CASE HISTORY

Introduction

This case history highlights the gross modification that severe sensory impairments can cause to the expression of psychotic symptoms due to restricted modes of communication and altered subjective sensory experiences. The methods of assessment, modes of treatment and consideration of diagnostic criteria need to be undertaken in a more flexible and creative manner in these patients.

Identifying data

The 45 year old single lady living alone in a house owned by her brother had longstanding severe physical disabilities in the form of profound sensori-neural deafness and advancing severe visual impairment due to retinitis pigmentosa, known as Usher’s Syndrome. She communicated by way of ‘finger spelling’, a form of sign language, and was effectively mute. She received a disability pension but lived independently and, up until some months previously, worked full-time at a sheltered workshop. She maintained a close supportive relationship with her brother & socialised regularly with friends within the local deaf community up until 12 months previous to the initial assessment.

Presenting symptoms

The subject complained that "evil angels" had arrived after her mother died 7 years earlier, who "hit" her around the head when they came, causing a painful headache. She could hear them walking. They "introduced" themselves. She could not see them. They spoke in "sound", not sign. They told her "lies and rubbish". She did not know why they tormented her. She did not believe she deserved the abuse & had done nothing to attract them or make enemies. She denied depressed mood, self-criticism, hopelessness or suicidal thoughts. She said, "I am good, they are bad". She denied insomnia, describing seven or eight hours uninterrupted sleep per night. However she complained of daytime hypersomnolesence, persistent lethargy and increased appetite. She had gained a significant amount of weight and been eating excessively. She had only been attending work one day per week but could not explain the reduction. She denied that her eyesight had deteriorated or that she was impaired from any disabilities.

History of psychiatric illness

Over the previous 3 years, her brother had found her conversing with an imaginary person on several occasions when he had entered her house unannounced and seen her signing in an interactive fashion. When he had asked her who she was conversing with, she had simply said, "friends". He did not wish to be intrusive by confronting her further. Over recent months he had found her conversing with increasingly larger numbers of imaginary people, up to 6, simultaneously. These occasions were described as resembling "dinner parties" of imaginary people, with rapid signing, gesticulating, rotation of her body direction and facial gaze. He was unable to interpret what was being said due to the rapid speed of the signing.

There developed episodes of agitated behaviour both at home and the workplace, where she would flail her arms franticly about her head and shoulders, grimacing, posturing and ducking – as if she were avoiding swooping birds. She became tearful, anxious and exhausted during these episodes. She said she had been attempting to fight off "evil angels", who were attacking and hitting her. These episodes interfered with her ability to carry out her work as the angels were distracting and she was at risk of accidental self-harm whilst using industrial machinery.

Over the previous 3 months, her brother described the emergence of additional symptoms. It was becoming increasingly difficult to communicate with the subject. She appeared confused by some questions, did not always reply, was non-spontaneous, provided "nonsense" responses and used signs that he had not seen before. She increasingly appeared detached, emotionless, distracted, preoccupied, inattentive and unmotivated to socialise, work or converse. She continued to shop, bank and cook, but spent most of her time in bed and only agreed to attend work infrequently.

PAST MEDICAL HISTORY

Profound deafness had been diagnosed a few weeks after her birth & poor visual acuity when she was 3 years old. Retinitis pigmentosa was discovered to be the cause and Usher’s Syndrome diagnosed. She was trained to communicate using ‘finger spelling’ rather than orthodox signing, due to the progressive nature of her blindness, from the age of 4. A considerable dependency on the residual vision available continued to be fostered, however. In addition to her walking cane, she used visual aids such as a projector & large screen, which enlarged print or pictures to a mammoth scale, and brightly coloured flashing lights, which functioned as visual door bells and telephone ringers. Five years previously she required surgery to remove a cataract. Over the previous 18 months, a dramatic acceleration of her visual impairment occurred. This had been due to continued pigment deposition in the light-sensistive retinae, damaging rods and cones, resulting in impaired visual acuity (blindness) and narrowing of the visual fields (tunnel vision).

