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Schizophrenia-like psychosis and Dandy–Walker variant
Letters to the Editors / Schizophrenia Research 48 (2001) 361±370 Narinsky, E.R., Kutzuk, D., Weizman, A., 1998. HLA-B38 and clozapine-induced agranulocytosis in Israeli Jewish schizophrenic patients. Eur. J. Immunogenet. 25, 11±13.
M. Marchini R. Scorza R. Antonioli S. Scarone Department of Internal Medicine, University of Milan Medical School, IRCCS H. Maggiore Milan, Italy B. Grassi M. Epifani C. Dragoni Psychiatric Branch, Department of Medicine, Surgery and Dentistry, University of Milan Medical School H.S. Paolo, Italy
0920-9964/01/$ - see front matter q 2001 Elsevier Science B.V. All rights reserved. PII: S 0920-996 4(00)00105-5
Schizophrenia-like psychosis and Dandy±Walker variant Dear Editors Previous research has proposed a possible role for the cerebellum in cognition (Schmahmann, 1991) and in schizophrenia (Martin and Albers, 1995). Dandy± Walker variant is a developmental malformation consisting of cerebellar hypoplasia and cystic dilatation of the fourth ventricle. It is believed to occur between the 7th and 10th week of gestation (Raybaud, 1982). We report a psychotic episode and repeat neuropsychological testing in a patient with Dandy± Walker variant. The patient, C, a Caucasian woman, was 18 years old when she was admitted compulsorily for assessment in November 1996. Her family described an 18-month deterioration in social function. She had dropped out of academic studies and lost her job due to poor performance. Eight weeks before admission, she started to express odd beliefs that passers-by could read her mind. Her speech became illogical and she replied to her family when nothing had been said. Occasionally she `froze' climbing stairs, unable to go forwards or backwards. She became hostile, for example swearing
365
at her terminally ill grandmother. A general practitioner prescribed Fluoxetine, which was discontinued after she immediately developed a coarse tremor and drooling. Two days before admission, she barricaded herself in a bedroom, shouted abuse at her family and urinated on the ¯oor. She was one of three siblings brought up by her divorced mother. It had recently been disclosed that there had been sexual abuse in the family prior to the divorce. Development and education was normal. Family history of psychiatric disorder and medical history was unremarkable. She had occasionally used cannabis and LSD in the past, but denied using them in the three months prior to admission. On mental state examination she displayed ¯orid thought disorder, drivelling speech and incongruous affect. She was suspicious, expressed paranoid ideas about her family and insisted there was nothing wrong with her. Neurological examination was unremarkable. CT and NMR scanning showed a Dandy± Walker variant with hypoplasia of the cerebellar hemispheres and vermis (Fig. 1); there was no evidence of hydrocephalus nor supratentorial abnormalities. C settled but remained thought-disordered and appeared cognitively impaired. Neuropsychological testing (WAIS-R) ®ve weeks after admission showed she was functioning within the average range, but indicated slow information processing. She performed poorly on mental arithmetic and verbal memory, but well above average on visual memory. Copy of the ®gure of Rey was normal, but its poor recall indicated problems with delayed visual memory. On the Stroop Test (attentional allocation), she performed at the 25th percentile. She improved without medication and was discharged two months after admission. Testing was repeated eight months later. Performance IQ and full scale IQ had improved signi®cantly. She showed signi®cant improvements in speed of information processing, attentional allocation, verbal memory and delayed visual recall. This is the ®rst recorded case of psychosis in a person with a Dandy±Walker abnormality. Her neuropsychological abnormalities are similar to results found from the testing of groups of patients with cerebellar pathology. Schmahmann and Sherman (1998) found that characteristic abnormalities were: impairment of executive functions such as planning, setshifting, abstract reasoning and working memory; dif®culties with spatial cognition including visual-spatial
366
Letters to the Editors / Schizophrenia Research 48 (2001) 361±370
Fig. 1. Mid-sagittal MR scan of patient A, displaying cerebellar vermal hypoplasia and cystic dilatation of the fourth ventricle.
