- Email: [email protected]
A Case of Cerebellar Psychopathology
A Case of Cerebellar Psychopathology Amy Rosinski, M.D. Mona Goldman, Ph.D. Oliver Cameron, M.D., Ph.D.
Background: Pathology of the cerebellum has traditionally been associated with motor symptoms, vertigo, and nystagmus. Patients with cerebellar disorders do not usually receive psychiatric evaluations. Objective: The authors seek to alert clinicians to the association between cerebellar disease and psychiatric symptoms. Method: The authors describe a patient with uncommon psychiatric morbidity associated with cerebellar dysfunction, and provide a brief review of previous research on this phenomenon. Results: Neurology consultants suggested that physical exam findings and behavioral changes could be accounted for by cerebellar cognitive affective syndrome. This syndrome involves dysfunction of the cerebellum, including classic cerebellar findings, in addition to cognitive difficulties and affective/personality changes. Conclusion: The suspected etiology was post-infectious cerebellitis from Epstein-Barr virus infection. (Psychosomatics 2010; 51:171–175)
Case Report
T
he patient, “Ms. C,” was an 18-year-old woman with no previous psychiatric history, and a medical history only for migraines. She went on a camping trip to Northern Michigan, returned 4 days later, and started having headache, nausea, vomiting, and vertigo. Her primary-care physician prescribed Augmentin (amoxicillin and clavulanate potassium) for a presumptive ear/sinus infection. Two weeks later, she was hospitalized for continuing nausea and vertigo, and was diagnosed with Rocky Mountain spotted fever (RMSF) on the basis of a positive RMSF IgM test. She also had a positive Monospot test, and Epstein-Barr virus IgG and IgM. She was afebrile, had a normal white blood cell count, no lymphadenopathy, no pharyngitis, no rash, and no cough. She was unaware of recent tick bites. She was treated with doxycycline for presumed RMSF, and acyclovir and methylprednisolone (Solu-Medrol) for possible vestibular neuritis or labyrinthitis. A head CT and MRI of the head and auditory canal
Psychosomatics 51:2, March-April 2010
were normal. Lumbar puncture was normal, with no oligoclonal bands. Serum ANA, TSH, SPEP, hepatitis B and C virus, cytomegalovirus, Lyme antibody, and HIV tests were negative. Seizure activity was denied. After 1 week at that hospital, the patient’s parents observed a substantial change in her personality. They described her as withdrawn, talking minimally, and speaking in a quiet voice. Because her symptoms were not improving, her parents requested a transfer to the University of Michigan Hospital. Admission labs were significant only for a urinary tract infection and an elevated ALT, at 87 (normal: 7–35). Acyclovir and methylprednisolone were not restarted. Doxycycline was stopped because the RMSF IgM was considered a false positive, since her clinical picture was not consistent with RMSF. Her symptoms were Received August 6, 2008; revised January 21, 2009; accepted January 22, 2009. From the Dept. of Psychiatry, School of Medicine, University of Michigan, Ann Arbor, MI. Send correspondence and reprint requests to Amy Rosinski, M.D., Dept. of Psychiatry, School of Medicine, University of Michigan, Ann Arbor, MI. e-mail: [email protected] © 2010 The Academy of Psychosomatic Medicine
http://psy.psychiatryonline.org
171
Case Reports felt to be consistent with acute vestibular neuritis or labyrinthitis, possibly due to Epstein-Barr virus (EBV). EBV PCR was positive. Otolaryngology did not find evidence of peripheral vestibulopathy. Cerebellar pathology was suspected because of saccadic smooth pursuit, slow/rapid alternating movements, and unsteadiness with Romberg testing, and it was confirmed by vestibular testing. During her hospitalization, the patient continued to exhibit a flat affect, atypical for her. She answered questions with one word at most, and her speech was quiet and dysprosodic. This behavior prompted a psychiatric consult. On psychiatric evaluation, the patient denied feeling sad or depressed. Her parents described her as “bubbly” and active before her illness. She denied problems with