Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD.

BACKGROUND: Behavioral variant frontotemporal dementia (bvFTD) is a relatively well-defined clinical syndrome. It is associated with frontal and temporal lobe structural/metabolic changes and pathologic findings of a neurodegenerative disease. We have been evaluating patients with clinical and imaging features partially consistent with bvFTD but with evidence also suggestive of brain sagging, which we refer to as frontotemporal brain sagging syndrome (FBSS). METHODS: Retrospective medical chart review to identify all patients seen at our institution between 1996 and 2010, who had a clinical diagnosis of FTD and imaging evidence of brain sag. RESULTS: Eight patients, 7 male and 1 female, were diagnosed with FBSS. The median age at symptom onset was 53 years. All patients had insidious onset and slow progression of behavioral and cognitive dysfunction accompanied by daytime somnolence and headache. Of the 5 patients with functional imaging, all showed evidence of hypometabolism of the frontotemporal regions. On brain MRI, all patients had evidence of brain sagging with distortion of the brainstem; 3 patients had diffuse pachymeningeal enhancement. CSF opening pressure was varied and CSF protein was mildly elevated. A definite site of CSF leak was not identified by myelogram or cisternography, except in one patient with a site highly suggestive of leak who subsequently underwent surgery confirming a CSF leak. In 2 patients with a neuropathologic examination, there was no evidence of a neurodegenerative disease. CONCLUSIONS: This case series demonstrates that FBSS may mimic typical bvFTD but should be recognized as an unusual presentation that is potentially treatable.

Imaging correlates of pathology in corticobasal syndrome.

BACKGROUND: Corticobasal syndrome (CBS) can be associated with different underlying pathologies that are difficult to predict based on clinical presentation. The aim of this study was to determine whether patterns of atrophy on imaging could be useful to help predict underlying pathology in CBS. METHODS: This was a case-control study of 24 patients with CBS who had undergone MRI during life and came to autopsy. Pathologic diagnoses included frontotemporal lobar degeneration (FTLD) with TDP-43 immunoreactivity in 5 (CBS-TDP), Alzheimer disease (AD) in 6 (CBS-AD), corticobasal degeneration in 7 (CBS-CBD), and progressive supranuclear palsy in 6 (CBS-PSP). Voxel-based morphometry and atlas-based parcellation were used to assess atrophy across the CBS groups and compared to 24 age- and gender-matched controls. RESULTS: All CBS pathologic groups showed gray matter loss in premotor cortices, supplemental motor area, and insula on imaging. However, CBS-TDP and CBS-AD showed more widespread patterns of loss, with frontotemporal loss observed in CBS-TDP and temporoparietal loss observed in CBS-AD. CBS-TDP showed significantly greater loss in prefrontal cortex than the other groups, whereas CBS-AD showed significantly greater loss in parietal lobe than the other groups. The focus of loss was similar in CBS-CBD and CBS-PSP, although more severe in CBS-CBD. CONCLUSIONS: Imaging patterns of atrophy in CBS vary according to pathologic diagnosis. Widespread atrophy points toward a pathologic diagnosis of FTLD-TDP or AD, with frontotemporal loss suggesting FTLD-TDP and temporoparietal loss suggesting AD. On the contrary, more focal atrophy predominantly involving the premotor and supplemental motor area suggests CBD or PSP pathology.

Prevalence of sleep disturbance in closed head injury patients in a rehabilitation unit.

UNLABELLED: Traumatic brain injury (TBI) is a leading cause of disability in young people in the United States. Disorders of arousal and attention are common in closed head injury (CHI). Daytime drowsiness impairs participation in rehabilitation, whereas nighttime wakefulness leads to falls and behavioral disturbances. Sleep disturbances in TBI reported in the literature have included excessive daytime somnolence, sleep phase cycle disturbance, narcolepsy, and sleep apnea. Although well known to the clinician treating these patients, the extent and prevalence of disrupted sleep in patients in an acute inpatient rehabilitation unit has not been described. OBJECTIVE: To determine the prevalence of sleep wake cycle disturbance (SWCD) in patients with CHI in a TBI rehabilitation unit. DESIGN: Prospective observational. SETTING: Inpatient specialized brain injury rehabilitation unit. Patients. Thirty-one consecutive admissions to a brain injury rehabilitation unit with the diagnosis of CHI. RESULTS: Twenty-one patients (68%) had aberrations of nighttime sleep. There was no significant difference in Glasgow Coma Score on admission to trauma nor was there any significant difference in age between the affected and unaffected groups. Patients with SWCD had longer stays in both the trauma center (P < .003) and the rehabilitation center (P < .03). CONCLUSIONS: There is a high prevalence of SWCD in CHI patients admitted to a brain injury rehabilitation unit. Patients with SWCD have longer stays in both acute and rehabilitation settings and may be a marker for more severe injury.