PAST PSYCHIATRIC HISTORY

She was referred to a psychiatrist 18 months previously. Over 12 months the episodes of hallucinosis were confined to solitary periods in her own home. She was prescribed thioridazine & then haloperidol, both of which provided inadequate symptom relief & caused drowsiness. There was no past history of substance abuse.

PREMORBID PERSONALITY

She was reported to be a staunchly independent woman who showed little concern for obstacles & did not perceive herself to be handicapped. She was stoic, capable and conservative. She enjoyed socialising and had multiple friendships, although much of her time was spent alone. She was pedantic about her domestic affairs, money, appointment times and daily routine.

SOCIAL HISTORY

She had been adopted at an early age & knew nothing about her biological parents. Her adoptee parents were both middle-aged, living in a medium sized coastal city. She was the youngest of the three unrelated siblings. When aged 4 years old, she became a boarder at an institution for disabled children in a metropolitan city for 2 years, separated from her family. The she became a day student until she left school aged 16. She had been sexually assaulted when aged 20 & the details remain unknown. She worked as a jewel setter for 11 years, a knitting mill for 10 years, then at the sheltered workshop. She had been an enthusiastic and diligent employee. Her brother, aged in his 60s, had been the only family member to learn to sign & was her primary support. He visited every weekend. He developed a serious general medical condition 18 months previously. Her father died 17 years previously & her mother 7 years prior. She was said to have handled the loss of her mother, who she had been living with, well. She continued to function independently. She moved into a smaller house, which was modified by occupational therapists to compensate for the subject’s sensory handicaps. She was able to mobilise independently in the streets and catch public transport, using her walking cane as the only aide. She conducted her own shopping, banking and cooking. A private bus transported her directly to and from the factory workshop.

Appearance and Behaviour: Obese middle-aged lady with short blandly styled straight black hair, casual attire, holding a walking cane. Rapid oscillating rotary optical nystagmus was a strikingly feature. Able to spontaneously determine the direction to face the interviewer. No glasses were worn. No evidence of self-neglect. She was noted to be rocking from side to side on the chair intermittently. She also made gutteral vocalisations randomly. (Her brother said neither of these behaviours were usual for her.) No psychomotor retardation or excitation was evident. She was cooperative and polite & did not appear guarded or suspicious. In fact, she was surprisingly passive and trusting of the author and other staff when they directed her around the ward.

Mood: Euthymic. Denied anxiety and dysphoria except for episodes when the angels visit her.

Affect: Blunted, with infrequent partial smiles and winces noted.

Speech: The interpreter sat right beside her, drawing shorthand-type symbols and letters on the palm of her hand with their index finger. She would then do the same on the interpreter’s palm to provide her response. Communication was slow and laborious – impeded by both her abnormal thought form as well as the lengthy process of spelling out each word through the translator.

Thought Form: She was non-spontaneous and there was a poverty of thought form and content. She had difficulty responding to other than ‘closed questions’ i.e. brief, simple questions requiring ‘yes-no’ answers. She often produced signs that were idiosyncratic and unrecognisable to the interpreter. Her responses were vague, disorganised and often incoherent. Questions frequently needed to be repeated, due to a lack of response or perseveration. Replies were tangential or loosely associated with the theme of the query.

Thought Content: She described somatic and possible auditory hallucinations. The source was attributed to persecutory delusional constructs with religious theme. She appeared preoccupied and distracted by these phenomena. She also complained of hypersomnolesence, lethargy, hyperphagia and weight gain. There was evidence of impaired communication due to thought disorder and disorganisation. Her occupational and social function had declined.

Perceptual disturbances: She described somatic and auditory hallucinations but was not observed to be responding to hallucinations during the interview.

Cognition: Her concentration and attention was impaired. She was oriented in person, place and time. When asked the time, she immediately flipped up the lid of her watch and felt for the position of the watch hands, and gave an accurate time. Registration was impaired, requiring multiple repetitions of three word items before she was able to repeat the items sequentially. 3-minute short-term memory was also impaired, the patient recalling one out of three items on listing. Responses were concrete.

Insight and Judgment: She was unclear of the reason for her admission although she was aware her psychiatrist had recommended and arranged it. She knew it was related to her somatic complaints, which were related to the ‘evil angels’.