concentration, guilt, or sleep. Appetite was diminished because of nausea, and she was fatigued. She had no thoughts of self-harm. She and her family denied any stressful recent events or a history of trauma or assault. She had no history of substance abuse. Ms. C denied delusional symptoms or hallucinations. Her speech had a high-pitched, child-like prosody and was slow, with minimal spontaneous speech. She showed psychomotor slowing and little range of facial expression. She was distrustful of staff, and her affect was blunted. She made comments about the doctors being “mean” for doing “all those tests.” Ms. C’s thought process was logical, although concrete, without much detail in her responses. Formal bedside cognitive tests were not performed because of her unwillingness to cooperate. She was alert, attentive, and oriented, without evidence of delirium. Ms. C had poor insight into the need for medical tests. She gave the impression of being younger than her 18 years. She clearly had had a change from her premorbid personality. We considered that this might be due to recent methylprednisolone administration, which had been discontinued a few days before psychiatric evaluation. Serial visits were planned to observe her behavior further. Given Ms. C’s cerebellar findings, the neurology department was consulted. The neurologic exam showed saccadic intrusions on extraocular muscle testing. She had bilateral dysmetria on finger–nose–finger and heel– knee– shin testing. She had a narrow-based, shuffling gait, and could not stand on either foot for a full second. The remainder of her exam was normal. Neurology consultants suggested that her physical exam findings and behavioral changes could be accounted for by cerebellar cognitive 172
http://psy.psychiatryonline.org
affective syndrome. This syndrome involves dysfunction of the cerebellum, including classic cerebellar findings, in addition to cognitive difficulties and affective/personality changes,1 and the suspected etiology was post-infectious cerebellitis from Epstein-Barr virus. Given the autoimmune nature of post-infectious cerebellitis, plasmapheresis or intravenous immunoglobulin (IVIG) are possible treatments,2 but these were deferred because the patient was improving. Ms. C’s nausea and vertigo resolved, and she was discharged home. Speech became more spontaneous, and she had more energy. She had a visit in the neurology clinic 3 weeks after hospital discharge and was doing well. Speech was fluent; affect was bright and reactive; and she had minimal evidence of ataxia or dysmetria. Given her resolution of symptoms, no further psychiatric evaluation was performed.
Discussion
Anatomic and physiologic studies of the brain have shown that the cerebellum does not function in isolation from other brain structures. Reciprocal connections exist between the cerebellar cortex and the parietal and prefrontal lobes, the hypothalamus, and the limbic system (Figure 1; adapted from Leiner et al.3). Evidence of these connections comes from functional neuroimaging studies, which have shown cerebellar activation during tests of language function, executive function (verbal working memory, strategy, reasoning, and verbal fluency), classical conditioning, mental imagery, shifting attention, sensory discrimination, and emotional perception and experience.4 Electrical stimulation of certain areas of the cerebellum has also led to the production of distinct emotions, such as fear.5 The role of the cerebellum in psychiatric disorders has been studied via structural and functional neuroimaging. Multiple MRI studies of schizophrenia patients have shown cerebellar atrophy, with some authors noting particular atrophy of the vermis.4,6 Some have questioned whether atrophy is related to neuroleptic use, although significantly reduced vermis (midline) volume was also reported in neuroleptic-naive schizophrenic patients.7 Functional-imaging studies in schizophrenic patients have also shown decreased activation of the cerebellum during a variety of cognitive tasks.4 There are fewer studies of the cerebellum in unipolar depression and bipolar disorder, Psychosomatics 51:2, March-April 2010
Rosinski et al.
FIGURE 1.