organisation and memory; personality change with blunting of affect or disinhibited and inappropriate behaviour. Other studies have found: de®cient visuospatial recall (Bracke-Tolkmitt et al., 1989), impaired voluntary attention shifting (Akshoomoff and Courchesne, 1992), reduced speed of information processing and poor mental arithmetic (Botez et al., 1989). Hamilton et al. (1983) described neuropsychological abnormalities in three patients with psychiatric illness and cerebellar lesions. These were: recent memory loss, poor concentration and impaired abstraction. The cognitive abnormalities in these studies, however, were persistent. This cerebellar case study is unique in having simultaneous transitory psychotic illness and cognitive abnormalities which resolved without treatment. Neuropsychological testing of patients with cerebellar pathology has not, however, produced consistent abnormalities; and has been weakened by the inclusion of patients with extra cerebellar damage (Schmahmann, 1991). It was also not possible to exclude the involvement of recreational drugs due to the patients refusal to give a urine sample. The occurrence of psychosis with a case of Dandy±
Walker variant may be due to chance. It is also compatible with the higher than expected rates of mid-line developmental abnormalities in schizophrenia including corpus callosum abnormalities and cavum septum pellucidum (Lewis, 1995). The rarity of the condition, the similar cognitive pro®le to populations with cerebellar pathology and the resolution of symptoms without treatment, however, are strongly suggestive of a direct relationship between this patient's mental illness and her cerebellar abnormality.
References Akshoomoff, N.A., Courchesne, E., 1992. A new role for the cerebellum in cognitive operations. Behav. Neurosci. 106, 731±738. Botez, M.I., Botez, T., Elie, E., et al., 1989. Role of the cerebellum in complex human behaviour. Ital. J. Neurol. Sci. 10, 291±300. Bracke-Tolkmitt, R., Linden, A., Canavan, A.G., et al., 1989. The cerebellum contributes to mental skills. Behav. Neurosci. 103, 442±446. Hamilton, N.G., Frick, R.B., Takahashi, T., 1983. Psychiatric symptoms and cerebellar pathology. Am. J. Psychiatry 140, 1322± 1326. Lewis, S.W., 1995. The secondary schizophrenias. In: Hirsh, S.,
Letters to the Editors / Schizophrenia Research 48 (2001) 361±370 Weinberger, D. (Eds.), Schizophrenia. Blackwell, Oxford, pp. 324±340. Martin, P., Albers, M., 1995. Cerebellum and schizophrenia: a selective review. Schizophr. Bull. 21, 241±250. Raybaud, C., 1982. Cystic malformations of the posterior fossa. J. Neuroradiology 9, 103±133. Schmahmann, J.D., 1991. An emerging concept. The cerebellar contribution to higher function. Arch. Neurol. 48, 1178±1185. Schmahmann, J.D., Sherman, J.C., 1998. The cerebellar cognitive affective syndrome. Brain 121, 561±579.
Simon J. Turner Central Manchester NHS Trust, Manchester Royal In®rmary, Manchester, UK Rob Poole Michael R. Nicholson North Mersey Community NHS Trust, Broad Oak Unit, Liverpool, UK Eric J. Ghadiali Walton Centre for Neurology and Neurosurgery NHS Trust, Liverpool, UK E-mail address: [email protected]
0920-9964/01/$ - see front matter q 2001 Elsevier Science B.V. All rights reserved. PII: S 0920-996 4(00)00048-7
Neurocognitive and social functioning schizophrenia and other diagnoses
in
Dear Editors, It has been well established that neurocognitive de®cits are a core feature of schizophrenia and are associated with impaired social functioning in individuals with schizophrenia. We wish to report on similar de®cits in people with other chronic mental disorders. In two comprehensive reviews of the topic, Green (Green, 1996; Green et al., 2000) concluded that there are significant associations between neurocognitive and functional outcome through a series of replicated ®ndings. In our own studies, we examined neurocognitive and social functioning in a sample of 80 stable outpatients with schizophrenia (Addington and Addington, 1999). The results were that social problem-solving, as assessed by the Assessment of Interpersonal Problem Solving Skills (AIPSS) (Donahoe et al., 1990), was associated with verbal ability, verbal memory, conceptual ¯exibility and vigilance.