PHYSICAL EXAMINATION

There was no pattern to her obesity. Her face and shins were hirsuitous. Her retinae were not able to be visualised due to the rapid rotatory nystagmus. She was able to tell it was daylight. She was able to visualise silhouettes and turned her face to my direction when seated in front of me. She was unable to count fingers held up in front of her eyes. Extra-ocular movements appeared to occur through full ranges of movement. Smell, taste and facial sensation were all intact. No primitive reflexes were present. There was no asymmetry or weakness of facial, masticatory or deglutition muscles. She was profoundly deaf. She could shrug her shoulders. Tone, power, reflexes, sensation to pin-prick and light touch, proprioception and vibration sensation of her upper and lower limbs was normal and symmetrical. Her Babinski reflexes were down-going and negative. There was no abnormality identified in her cardiorespiratory or gastrointestinal systems on examination.

 FORMULATION

The subject is a 47-year old woman living independently despite lifelong profound deafness, gradually deteriorating vision due to retinitis pigmentosa and secondary mutism, due to a hereditary disorder known as Usher's syndrome.

Her developmental history was marked by adoption, deficits of maternal and paternal attachments, a prolonged separation from the family between the ages of four and six years and a sexual assault at age 22. A close relationship had developed between the patient and her elder brother who served as interpreter with the other family members. Despite the early traumas, she compensated for her sensory deficits remarkably well and obtained optimal independence occupationally, socially and in terms of daily activities, with the continued assistance of her brother. There were no romantic relationships.

There was a 3-year history of the insidious development of a psychotic illness of increasing severity. The patient's presentation was characterised by persecutory delusions with religious themes, complex somatic (haptic and kinaesthetic) hallucinations, thought disorder, affective flattening, alogia and episodic catatonic excitation. There had been a 12-month history of decline in social and occupational function.

The precipitating and propagating factors appeared to be a combination of: an acceleration over 12-18 months of her progressive visual impairment, genetic and organic vulnerability factors, premorbid obsessive personality traits, neuroleptic induced sedation, impaired maternal and paternal and attachment, and the possible impact of her brother’s illness and hospitalisation.

PSYCHIATRIC DIAGNOSIS (BY DSM-IV CLASSIFICATION):

Axis I:     Psychotic Disorder Not Otherwise Specified

Axis II:    Obsessive personality traits

Axis III:    Usher’s syndrome
               (sensorineural deafness, retinitis pigmentosa, secondary mutism, cataract)
                Neuroleptic induced sedation

Axis IV:     Progressive visual impairment

                Reduced sensory stimulation due to social and occupational withdrawal
                Brother’s physical illness

Axis V:      Acute: GAF=34
                Chronic: GAF=95

1. Psychotic disorder due to Usher’s syndrome (‘organic’ psychosis)
2. Schizophrenia
3. Sensory deprivation induced psychosis
4. Dementia with hallucinations and delusions
5. Alternative general medical condition

1. Observe in hospital drug-free to assess behaviours and symptoms. Consent was obtained from both the subject and her brother to allow the author to videotape her whilst on the ward.
2. Exclude other organic causes or contributors with appropriate investigations
3. Evaluate response to re-establishing and maximising sensory stimulation
4. Balance pharmacological benefits and side effects
5. Formulate optimal discharge management

Accommodating to new environment

She was able to orientate herself around the ward within the first week. She was independent with activities of daily living, requiring minimal assistance or prompting. Her sleep-wake cycle was observed to be normal. No vegetative symptoms were evident.

Effect on ward staff and patients

Her presence stimulated patients and staff to learn to finger spell, so that they could communicate with her. A remarkable amount of interest was generated. Because the learners’ pace was very slow, this matched the patient’s impaired ability to sign.

Stimulatory Input

Two individual interviews were conducted on average per week, one with the author and the other occurring in a weekly case review with other members of the multidisciplinary team, using interpreters. Her brother was also utilised during his weekly visits. She was encouraged to attend groups, outings and craft sessions. Her magnifying projector was placed where the patient could have access. She was thus provided with a continuous input of sensory stimulation.