Cerebellar–Cortical Connections (adapted from Leiner et al.3; used by permission) Cerebral Cortex Limbic Structures
Hypothalamus
Neocortical Structures
Reticular Nuclei
Red Nucleus
Brainstem
Inferior Olive
Pontine Nuclei
Thalamus
Cerebellum
but evidence does point to disruption in cerebellar functioning, and possibly size, in these disorders.8 –10 Anatomic abnormality of the cerebellum has been found in the brains of patients with autism11 and in patients with attention-deficit hyperactivity disorder.12,13 Schmahmann and Sherman1 examined the clinical relevance of cerebellar dysfunction in 20 patients with a variety of cerebellar lesions that included pancerebellar processes such as post-infectious cerebellitis and cortical atrophy, as well as more circumscribed lesions occurring after stroke. Bedside neurologic and mentalstatus testing was done, as was neuropsychologic testing. Results showed that patients with cerebellar pathology shared a constellation of deficits that included impairments in executive function (planning, set-shifting, working memory, verbal fluency, abstraction), visuospatial skills, language production (agrammatism and anomia), and attention. Also, personality change was often seen, with either blunting of affect or inappropriate/disinhibited behavior. This pattern of deficits was labeled the cerebellar cognitive affective syndrome (CCAS). The investigators further conceptualized a “universal cerebellar transform,” whereby the cerebellum acts to modulate not only motor activity, but also cognition, affect, and other higher-order processes. They hypothesized that disruption in this process leads to a “dysmetria of thought,” often in addition to dysmetria of motor function.4 (See Table 1 for a description of common cerebellar syndromes, including CCAS. Since publication of those results in 1998, CCAS has been observed in other patient populations, and with different types of cerebellar insults; for example, in children who have undergone resection of cerebellar tumors,14 in children and adults with congenital cerebellar malformations,15 and in a patient with sporadic olivopontocerebellar Psychosomatics 51:2, March-April 2010
atrophy, a degenerative disease of the cerebellum and pons associated with dementia.16 Of particular interest to psychiatrists are the affective/ behavioral changes seen in CCAS, because these changes may prompt a psychiatric consultation to assess for a primary psychiatric disorder. Some patients may show evidence of disinhibition, with impulsive actions, overfamiliarity, and inappropriate comments. Regressed or childlike behavior has also been reported. Others may show more symptoms of apathy.1,4 Schmahmann and Sherman noted involvement of the vermis in the majority of patients with predominant affective symptoms of CCAS, whereas lesions of the posterior lobe resulted in more cognitive dysfunction.1 Cerebellar cognitive affective syndrome is not a readily recognized clinical syndrome for most psychiatrists. In our consultation with this patient, however, awareness of the syndrome was important. Treatment of the patient with psychotropic medication for a primary psychiatric disorder was avoided, and she returned to her normal premorbid behavioral condition with resolution of her cerebellar insult. Psychiatrists should thus include this syndrome in the differential diagnosis of patients with cerebellar injury/disease and accompanying affective, behavioral, speech, and cognitive changes. A neurologic exam testing cerebellar functioning should be helpful in patients with unexplained affective changes that do not match depression or other psychiatric disorders. Testing of higher-level executive function tasks may also help diagnose the syndrome, and neuroimaging should be considered to look for structural insults to the cerebellum. Little has been written about treatment options for CCAS. Our patient’s symptoms resolved over time without intervention. Other cases of Epstein-Barr–inhttp://psy.psychiatryonline.org
173
Case Reports
TABLE 1.
Common Cerebellar Syndromes
Type of Insult Toxic (alcohol, organic solvents, heavy metals, phenytoin, chemotherapy, lithium) Multiple sclerosis
Hemorrhage
Location of Insult in Cerebellum Most toxins cause atrophy of cerebellar hemispheres. Chronic alcohol use 3 vermal atrophy. Cerebellar connections in the brainstem, especially the middle cerebellar peduncle. Can be hemispheric or vermal.
Timing
Signs and Symptoms ● Hemispheric atrophy 3 limb ataxia, dysmetria, horizontal nystagmus. Vermal atrophy 3 truncal ataxia. ● Ipsilateral limb ataxia, nystagmus, dysarthria, hearing loss.
Gradual progression over months.
Acute onset
● Hemispheric/vermal symptoms as above depending on location. ● Can have signs of increased intracranial pressure such as headache, nausea/vomiting, decreased level of consciousness. ● Same as hemorrhage above.
Acute onset
Tumor (astrocytoma, primary neuroectodermal, medulloblastoma, ependymoma, hemangioblastoma, dermoid, metastasis) Hereditary ataxias (spinocerebellar ataxias, Friedrich’s ataxia)
Variable
Gradual progression over months
Variable: atrophy can occur globally or in specific regions.