367
With one exception (Dickerson et al., 1999), the majority of studies to date are cross-sectional. Our second study (Addington and Addington, in press), a longitudinal study of 65 of the 80 original subjects, demonstrated that the association between neurocognition at one point in time and social functioning assessed 2.5 years later remained consistent over time. As with earlier results, poor verbal ability and poor verbal memory were associated with current poor social problem-solving as assessed by the AIPSS. Thus, there is evidence that neurocognitive impairments are consistent predictors of poor social functioning in schizophrenia. One question is whether this relationship exists in other chronic psychiatric groups. To examine this, we recruited a sample of 13 males and 24 females from the same Day Program and outpatient program that our sample of individuals with schizophrenia were attending. These were all individuals with DSM-IV diagnoses, as assessed with the SCID that included bipolar disorder (21), major depression (11) and anxiety disorders (5). They were selected because they appeared to be functioning at a level similar to those with schizophrenia. Our criteria included repeated psychiatric admissions to hospital, functioning over the past 6 months at a lower level than they had been in the past and unable to work full-time therefore on social assistance. This psychiatric comparison group was signi®cantly older than the schizophrenia group (42 years vs. 36 years, P , 0.05); the group had a later age of onset of illness (29 years vs. 22 years, P , 0.05), less number of months since last psychiatric admission (43.2 vs. 19.7, P , 0.001) and a greater number of previous psychiatric admissions (7.4 vs. 4.5, P , 0.01) We compared these 37 psychiatric controls with the 80 schizophrenia subjects from our ®rst study (Addington and Addington, 1999). They were compared on the neurocognitive battery and on the three measures of social functioning Ð The Social Functioning Scale (SFS) (Birchwood et al., 1990), The Quality of Life Scale (QLS) (Heinrichs et al., 1984) and the AIPSS (Donahoe et al., 1990). The psychiatric control group performed signi®cantly better on all of the social functioning measures (see Table 1). Neurocognitive measures were grouped according to the two previous publications (verbal ability, verbal memory, visual memory, visual spatial
M. Marchini R. Scorza R. Antonioli S. Scarone Department of Internal Medicine, University of Milan Medical School, IRCCS H. Maggiore Milan, Italy B. Grassi M. Epifani C. Dragoni Psychiatric Branch, Department of Medicine, Surgery and Dentistry, University of Milan Medical School H.S. Paolo, Italy
0920-9964/01/$ - see front matter q 2001 Elsevier Science B.V. All rights reserved. PII: S 0920-996 4(00)00105-5
Schizophrenia-like psychosis and Dandy±Walker variant Dear Editors Previous research has proposed a possible role for the cerebellum in cognition (Schmahmann, 1991) and in schizophrenia (Martin and Albers, 1995). Dandy± Walker variant is a developmental malformation consisting of cerebellar hypoplasia and cystic dilatation of the fourth ventricle. It is believed to occur between the 7th and 10th week of gestation (Raybaud, 1982). We report a psychotic episode and repeat neuropsychological testing in a patient with Dandy± Walker variant. The patient, C, a Caucasian woman, was 18 years old when she was admitted compulsorily for assessment in November 1996. Her family described an 18-month deterioration in social function. She had dropped out of academic studies and lost her job due to poor performance. Eight weeks before admission, she started to express odd beliefs that passers-by could read her mind. Her speech became illogical and she replied to her family when nothing had been said. Occasionally she `froze' climbing stairs, unable to go forwards or backwards. She became hostile, for example swearing
365
at her terminally ill grandmother. A general practitioner prescribed Fluoxetine, which was discontinued after she immediately developed a coarse tremor and drooling. Two days before admission, she barricaded herself in a bedroom, shouted abuse at her family and urinated on the ¯oor. She was one of three siblings brought up by her divorced mother. It had recently been disclosed that there had been sexual abuse in the family prior to the divorce. Development and education was normal. Family history of psychiatric disorder and medical history was unremarkable. She had occasionally used cannabis and LSD in the past, but denied using them in the three months prior to admission. On mental state examination she displayed ¯orid thought disorder, drivelling speech and incongruous