Observed abnormal behaviour

She was observed to be responding to perceptual phenomena, ‘conversing’ using sign episodically when alone. This never occurred when she was in a public area, in others’ presence, while interacting with people or involved in ward activities. It took place in her own living space, a small area in the female dorm demarcated by curtains, whilst sitting on her bed. She gesticulated and signed at a rapid pace, using a wide range of facial expressions - frowning, smiling, laughing, perplexity, irritability - within short time frames. She became startled if interrupted during an episode, but interacted with the interjector appropriately with minimal hesitation. These episodes were videotaped. It was not possible to interpret the content of the signing & she did not appear to be agitated or distressed during the episodes.

Phenomenology

She acknowledged daily visitations from the angels. She described them in persecutory terms, as "teasing" her, "gossiping" & playing "sex games". She believed they were sent from God. Her level of insight was inconsistent, at times realising they were figmentary and at other times convinced they were sentient entities. She denied painful tactile hallucinatory experiences.

Mental state improvements

She began to appear more reactive, responsive and attentive. She seemed less disorganised and was using more identifiable signs. This enabled her to express and exchange more complex ideas. She remained moderately thought disordered, using nonsensical sentences and words, tangential replies and incongruent affect. She reconnected socially with her Deaf & Blind Society friends. She began to knit a scarf.

Phenomenology

She provided more complex descriptions of the angels. Olfactory hallucinations were described - the angels smelt "smoky" but "fair", as well as somatic hallucinations - they made her feet "drunk" and "dizzy", she felt them "kiss" her and play "sex games", pointing to her groin whilst giggling, suggesting physical sensation; she also complained of a choking sensation in her throat when they visited. She said the angels "follow" her, coming in groups of up to 9. She found the question whether they could fly or have wings very humorous, denying these characteristics.

Mental state improvements

She was signing without fault by the third week, presenting her thoughts in an organised, complex, spontaneous fashion that she had up til then been unable to do. Her facial mobility and expressiveness improved. The interpreters confirmed that her degree of repetition and perseveration returned to within normal limits for deaf people. She spontaneously indicated that the angels come and go as they please, any time of the day. She complained of the intrusion upon her privacy and interference in her life. It was decided to re-introduce haloperidol at the small dose of 2.5 mg daily in an attempt to eliminate these residual psychotic symptoms.

Social and occupational functioning

Her interactions and sociability were nearing normal baseline levels according to her brother. She was fully oriented to the ward and coping well with outings. Interest in the world around her returned, such that she began to attend more social functions at the Deaf Society and requested to return to her factory job. She began to use her magnifying projector intermittently. Her mental state improvement was also evident outside the ward environment.

It was clear that the subject had responded positively to a stimulating environment. A dramatic remission of her psychosis, including thought disorder and negative symptoms, occurred solely by bringing her into hospital, and despite cessation of antipsychotic medication. Contact was made with the manager of a Deaf Society hostel to consider her suitability for entry. She was surprisingly quick to agree to moving into the hostel, signalling that she was gaining insight into her disability.

WEEK 4

The subject was communicating in sign at a rapid pace, such that the interpreters were finding it difficult to follow. There was no evidence of formal thought disorder. She became more disturbed by the angels. She reported that they had become angry about the hostel, had been teasing her and gossiping. During one interview, she became clearly irritated and too upset to continue talking about them. There were continued episodes of signing whilst by herself. Psychomotor agitation was not observed, however nursing staff reported the emergence of infrequent episodes of frantic motor activity which sounded similar to the 'agitated episodes' that had occurred in her workplace. Haloperidol was increased to 5 mg daily.

WEEK 5

She became more withdrawn and sleepy and therefore less communicative. Concern arose that sedation would initiate a cycle whereby she would become less stimulated, exacerbating psychotic symptoms due to sensory deprivation. Haloperidol was reduced back to 2.5 mg daily. Her mental state on discharge represented a balance between the subject's residual rumbling hallucinosis and drug side effects, the latter appearing capable of undermining the benefits obtained should the degree of sedation be too substantial.