Gradual progression, beginning in childhood (Friedrich’s ataxia) or adulthood.
Cerebellar Cognitive Affective Syndrome: results from a variety of cerebellar insults, including the above.
Bilateral hemispheric, pancerebellar, or large lesions are more likely to cause CCAS.
Acute-onset insults more likely to result in CCAS.
duced post-infectious cerebellitis have been treated successfully with plasmapheresis or IVIG.2 Schmahmann and Sherman did repeat neuropsychological evaluation in four patients with acute cerebellar injury (stroke or cerebellitis), with improvement found in multiple do-
● Mental retardation may be present. ● Can be differentiated from each other by other nervous system involvement. ● Disturbance of executive function ● Impaired spatial cognition ● Personality change ● Disturbed language production ● Affective changes (usually from vermal lesions)
mains over time. One patient with cerebellar cortical atrophy worsened over time during follow-up testing.1 Acute injuries would thus seem to have a more favorable prognosis. Treatment of the syndrome is highly related to resolution of the underlying cerebellar insult.
References 1. Schmahmann JD, Sherman JC: The cerebellar cognitive affective syndrome. Brain 1998; 121:561–579 2. Schmahmann JD: Plasmapheresis improves outcome in children with post-infectious cerebellitis induced by Epstein-Barr virus. Neurology 2004; 62:1443 3. Leiner HC, Leiner AL, Dow RS: The human cerebro-cerebellar system: its computing, cognitive, and language skills. Behav Brain Res 1991; 44:113–128 4. Schmahmann JD: Disorders of the cerebellum: ataxia, dysmetria of thought, and the cerebellar cognitive affective syndrome. J Neuropsychiatry Clin Neurosci 2004; 16:367–376 5. Nashold BS Jr, Slaughter DG: Effects of stimulating or destroying the deep cerebellar regions in man. J Neurosurg 1969; 31: 172–186
174
http://psy.psychiatryonline.org
6. Konarski JZ, McIntyre RS, Grupp LA, et al: Is the cerebellum relevant in the circuitry of neuropsychiatric disorders? J Psychiatry Neurosci 2005; 30:178 –186 7. Ichimiya T, Okubo Y, Suhara T, et al. Reduced volume of the cerebellar vermis in neuroleptic-naive schizophrenia. Biol Psychiatry 2001; 49:20 –27 8. Loeber RT, Sherwood AR, Renshaw PF, et al: Differences in cerebellar blood volume in schizophrenia and bipolar disorder. Schizophr Res 1999; 37:81– 89 9. Beauregard M, Leroux JM, Bergman S, et al: The functional neuroanatomy of major depression: an fMRI study using an emotional activation paradigm. Neuroreport 1998; 9:3253–328 10. Liotti M, Mayberg HS, McGinnis S, et al: Unmasking diseasespecific cerebral blood flow abnormalities: mood challenge in
Psychosomatics 51:2, March-April 2010
Rosinski et al. patients with remitted unipolar depression. Am J Psychiatry 2002; 159:1830 –1840 11. Bauman ML, Kemper TL: Neuroanatomic observations of the brain in autism. Int J Dev Neurosci 2005; 23:183–187 12. Castellanos FX, Giedd JN, Berquin PC, et al: Quantitative brain magnetic resonance imaging in girls with attention-deficit/hyperactivity disorder. Arch Gen Psychiatry 2001; 58:289 –295 13. Berquin PC, Giedd JN, Jacobsen LK, et al: Cerebellum in attention-deficit hyperactivity disorder: a morphometric MRI study. Neurology 1998; 50:1087–1093
Psychosomatics 51:2, March-April 2010
14. Levisohn L, Cronin-Golomb A, Schmahmann JD: Neuropsychological consequences of cerebellar tumor resection in children: cerebellar cognitive affective syndrome in a paediatric population. Brain 2000; 123:1041–1050 15. Tavano A, Grasso R, Gagliardi C, et al: Disorders of cognitive and affective development in cerebellar malformations. Brain 2007; 130:2646 –2660 16. Duggal HS: Cognitive affective psychosis syndrome in a patient with sporadic olivopontocerebellar atrophy. J Neuropsychiatry Clin Neurosci 2005; 17:260 –262
http://psy.psychiatryonline.org
175