Substance abuse, traumatic brain injury and neuropsychological outcome.

The neuropsychological performance of 119 patients with severe closed traumatic brain injury (TBI) who had received toxicology screens at the time of trauma centre admission was examined. Three groups were created: normal screen, positive alcohol screen, or positive abused drugs screen (with or without the presence of alcohol). The admitting Glasgow Coma Scale (GCS) score was significantly lower in the positive alcohol screen group than the normal screen group, while the three groups did not differ in length of post-traumatic amnesia (PTA) or years of education. Neuropsychological assessment was conducted during inpatient rehabilitation, following resolution of PTA. Normal screen patients obtained significantly better scores than the abused-drugs patients on the Full Scale IQ (FIQ) and Verbal IQ (VIQ) indices of the Wechsler Adult Intelligence Scale-Revised and the Verbal Memory, General Memory, Attention-Concentration, and Delayed Recall indices of the Wechsler Memory Scale-Revised. Normal screen patients also scored significantly higher than positive alcohol screen patients on FIQ and VIQ indices and all five indices from the Wechsler Memory Scale-Revised. These data suggest the existence of an additive effect of substance abuse on neuropsychological outcome in TBI. Findings have potential implications for both acute management and rehabilitation of TBI.

Pathological laughter and crying in patients with closed traumatic brain injury.

We report on the clinical and radiological features in 16 adult patients who suffered a traumatic brain injury and subsequently developed pathological laughter and crying. Patients with pathological laughter and crying were identified from among 301 consecutive brain-injured admissions to a trauma centre and subsequently to a rehabilitation facility. Patients displaying pathological laughter and crying had a greater severity of injury than patients without the syndrome; they also had other associated neurological features compatible with pseudobulbar palsy. Pathological laughter alone, or combined with crying, was more frequent than crying alone. An attempt to correlate clinical features with focal lesions on neuroimaging studies yielded inconsistent results. The theoretical anatomical substrate for pathological laughter and crying in patients with traumatic brain injury is discussed.

Traumatic injury in patients with neurologic and psychiatric disease.

Our work and multiple other lines of evidence suggest that patients suffering from neurologic and psychiatric disorders are over-represented in the population suffering traumatic injuries. In addition, a premorbid history of such disorders can impact on the severity of injury, as well as present particular problems in the acute management of this population. Constant vigilance, leading to prompt identification of concurrent neurologic and psychiatric illness in trauma victims, is critical for patient management. The interactions of these disorders and their treatments present complex clinical challenges to the traumatologist and the intensivist.

Brain SPECT in a case of cortical blindness.

BACKGROUND: Published reports on patients with cortical blindness describe bilateral brain hemispheric lesions visualized in radiological and functional imaging studies. CASE DESCRIPTION: We present a case with a unilateral lesion on radiological studies and bilateral abnormalities on single-photon emission-computed tomographic (SPECT) scanning. CONCLUSIONS: SPECT images correlated much more closely than radiological studies with the patient's clinical status. We suggest that SPECT scanning can be a useful indicator of focal brain dysfunction in brain injury in spite of normal radiological studies. We also hypothesize that our patient's clinical and functional imaging findings could be attributed in part to the process of diaschisis.

Substance abuse as a factor in the causality, severity, and recurrence rate of traumatic brain injury.

The effects of substance abuse on the cause, severity, and recurrence of traumatic brain injury in 322 admissions to a large rehabilitation inpatient facility are explored. Study patients tended to be young and predominantly male. Few had completed high school and a significant minority had a history of cerebral dysfunction. Patients tended to have moderate to severe closed head injuries. Motor vehicle crashes were the most common cause of injury, but patients reporting drug or drug and alcohol abuse were more likely to sustain violent injuries (e.g., gunshot wounds).

Viral and immune-mediated factors in the pathogenesis of psychiatric disease.

The available data supporting a viral or immune mediated etiology of psychiatric disease is indirect and controversial, but there is a sufficient evidence to implicate such a process in a subgroup of the psychiatric population. Recent technological advances in neurovirology and neuroimmunology justify a re-assessment of this etiology in psychiatric disease. The author describes three patients with psychiatric symptomatolgy and cerebrospinal changes compatible with such a process. The evidence supporting a role for viral or immunological factors in the pathogenesis of psychiatric disease is reviewed, and guidelines for future research which could be of use in further clarifying this issue are proposed.