INVESTIGATIONS:

Electrolytes, liver function tests, calcium, full blood count: all within normal limits. Reproductive, adrenal and growth hormones: all within normal limits.

Folate, vitamin B12: within normal limits.

Thyroid function tests: within normal limits.

Syphilis serology: negative.

Cerebral CT scan: within normal limits.

EEG: within normal limits.

FTG-PET scan:

DISCUSSION

For a mental disorder to be considered to occur secondary to a general medical condition there must be evidence of a direct physiological mechanism¹. Evidence may include a direct temporal association between the onset, exacerbation or remission of the general medical condition. Late age of onset (over 35 years old) occurs more rarely in schizophrenia than an organic psychosis¹. Treatment targeted to the general medical condition that alleviates the symptoms of both it and the mental disorder may provide evidence of an aetiological relationship. It was thus considered significant that ‘treating’ the subject’s sensory deprivation with a sensorially stimulating environment led to a partial but significant remission of psychotic symptoms.

If the mental disorder is a psychologically mediated response to an acute stressor Brief Psychotic Disorder may be considered. Losing one's vision could be considered a very significant stressor. The subject did not acknowledge the deterioration in her visual impairment when she was admitted, but she was acutely psychotic at the time. Although it represented an acceleration in the progress of visual deterioration, a 12-18 month time frame is too lengthy a period to be considered ‘acute’ and DSM-4¹ suggests a time period of one month for the duration of the psychosis itself as a cut-off. The subject's psychosis was of 2-3 years standing, with a gradual progression of positive and negative symptoms. The increasing visual impairment was considered to be a significant precipitating and propagating factor.

The specific phenomenological characteristics may also be suggestive. Atypical symptoms are those that lean away from a diagnosis of schizophrenia. Olfactory and gustatory hallucinations and religious delusions suggest TLE¹ and other organic states¹³. Visual and somatic (haptic and kinaesthetic) hallucinations are common in delirium and substance withdrawal¹³.

The subject's presentation was characterised by persecutory delusions with religious themes, complex somatic (haptic and kinaesthetic) hallucinosis, disorganised signing (speech), affective flattening, alogia, episodic catatonic excitation, social and occupational dysfunction, a symptom duration over six months, and lack of mood symptoms (distinguishing from a mood or schizoaffective disorder).

The uniqueness of the subject's context—her combined visual and hearing sensory deficits—needs to be taken into account when considering the atypical nature of her presentation. Her restricted sensory experience may hypothetically make her more likely to encounter hallucinations in the remaining modalities. This would 'fit' with the anecdotal belief that remaining senses become heightened in sensorially impaired people. There is evidence that the brain's plasticity permits a process of "replacement of idle synapses by connections reflecting activity".¹⁹

Although the subject described hearing angels communicating in "sound" rather than sign, she was born profoundly deaf and this precluded the opportunity to learn spoken speech. Also, evidence from animal experiments indicates that a lack of visual stimulation during a critical period of early development, even in a potentially functional (structurally intact) occipital cortex, prevents further function of that cortex:

If one eye of a newborn animal is covered for the first few months of life, that eye will be permanently blind (in a perceptual sense) when uncovered .... It is not simply a result of the eye not being exposed to light . . . .if the retina is exposed to light but not patterns, the same functional blindness results.¹⁹

If this process is extrapolated to the auditory system, it would suggest that the subject simply does not have the occipital apparatus necessary to experience auditory experiences. The subject described the nature of her communication with the angels to be unlike ‘signing’, which in her case is purely somatosensory i.e. the production of symbols impressed on the palm of the hand, rather than visual gesturing as conducted by the purely deaf It was also perceived as being distinct from her own thoughts. We might consider that she was more likely to be experiencing thought insertion than auditory hallucinations.

Alternatively, the subject was observed to be 'signing' vigorously whilst responding to the hallucinations, so it is also feasible that she was experiencing haptic hallucinations - hallucinations of touch¹³ felt cutaneously on her palrns and perceived as signing originating from the 'evil angels', although she denied this when questioned on one occasion. Certainly, she experienced haptic as well as kinaesthetic hallucinations - those of muscle and joint sense¹³, during the 'agitated episodes' associated with flailing arms and bizarre posturing, which she attributed to the 'evil angels' hitting her.