Background: Pathology of the cerebellum has traditionally been associated with motor symptoms, vertigo, and nystagmus. Patients with cerebellar disorders do not usually receive psychiatric evaluations. Objective: The authors seek to alert clinicians to the association between cerebellar disease and psychiatric symptoms. Method: The authors describe a patient with uncommon psychiatric morbidity associated with cerebellar dysfunction, and provide a brief review of previous research on this phenomenon. Results: Neurology consultants suggested that physical exam findings and behavioral changes could be accounted for by cerebellar cognitive affective syndrome. This syndrome involves dysfunction of the cerebellum, including classic cerebellar findings, in addition to cognitive difficulties and affective/personality changes. Conclusion: The suspected etiology was post-infectious cerebellitis from Epstein-Barr virus infection. (Psychosomatics 2010; 51:171–175)
Case Report
T
he patient, “Ms. C,” was an 18-year-old woman with no previous psychiatric history, and a medical history only for migraines. She went on a camping trip to Northern Michigan, returned 4 days later, and started having headache, nausea, vomiting, and vertigo. Her primary-care physician prescribed Augmentin (amoxicillin and clavulanate potassium) for a presumptive ear/sinus infection. Two weeks later, she was hospitalized for continuing nausea and vertigo, and was diagnosed with Rocky Mountain spotted fever (RMSF) on the basis of a positive RMSF IgM test. She also had a positive Monospot test, and Epstein-Barr virus IgG and IgM. She was afebrile, had a normal white blood cell count, no lymphadenopathy, no pharyngitis, no rash, and no cough. She was unaware of recent tick bites. She was treated with doxycycline for presumed RMSF, and acyclovir and methylprednisolone (Solu-Medrol) for possible vestibular neuritis or labyrinthitis. A head CT and MRI of the head and auditory canal
Psychosomatics 51:2, March-April 2010
were normal. Lumbar puncture was normal, with no oligoclonal bands. Serum ANA, TSH, SPEP, hepatitis B and C virus, cytomegalovirus, Lyme antibody, and HIV tests were negative. Seizure activity was denied. After 1 week at that hospital, the patient’s parents observed a substantial change in her personality. They described her as withdrawn, talking minimally, and speaking in a quiet voice. Because her symptoms were not improving, her parents requested a transfer to the University of Michigan Hospital. Admission labs were significant only for a urinary tract infection and an elevated ALT, at 87 (normal: 7–35). Acyclovir and methylprednisolone were not restarted. Doxycycline was stopped because the RMSF IgM was considered a false positive, since her clinical picture was not consistent with RMSF. Her symptoms were Received August 6, 2008; revised January 21, 2009; accepted January 22, 2009. From the Dept. of Psychiatry, School of Medicine, University of Michigan, Ann Arbor, MI. Send correspondence and reprint requests to Amy Rosinski, M.D., Dept. of Psychiatry, School of Medicine, University of Michigan, Ann Arbor, MI. e-mail: [email protected] © 2010 The Academy of Psychosomatic Medicine
http://psy.psychiatryonline.org
171
Case Reports felt to be consistent with acute vestibular neuritis or labyrinthitis, possibly due to Epstein-Barr virus (EBV). EBV PCR was positive. Otolaryngology did not find evidence of peripheral vestibulopathy. Cerebellar pathology was suspected because of saccadic smooth pursuit, slow/rapid alternating movements, and unsteadiness with Romberg testing, and it was confirmed by vestibular testing. During her hospitalization, the patient continued to exhibit a flat affect, atypical for her. She answered questions with one word at most, and her speech was quiet and dysprosodic. This behavior prompted a psychiatric consult. On psychiatric evaluation, the patient denied feeling sad or depressed. Her parents described her as “bubbly” and active before her illness. She denied problems with concentration, guilt, or sleep. Appetite was diminished because of nausea, and she was fatigued. She had no thoughts of self-harm. She and her family denied any stressful recent