affect. She was suspicious, expressed paranoid ideas about her family and insisted there was nothing wrong with her. Neurological examination was unremarkable. CT and NMR scanning showed a Dandy± Walker variant with hypoplasia of the cerebellar hemispheres and vermis (Fig. 1); there was no evidence of hydrocephalus nor supratentorial abnormalities. C settled but remained thought-disordered and appeared cognitively impaired. Neuropsychological testing (WAIS-R) ®ve weeks after admission showed she was functioning within the average range, but indicated slow information processing. She performed poorly on mental arithmetic and verbal memory, but well above average on visual memory. Copy of the ®gure of Rey was normal, but its poor recall indicated problems with delayed visual memory. On the Stroop Test (attentional allocation), she performed at the 25th percentile. She improved without medication and was discharged two months after admission. Testing was repeated eight months later. Performance IQ and full scale IQ had improved signi®cantly. She showed signi®cant improvements in speed of information processing, attentional allocation, verbal memory and delayed visual recall. This is the ®rst recorded case of psychosis in a person with a Dandy±Walker abnormality. Her neuropsychological abnormalities are similar to results found from the testing of groups of patients with cerebellar pathology. Schmahmann and Sherman (1998) found that characteristic abnormalities were: impairment of executive functions such as planning, setshifting, abstract reasoning and working memory; dif®culties with spatial cognition including visual-spatial
366
Letters to the Editors / Schizophrenia Research 48 (2001) 361±370
Fig. 1. Mid-sagittal MR scan of patient A, displaying cerebellar vermal hypoplasia and cystic dilatation of the fourth ventricle.
organisation and memory; personality change with blunting of affect or disinhibited and inappropriate behaviour. Other studies have found: de®cient visuospatial recall (Bracke-Tolkmitt et al., 1989), impaired voluntary attention shifting (Akshoomoff and Courchesne, 1992), reduced speed of information processing and poor mental arithmetic (Botez et al., 1989). Hamilton et al. (1983) described neuropsychological abnormalities in three patients with psychiatric illness and cerebellar lesions. These were: recent memory loss, poor concentration and impaired abstraction. The cognitive abnormalities in these studies, however, were persistent. This cerebellar case study is unique in having simultaneous transitory psychotic illness and cognitive abnormalities which resolved without treatment. Neuropsychological testing of patients with cerebellar pathology has not, however, produced consistent abnormalities; and has been weakened by the inclusion of patients with extra cerebellar damage (Schmahmann, 1991). It was also not possible to exclude the involvement of recreational drugs due to the patients refusal to give a urine sample. The occurrence of psychosis with a case of Dandy±
Walker variant may be due to chance. It is also compatible with the higher than expected rates of mid-line developmental abnormalities in schizophrenia including corpus callosum abnormalities and cavum septum pellucidum (Lewis, 1995). The rarity of the condition, the similar cognitive pro®le to populations with cerebellar pathology and the resolution of symptoms without treatment, however, are strongly suggestive of a direct relationship between this patient's mental illness and her cerebellar abnormality.
References Akshoomoff, N.A., Courchesne, E., 1992. A new role for the cerebellum in cognitive operations. Behav. Neurosci. 106, 731±738. Botez, M.I., Botez, T., Elie, E., et al., 1989. Role of the cerebellum in complex human behaviour. Ital. J. Neurol. Sci. 10, 291±300. Bracke-Tolkmitt, R., Linden, A., Canavan, A.G., et al., 1989. The cerebellum contributes to mental skills. Behav. Neurosci. 103, 442±446. Hamilton, N.G., Frick, R.B., Takahashi, T., 1983. Psychiatric symptoms and cerebellar pathology. Am. J. Psychiatry 140, 1322± 1326. Lewis, S.W., 1995. The secondary schizophrenias. In: Hirsh, S.,
Letters to the Editors / Schizophrenia Research 48 (2001) 361±370 Weinberger, D. (Eds.), Schizophrenia. Blackwell, Oxford, pp. 324±340. Martin, P., Albers, M., 1995. Cerebellum and schizophrenia: a selective review. Schizophr. Bull. 21, 241±250. Raybaud, C., 1982. Cystic malformations of the posterior fossa. J. Neuroradiology 9, 103±133. Schmahmann, J.D., 1991. An emerging concept. The cerebellar contribution to higher function. Arch. Neurol. 48, 1178±1185. Schmahmann, J.D., Sherman, J.C., 1998. The cerebellar cognitive affective syndrome. Brain 121, 561±579.