Conversely, it could be argued that the subject's symptoms are remarkably 'typical' of schizophrenia in their characteristics, for someone in her context. Persecutory delusions are most common, and the subject described being tormented, followed, subjected to ridicule and physically assaulted by 'evil angels', who she had read about in a biblical passage. As illustrated, she was probably experiencing bizarre delusions of thought insertion. Although auditory hallucinations are most common in schizophrenia, hallucinations may occur in any modality, and the auditory modality can probably be ruled out for the reasons given above. The nature of her hallucinatory phenomena was clearly complex, as evidenced by the subject's describing up to nine 'evil angels' conversing with her simultaneously. This phenomenon, along with thought insertion, may be included in Schneider's list of "first-rank symptoms" - considered to be especially characteristic of schizophrenia¹⁴.

Other features that were consistent with a diagnosis of schizophrenia were:

Against a diagnosis of schizophrenia was:

Psychotic disorder Not Otherwise Specified is suggested by DSM-4 when inadequate or contradictory information prevents determination whether the psychosis is primary (i.e. schizophrenia) or due to a general medical condition. For this reason, as dictated by DSM-4, the author chose this somewhat ambivalent position. Additionally, it is unclear how a sensory deprivation induced psychosis should be considered under the DSM-4 classificatory system.

Usher's syndrome, also known as hereditary deafness-retinitis pigmentosa, dystrophia retinae pigmentosa-dysacusis syndrome, and Graefe-Sjogren, Hallgren's and Helen-Keller syndromes, is an autosomal recessive genetic disorder with complete penetrance. It has a prevalence of 3 per 100,000 in the general population and 3-6% amongst all deaf people^2,10. There have been reports of associated neurological symptoms such as labyrinthine ataxia, intellectual handicap, reduced olfactory and gustatory sensitivity, epilepsy and psychiatric morbidity.

There have been few reliable studies examining the prevalence and phenomenology of psychiatric disorder in subjects with Usher's Syndrome. The main reasons are:

Hallgren⁵ (1959) found that 23% (26 out of 114) of his cases of Usher's Syndrome developed psychoses. Of these, 16 presented with a "schizophrenia-like picture", of whom 13 experienced hallucinations and 11 paranoid delusions. Two cases had acute psychotic episodes and recovered. Hallgren noted that the age of psychosis onset in Usher's patients, at 37 years, was several years later than that for schizophrenia in the general Swedish population. This correlated with the time when visual impairment commonly becomes severe in Usher's patients.

Hicks et al¹⁵ (1978) similarly found that all of his 11 psychotic patients with Usher's had onsets temporally related to the final stages of their loss of vision. They were schizophreniform presentations requiring brief admissions only.

Mangotich et al⁸ (1983) wrote a case report on a 55 year old woman with Usher's who had two admissions for schizophreniform episodes, in which persecutory delusions with religious themes, agitation, anxiety and hallucinations were described. Her initial psychotic episode had occurred at age 48. Her visual impairment began to accelerate after 40 years and was then complicated by the development of glaucoma and cataracts. Her episodes resolved within a few weeks after admission and treatment with chlorpromazine. Her cerebral CT and EEG were both normal.

Mangotich noted that studies had failed to demonstrate an increased incidence of psychosis (or other mental disorders) in early-childhood deaf or blind subjects,whereas progressive loss of hearing or vision in adults is associated with significantly higher rates of paranoid psychoses, as well as depression, suicide and social withdrawal. She considered:

Chronic stress may be induced by gradual loss of vision in persons who have learned to cope with another major sensory disability, deafness, in part by relying more heavily on visual input . . .the interruption of established communication patterns is the source of greatest psychological morbidity⁸.

Certainly there is evidence from studies that progressive sensory impairment is associated with the delusions and hallucinations of late-onset schizophrenia, and, more specifically, visual impairment with Charles-Bonnet syndrome and hearing impairment with musical hallucinosis¹⁸ and delusional states. Indeed, Kraeplin described 'deaf paranoia' in 1919. Studies have also found that general degrees of social isolation, also a form of sensory deprivation, pose a risk factor to the development of late-onset schizophrenia.