events or a history of trauma or assault. She had no history of substance abuse. Ms. C denied delusional symptoms or hallucinations. Her speech had a high-pitched, child-like prosody and was slow, with minimal spontaneous speech. She showed psychomotor slowing and little range of facial expression. She was distrustful of staff, and her affect was blunted. She made comments about the doctors being “mean” for doing “all those tests.” Ms. C’s thought process was logical, although concrete, without much detail in her responses. Formal bedside cognitive tests were not performed because of her unwillingness to cooperate. She was alert, attentive, and oriented, without evidence of delirium. Ms. C had poor insight into the need for medical tests. She gave the impression of being younger than her 18 years. She clearly had had a change from her premorbid personality. We considered that this might be due to recent methylprednisolone administration, which had been discontinued a few days before psychiatric evaluation. Serial visits were planned to observe her behavior further. Given Ms. C’s cerebellar findings, the neurology department was consulted. The neurologic exam showed saccadic intrusions on extraocular muscle testing. She had bilateral dysmetria on finger–nose–finger and heel– knee– shin testing. She had a narrow-based, shuffling gait, and could not stand on either foot for a full second. The remainder of her exam was normal. Neurology consultants suggested that her physical exam findings and behavioral changes could be accounted for by cerebellar cognitive 172
http://psy.psychiatryonline.org
affective syndrome. This syndrome involves dysfunction of the cerebellum, including classic cerebellar findings, in addition to cognitive difficulties and affective/personality changes,1 and the suspected etiology was post-infectious cerebellitis from Epstein-Barr virus. Given the autoimmune nature of post-infectious cerebellitis, plasmapheresis or intravenous immunoglobulin (IVIG) are possible treatments,2 but these were deferred because the patient was improving. Ms. C’s nausea and vertigo resolved, and she was discharged home. Speech became more spontaneous, and she had more energy. She had a visit in the neurology clinic 3 weeks after hospital discharge and was doing well. Speech was fluent; affect was bright and reactive; and she had minimal evidence of ataxia or dysmetria. Given her resolution of symptoms, no further psychiatric evaluation was performed.
Discussion
Anatomic and physiologic studies of the brain have shown that the cerebellum does not function in isolation from other brain structures. Reciprocal connections exist between the cerebellar cortex and the parietal and prefrontal lobes, the hypothalamus, and the limbic system (Figure 1; adapted from Leiner et al.3). Evidence of these connections comes from functional neuroimaging studies, which have shown cerebellar activation during tests of language function, executive function (verbal working memory, strategy, reasoning, and verbal fluency), classical conditioning, mental imagery, shifting attention, sensory discrimination, and emotional perception and experience.4 Electrical stimulation of certain areas of the cerebellum has also led to the production of distinct emotions, such as fear.5 The role of the cerebellum in psychiatric disorders has been studied via structural and functional neuroimaging. Multiple MRI studies of schizophrenia patients have shown cerebellar atrophy, with some authors noting particular atrophy of the vermis.4,6 Some have questioned whether atrophy is related to neuroleptic use, although significantly reduced vermis (midline) volume was also reported in neuroleptic-naive schizophrenic patients.7 Functional-imaging studies in schizophrenic patients have also shown decreased activation of the cerebellum during a variety of cognitive tasks.4 There are fewer studies of the cerebellum in unipolar depression and bipolar disorder, Psychosomatics 51:2, March-April 2010
Rosinski et al.
FIGURE 1.