Simon J. Turner Central Manchester NHS Trust, Manchester Royal In®rmary, Manchester, UK Rob Poole Michael R. Nicholson North Mersey Community NHS Trust, Broad Oak Unit, Liverpool, UK Eric J. Ghadiali Walton Centre for Neurology and Neurosurgery NHS Trust, Liverpool, UK E-mail address: [email protected]
0920-9964/01/$ - see front matter q 2001 Elsevier Science B.V. All rights reserved. PII: S 0920-996 4(00)00048-7
Neurocognitive and social functioning schizophrenia and other diagnoses
in
Dear Editors, It has been well established that neurocognitive de®cits are a core feature of schizophrenia and are associated with impaired social functioning in individuals with schizophrenia. We wish to report on similar de®cits in people with other chronic mental disorders. In two comprehensive reviews of the topic, Green (Green, 1996; Green et al., 2000) concluded that there are significant associations between neurocognitive and functional outcome through a series of replicated ®ndings. In our own studies, we examined neurocognitive and social functioning in a sample of 80 stable outpatients with schizophrenia (Addington and Addington, 1999). The results were that social problem-solving, as assessed by the Assessment of Interpersonal Problem Solving Skills (AIPSS) (Donahoe et al., 1990), was associated with verbal ability, verbal memory, conceptual ¯exibility and vigilance.
367
With one exception (Dickerson et al., 1999), the majority of studies to date are cross-sectional. Our second study (Addington and Addington, in press), a longitudinal study of 65 of the 80 original subjects, demonstrated that the association between neurocognition at one point in time and social functioning assessed 2.5 years later remained consistent over time. As with earlier results, poor verbal ability and poor verbal memory were associated with current poor social problem-solving as assessed by the AIPSS. Thus, there is evidence that neurocognitive impairments are consistent predictors of poor social functioning in schizophrenia. One question is whether this relationship exists in other chronic psychiatric groups. To examine this, we recruited a sample of 13 males and 24 females from the same Day Program and outpatient program that our sample of individuals with schizophrenia were attending. These were all individuals with DSM-IV diagnoses, as assessed with the SCID that included bipolar disorder (21), major depression (11) and anxiety disorders (5). They were selected because they appeared to be functioning at a level similar to those with schizophrenia. Our criteria included repeated psychiatric admissions to hospital, functioning over the past 6 months at a lower level than they had been in the past and unable to work full-time therefore on social assistance. This psychiatric comparison group was signi®cantly older than the schizophrenia group (42 years vs. 36 years, P , 0.05); the group had a later age of onset of illness (29 years vs. 22 years, P , 0.05), less number of months since last psychiatric admission (43.2 vs. 19.7, P , 0.001) and a greater number of previous psychiatric admissions (7.4 vs. 4.5, P , 0.01) We compared these 37 psychiatric controls with the 80 schizophrenia subjects from our ®rst study (Addington and Addington, 1999). They were compared on the neurocognitive battery and on the three measures of social functioning Ð The Social Functioning Scale (SFS) (Birchwood et al., 1990), The Quality of Life Scale (QLS) (Heinrichs et al., 1984) and the AIPSS (Donahoe et al., 1990). The psychiatric control group performed signi®cantly better on all of the social functioning measures (see Table 1). Neurocognitive measures were grouped according to the two previous publications (verbal ability, verbal memory, visual memory, visual spatial