplegia, the characteristics o f the lower extremities and lower trunk is similar to the quadriplegia, but the upper trunk, face and upper lim bs m ay show little or no signs o f spasticity. 3. The double hem iplegia is also similar, except that the upper trunk and upper extremities are m ore severely affected than the lower trunk and lower extremities. 4. and 5. Triplegia a n d m onoplegia. In triplegia and m ono­ plegia to o the affected lim bs sh ow similar characteristics of spasticity. In hem iplegias only o n e h a lf o f the body shows these signs. The extension elem ent o f the positive supporting reflex is m ore dom inant in hem iplegias with the result that the leg is u su a lly a d d u c te d , internally rotated and flexed at the hip, extended at the knee with plantarflexion and inver­ sion o f the fo o t. The shoulder is retracted. B . Athetoid T he term “ A th etosis” was first used by H am m ond 1873 and means “ w ithout a fixed p osition ” or m ore generally “ w ithout co-ord in ation” . It is caused by dam age to the extra-pyramidal system. T he tone fluctuates continually causing grotesque mass m ovem ents (which are n ot necessarily due to increased reflex activity) especially when voluntary - m ovem ents are attem pted. The m uscles o f facial expression, the tongue, breathing, sw allow ing and articulation are affected. This o ften leads to frustration, especially in moments o f em otion al excitem ent. It is necessaiy to include emotional control and quietness in therapy. A th etosis m ay be accom­ panied by spasticity. A th etosis is often n o t observed in the very youn g baby, and usually appears when intentional m ovem ents are attem pted later. D u e to the m ass movements, difficulty with articulation, m astication and sw allowing as well as the com m on deafness, the intelligence o f these children is often underestimated. Y et, w hen athetosis is not com bined with other defects, the average athetoid usually has a higher intelligence than the average spastic. The frustrations these children experience often leads to the developm ent o f faulty social relationships. Even paranoid tendencies may som etim es develop. Intensive physiotherapy to teach quietness and control of grotesque m ovem ents, must be accom panied by remedial teaching. Progress will be determined by the degree of existing central personality functions (e.g. how much the child understands, how much he absorbs, h o w much drive and interest he has to com m unicate with the world etc.). C. Ataxia A taxia presents a disorder o f the afferent and efferent pathways. It can be accom panied by spasticity. There are clear co-ordination disturbances, especially o f the finer m ovem ents which are often accom panied by an intention tremor. The co-ordination disturbances are n o t to be con­ fused with those which in athetosis are caused by fluctuating tone. In add ition there are balance and equilibrium reaction R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. ) disturbances. T hese balance and equilibrium reactions d e v e l o p later than normal and remain slow and weak, the hildren often walking with a wide base or a type o f P a r k i n s o n i a n gait (this is seldom seen). The w hole develop­ m e n t a l pattern is retarded. U su ally there are also disturbances !" sucking and sw allow ing in the baby; and som etimes b r e a t h i n g difficulties too. T hese signs are less clear in the small baby, but becom e more clear with increasing age. A very typical sign is the intention tremor present during movements o f fine co-ordination. D . Hypotonic spastic These children sh ow hypotonus with extremely weak head and trunk control. They are often m entally retarded a n d lacking in m otivation. They show m any o f the abnormal re fle x patterns even in later life. A th etoid, spastic and/or ataxic tendencies usually develop later (abou t puberty). E. Hyperkinetic These children have m ainly frontal lobe lesions causing defective intelligence, various types o f perceptual problems, lack o f concentration and hyperactivity or clumsiness. Often a retarded m otor developm ent may be noticed— this m ay be due to the defective intelligence. U sually there is little or no motor disturbance. The characteristics o f the cerebral palsied are seldom observed at or shortly after birth. S oon , however, the following anom alies o f the sim plest vital functions m ay be observed: 1. Difficulty in sucking, sw allow ing and later chewing too. 2. Breathing anom alies— e.g. very shallow breathing, frequent bronchitis or pneum onia, etc. 3. Peculiar crying: (i) Very soft and without strength. (ii) M ore often a shrill, piercing cry. JUNE, 1971 P H Y S I O H E R A P Y Page 11 4. S o o n a disturbance in concentration pow er is noticed. T h e eyes o f the baby cannot follow m oving objects or fixate. A squint is often present. 