Cerebellar–Cortical Connections (adapted from Leiner et al.3; used by permission) Cerebral Cortex Limbic Structures
Hypothalamus
Neocortical Structures
Reticular Nuclei
Red Nucleus
Brainstem
Inferior Olive
Pontine Nuclei
Thalamus
Cerebellum
but evidence does point to disruption in cerebellar functioning, and possibly size, in these disorders.8 –10 Anatomic abnormality of the cerebellum has been found in the brains of patients with autism11 and in patients with attention-deficit hyperactivity disorder.12,13 Schmahmann and Sherman1 examined the clinical relevance of cerebellar dysfunction in 20 patients with a variety of cerebellar lesions that included pancerebellar processes such as post-infectious cerebellitis and cortical atrophy, as well as more circumscribed lesions occurring after stroke. Bedside neurologic and mentalstatus testing was done, as was neuropsychologic testing. Results showed that patients with cerebellar pathology shared a constellation of deficits that included impairments in executive function (planning, set-shifting, working memory, verbal fluency, abstraction), visuospatial skills, language production (agrammatism and anomia), and attention. Also, personality change was often seen, with either blunting of affect or inappropriate/disinhibited behavior. This pattern of deficits was labeled the cerebellar cognitive affective syndrome (CCAS). The investigators further conceptualized a “universal cerebellar transform,” whereby the cerebellum acts to modulate not only motor activity, but also cognition, affect, and other higher-order processes. They hypothesized that disruption in this process leads to a “dysmetria of thought,” often in addition to dysmetria of motor function.4 (See Table 1 for a description of common cerebellar syndromes, including CCAS. Since publication of those results in 1998, CCAS has been observed in other patient populations, and with different types of cerebellar insults; for example, in children who have undergone resection of cerebellar tumors,14 in children and adults with congenital cerebellar malformations,15 and in a patient with sporadic olivopontocerebellar Psychosomatics 51:2, March-April 2010
atrophy, a degenerative disease of the cerebellum and pons associated with dementia.16 Of particular interest to psychiatrists are the affective/ behavioral changes seen in CCAS, because these changes may prompt a psychiatric consultation to assess for a primary psychiatric disorder. Some patients may show evidence of disinhibition, with impulsive actions, overfamiliarity, and inappropriate comments. Regressed or childlike behavior has also been reported. Others may show more symptoms of apathy.1,4 Schmahmann and Sherman noted involvement of the vermis in the majority of patients with predominant affective symptoms of CCAS, whereas lesions of the posterior lobe resulted in more cognitive dysfunction.1 Cerebellar cognitive affective syndrome is not a readily recognized clinical syndrome for most psychiatrists. In our consultation with this patient, however, awareness of the syndrome was important. Treatment of the patient with psychotropic medication for a primary psychiatric disorder was avoided, and she returned to her normal premorbid behavioral condition with resolution of her cerebellar insult. Psychiatrists should thus include this syndrome in the differential diagnosis of patients with cerebellar injury/disease and accompanying affective, behavioral, speech, and cognitive changes. A neurologic exam testing cerebellar functioning should be helpful in patients with unexplained affective changes that do not match depression or other psychiatric disorders. Testing of higher-level executive function tasks may also help diagnose the syndrome, and neuroimaging should be considered to look for structural insults to the cerebellum. Little has been written about treatment options for CCAS. Our patient’s symptoms resolved over time without intervention. Other cases of Epstein-Barr–inhttp://psy.psychiatryonline.org
173
Case Reports
TABLE 1.
Common Cerebellar Syndromes
Type of Insult Toxic (alcohol, organic solvents, heavy metals, phenytoin, chemotherapy, lithium) Multiple sclerosis
Hemorrhage
Location of Insult in Cerebellum Most toxins cause atrophy of cerebellar hemispheres. Chronic alcohol use 3 vermal atrophy. Cerebellar connections in the brainstem, especially the middle cerebellar peduncle. Can be hemispheric or vermal.
Timing
Signs and Symptoms ● Hemispheric atrophy 3 limb ataxia, dysmetria, horizontal nystagmus. Vermal atrophy 3 truncal ataxia. ● Ipsilateral limb ataxia, nystagmus, dysarthria, hearing loss.
Gradual progression over months.
Acute onset
● Hemispheric/vermal symptoms as above depending on location. ● Can have signs of increased intracranial pressure such as headache, nausea/vomiting, decreased level of consciousness. ● Same as hemorrhage above.
Acute onset
Tumor (astrocytoma, primary neuroectodermal, medulloblastoma, ependymoma, hemangioblastoma, dermoid, metastasis) Hereditary ataxias (spinocerebellar ataxias, Friedrich’s ataxia)
Variable
Gradual progression over months
Variable: atrophy can occur globally or in specific regions.
Gradual progression, beginning in childhood (Friedrich’s ataxia) or adulthood.