Of interest, in terms of content, is Werthheimer's description (quoted in Delusions and Hallucinations in Old Age¹⁸) of visual hallucinations in old people:

Their content is often peopled by menacing and impassive individuals ... The scene ... frequently has sexual overtones.

Also of relevance are the many studies conducted on the psychopathological effects of sensory deprivation and solitary confinement in the German and American literature respectively. Grassian & Friedman¹⁷ (1983) in their review summarised:

The literature described a hallucinatory, paranoid, confusional psychosis in which characteristic symptoms included . . .1) vivid hallucinations in multiple sensory modalities, including the visual, auditory, tactile . . . proprioceptive . . . and olfactory 2) dissociative features ... 3) agitation and "motor excitement" . . . 4) persecutory delusions ... cognitive impairment ...

It was interesting for the author to note that beside the prison at Port Arthur, Tasmania, a psychiatric asylum was built to literally connect to the prison proper. This was in view of the large proportion of inmates who developed psychosis in the course of their extended periods of internment, requiring transfer. The prison boasted an ideology that the prisoners would benefit from a process of spiritual cleansing which would be obtained by sensorially depriving them of all input other than a bible in their cell and weekly sermons. They were allowed out of their cells one-hour per day, to walk in a designated area without social contact. The church was built so that it was not possible to see any other inmates at the sermons. The windows were positioned so that only the sky could be seen, door holes such that only food trays could be inserted (no visual contact made with guards) and the walls and doors built to insulate sound. The prison was in effect a brutal experiment in chronic low-grade sensory deprivation.

In another paper by Grassian & Friedman¹⁶, Smith et al (1961) is quoted, in their study finding:

Normal subjects find great difficulty in adapting to or tolerating the stress of sensory isolation, but schizophrenics find no difficulty.¹⁶

The subject's symptoms clearly remitted in a sensorially stimulating environment and worsened if her senses were further dulled by increasing her antipsychotic to doses that tended to sedate her. These findings would support a conclusion that the subject was in fact not suffering from a primary psychosis (schizophrenia), but suffering from the state of chronic severe sensory deprivation - similar to that of the inmates of Port Arthur prison, except that in her case it was an experiment of nature.

In contrast to these studies, however, is that of Koizumi et al⁶ (1988), who published a case report of a 44 year old male with Usher's with his first episode of a schizophrenia-like illness which had been present for one year. The psychosis was characterised by persecutory delusions, command hallucinations, psychomotor excitation, blunted affect and autism. Cerebral CT and MRI scans revealed diffuse cortical and cerebellar atrophy, bilateral calcification of the globus pallidi, a dilated fourth ventricle, thin corpus callosum and diffuse periventricular high intensity. PET images indicated generalised reduction of cerebral blood flow and cerebral oxygen consumption in the whole cortex and subcortex, but preserved in the basal ganglia. They concluded that "the neuro-radiological findings suggest . . . a metabolic and degenerative disorder in the whole brain, especially the subcortical areas". Thus, although the "mental disorder is almost indistinguishable from that of schizophrenia" it should be "regarded as an atypical or mixed organic brain syndrome" according to DSM-3. This case raises the possibility of a cerebral lesion related to the pleiotropic effects of the Usher's gene as an alternate aetiology.

In our subject, epilepsy was excluded by a negative EEG, normal peripheral nervous system examination, her tendency to cease experiencing hallucinations when interrupted (stimulation intervened) and the neurologist's opinion on examination of the patient and videotapes. Dementia was considered and excluded by observation of the subject through the course of the admission. Clearly her initial mild deficits in attention, registration and short term memory were a result of pseudo-dementia due to disorganisation and acute psychotic symptoms. They resolved as her psychosis remitted.