5. T h e child does not kick (or kicks more with one leg and keeps the other fairly still). H e does not attem pt to grasp. H is hand-m outh co-ord in ation and hand-feet m otor developm ent are slow to develop. 6. Primitive reflexes are slow to disappear. P ositional reflexes appear later than norm ally, and the normal developm ental pattern is retarded. 7. Epileptic attacks may occur in different form s and inten­ sity. 8. According to C ollis,1 intelligence in m ore than 75 per cent o f Cerebral palsied children, is below normal and less than 25 per cent have a norm al I.Q. A b ou t 25 per cent have severe m ental retardation and w ill never be educable, and co uld n o t e ve n be trained to perform the sim plest tasks or ow n bodily care. A b o u t 50 per cent have less severe m ental retardation in different degrees and may be educable or may at least be trained to perform basic activities o f daily living and sim ple tasks. B IB L IO G R A PH Y 1. Collis, E., and others: The In fantile C erebral Palsies. (W . H einem an, L ondon, 1956). 2. Lindem ann, Prof. K ., and others: D ie I n fa n tile n Z e r e b r a l Paresen. (G eorge T hiem e Verlag, Stuttgart, 1963). T his last b o o k was used for all reference to the aetiology and aided in com piling the characteristics o f the types o f Cerebral Palsy. Part II, about the treatment for Cerebral Palsy, will be published in the next magazine. G E N E R A L R E T IR E M E N T — R E S IG N A T IO N Miss G . Jones has retired from the post o f Principal o f the Physiotherapy Sch ool, K in g Edward VTII H ospital, Durban. Mr. C. A . Liggins, M .C .S.P., H .T ., D ip .T .P ., has taken over this responsible position and it is understood that M iss Jones will return to a teaching post at the school following overseas leave. Mr. J. Stockton, M .B:E., S .R .N ., M .C .S.P ., H .T ., D ip.T.P., has resigned from the Physiotherapy College, Pretoria and his place has been filled by M iss A n ne Hendry, who is A cting Principal from April, 1971. Mr. Stockton , w h o has left for overseas played a very active part in Physiotherapy in South A frica from the day he arrived in the country in A pril, 1965. H e was appointed to the N ational Executive C om m ittee and the Training and Registration C om m ittee, where his past experience, clear thinking and quick appreciation o f the im portance o f a situation under discussion were o f inestim able value. H e will be greatly m issed in all fields o f the profession, teaching and administrative in particular. We take this opportunity o f wishing b oth these ex- Principals “G o o d Luck” for the future and offer them our sincere thanks for the loyal service they have given to the profession in South Africa. N O T IC E T O A L L M E M B E R S C H A N G E O F A D D R E S S A ll members o f S.A .S.P . and those registered with S .A .M .D .C . are reminded o f the im portance o f keeping the Society and the South A frican M edical and Council informed o f any change o f address. T hose members w h o have recently married must accom pany their change o f address with a p h otostat copy o f their marriage certificate. N O R T H E R N CA PE SO C IE T Y F O R T H E CARE O F C R IP PL E S R E : P O S T -M A T R IC B U R SA R IE S The Executive C om m ittee o f the N orthern Cape Society for the Care o f Cripples has created a Bursary o f R2 00 per annum for C oloured or Bantu Post-M atric students who wish to qualify as Social W orkers or Physiotherapists at recognised Universities or Training Schools. Bursary holders would be required to w ork in the Cripple Care field for a suitable number o f years, if necessary. A p plication forms m ay be obtained from: The Secretary, Northern Cape Society for the Care o f Cripples, P.O . B ox 928, Kimberley. R ep ro du ce d by S ab in et G at ew ay u nd er li ce nc e gr an te d by th e P ub lis he r (d at ed 2 01 3. )