Cerebellar Cognitive Affective Syndrome: results from a variety of cerebellar insults, including the above.
Bilateral hemispheric, pancerebellar, or large lesions are more likely to cause CCAS.
Acute-onset insults more likely to result in CCAS.
duced post-infectious cerebellitis have been treated successfully with plasmapheresis or IVIG.2 Schmahmann and Sherman did repeat neuropsychological evaluation in four patients with acute cerebellar injury (stroke or cerebellitis), with improvement found in multiple do-
● Mental retardation may be present. ● Can be differentiated from each other by other nervous system involvement. ● Disturbance of executive function ● Impaired spatial cognition ● Personality change ● Disturbed language production ● Affective changes (usually from vermal lesions)
mains over time. One patient with cerebellar cortical atrophy worsened over time during follow-up testing.1 Acute injuries would thus seem to have a more favorable prognosis. Treatment of the syndrome is highly related to resolution of the underlying cerebellar insult.
References 1. Schmahmann JD, Sherman JC: The cerebellar cognitive affective syndrome. Brain 1998; 121:561–579 2. Schmahmann JD: Plasmapheresis improves outcome in children with post-infectious cerebellitis induced by Epstein-Barr virus. Neurology 2004; 62:1443 3. Leiner HC, Leiner AL, Dow RS: The human cerebro-cerebellar system: its computing, cognitive, and language skills. Behav Brain Res 1991; 44:113–128 4. Schmahmann JD: Disorders of the cerebellum: ataxia, dysmetria of thought, and the cerebellar cognitive affective syndrome. J Neuropsychiatry Clin Neurosci 2004; 16:367–376 5. Nashold BS Jr, Slaughter DG: Effects of stimulating or destroying the deep cerebellar regions in man. J Neurosurg 1969; 31: 172–186
174
http://psy.psychiatryonline.org
6. Konarski JZ, McIntyre RS, Grupp LA, et al: Is the cerebellum relevant in the circuitry of neuropsychiatric disorders? J Psychiatry Neurosci 2005; 30:178 –186 7. Ichimiya T, Okubo Y, Suhara T, et al. Reduced volume of the cerebellar vermis in neuroleptic-naive schizophrenia. Biol Psychiatry 2001; 49:20 –27 8. Loeber RT, Sherwood AR, Renshaw PF, et al: Differences in cerebellar blood volume in schizophrenia and bipolar disorder. Schizophr Res 1999; 37:81– 89 9. Beauregard M, Leroux JM, Bergman S, et al: The functional neuroanatomy of major depression: an fMRI study using an emotional activation paradigm. Neuroreport 1998; 9:3253–328 10. Liotti M, Mayberg HS, McGinnis S, et al: Unmasking diseasespecific cerebral blood flow abnormalities: mood challenge in
Psychosomatics 51:2, March-April 2010
Rosinski et al. patients with remitted unipolar depression. Am J Psychiatry 2002; 159:1830 –1840 11. Bauman ML, Kemper TL: Neuroanatomic observations of the brain in autism. Int J Dev Neurosci 2005; 23:183–187 12. Castellanos FX, Giedd JN, Berquin PC, et al: Quantitative brain magnetic resonance imaging in girls with attention-deficit/hyperactivity disorder. Arch Gen Psychiatry 2001; 58:289 –295 13. Berquin PC, Giedd JN, Jacobsen LK, et al: Cerebellum in attention-deficit hyperactivity disorder: a morphometric MRI study. Neurology 1998; 50:1087–1093
Psychosomatics 51:2, March-April 2010
14. Levisohn L, Cronin-Golomb A, Schmahmann JD: Neuropsychological consequences of cerebellar tumor resection in children: cerebellar cognitive affective syndrome in a paediatric population. Brain 2000; 123:1041–1050 15. Tavano A, Grasso R, Gagliardi C, et al: Disorders of cognitive and affective development in cerebellar malformations. Brain 2007; 130:2646 –2660 16. Duggal HS: Cognitive affective psychosis syndrome in a patient with sporadic olivopontocerebellar atrophy. J Neuropsychiatry Clin Neurosci 2005; 17:260 –262
http://psy.psychiatryonline.org
175