It is probable that, like in the model of late-onset schizophrenia where "the lower genetic loading in paraphrenic patients appears to be offset by a higher prevalence of exogenous factors (e.g. sensory impairment, social isolation, personality structure, organic brain disease) than those suffering from schizophrenia with early onset",¹⁸ the aetiology of the subject's psychosis is multi-factorial. The factors that may have contributed to the subject's development of a psychosis are:

The reliance on interpreters to not only interpret but also explain and teach about the 'cultural' factors inherent in the subject's subgroup (deaf community), which further modifies symptom-presentation, mental state and communication styles, was considerable. For example, the subject's concrete cognitive style was considered 'normal' for her subgroup. In addition, obsessive personality traits are considered common and adaptive in the deaf and blind communities.

The painstakingly slow pace of communication (due to its mode and the patient's thought disorder) was an obstacle in the context of limited resources with regard to interpreter availability. The lack of a consistent interpreter proved to be both an impediment and clinically useful. With regard to the former, gauging progress may have been more difficult with multiple yardsticks being used. It was beneficial in that it was possible to obtain multiple opinions, perspectives and sources of information. This possibly permitted a more objective analysis of the patient's thought form and content, in addition to an idea as to what would be considered ‘culturally’ appropriate or inappropriate, at baseline for a person with her disabilities.

1. American Psychiatric Association, DSM-IV, 1994.
2. Boughman, J et al, Usher syndrome: definition and estimate of prevalence from two high-risk populations, Journal of Chronic Diseases, 36(8):595-603, 1983.
3. Grondahl, J, Estimation of prognosis and prevalence of retinitis pigmentosa and Usher syndrome in Norway, Clinical Genetics, 31(4):255-64, April 1987
4. Grondahl, J et al, Usher syndrome in four Norwegian counties, Clinical Genetics, 30(l):14-28, July 1986.
5. Hallgren B, Retinitis pigmentosa in combination with congenital deafness and vestibulocerebellar ataxia; with psychiatric abnormality in some cases: A clinical and genetic study, Acta Psychiatrica Scandinavia, 34(suppl 138): 1-101, 1959.
6. Koizumi J et al, CNS changes in Usher's syndrome with mental disorder: CT, MRI and PET findings, Journal of Neurology, Neurosurgery and Psychiatry, 51:987-90,1988.
7. Magalini, Usher's, Dictionary of Medical Syndromes, 3d Edition, p 896, 1987
8. Mangotich M et al, Atypical psychosis in Usher's syndrome, Psychosomatics, 24:674-5, 1983.
9. Nuutila A, Dystrophia retinae pigmentosa-dysacusis syndrome (DRD): A study of the Usher- or Hallgren syndrome, Journal de Genetique Humaine, 18(l):57-88, 1970.
10. Vernon M, Usher's syndrome - deafness and progressive blindness: Clinical cases, prevention, theory and literature survey, Journal of Chronic Diseases, 22:133-151, 1969.
11. Katona C and Levy R (Editors), Delusions and Hallucinations in Old Age, Gaskell (Royal College of Psychiatrists), 1992.
12. Miller N and Cohen G (Editors), Schizophrenia and Aging, The Guilford Press (NIMH), 1987.
13. Sims A, Symptoms in the Mind: an introduction to descriptive psychopathology, Bailliere Tindall, 1988.
14. Schneider, K, Clinical Psychopathology, 5th Edition, Gune & Stratton, 1959.
15. Hicks D, Usher's syndrome: Programmatic considerations, American Annual of Deaf, 123:373-6, 1978.
16. Grassian S and Friedman N, Effects of Sensory Deprivation in Psychiatric Seclusion and Solitary Confinement, International Journal of Law and Psychiatry, 8:49-65, 1986.
17. Grassian S, Psychopathological Effects of Solitary Confinement, American Journal of Psychiatry, 140(1l):1450-4, 1983.
18. Miller N and Cohen G, Schizophrenia and Aging, The Guilford Press, 1987.
19. Nolte J, The Human Brain: an introduction to its functional anatomy, The CV Mosby Company, p 204, 1981.

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Citation suggestion: Dr Gary Galambos, A Case History of Sensory Deprivation, (http://www.ep.org.au/gg/int/sens_imp.htm) [date accessed]

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