Researchers are constantly defining new forms of dementia as research tools become better: genetics, MRI scans, analysis of inclusions, etc.  Dementias on this page include: ALS Dementia, CADASIL dementia, AV Fistula dementia, Idiopathic Normo-Pressure Hydrocephalus dementia, NIFID dementia, Pick's Disease, Posterior Cortical Atrophy dementia, Primary Progressive Aphasia, and Subcortical dementia. Thanks to better genetic and lab tools, new diseases are popping up every time I turn on my computer.  

ALS Dementia Has Ubiquitin Inclusions: Peculiar tau-negative, ubiquitin-positive inclusions appear in dementia with ALS (ALS-D), the majority of lobar atrophy (Pick's disease) without Pick body and a small portion of ALS. Another common neuropathological lesion in these diseases is the motor neuron involvement with the degenerative processes. The lower motor neuron is predominantly involved in ALS and ALS-D the upper motor neuron is predominantly involved, but in varying degrees in a considerable number of patients with lobar atrophy that lack Pick bodies. The boundary between these diseases is not always clear and a significant number of cases are considered to be the transitional form. Motor neuron disease group accompanied by inclusions of unidentified protein signaled by ubiquitin. Ikeda K, Tsuchiya K. Tokyo Institute of Psychiatry. Neuropathology. 2004 Jun;24(2):117-24

CADASIL: Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy:   CADASIL is an inherited small vessel disease causing stroke and dementia. Eighty CADASIL subjects average age 46 were followed for 2 years. Standardized scales on disability (Rankin), activities of daily living (Barthel index), neurological outcome (National Institutes of Health Stroke Scale [NIHSS]), and cognition (structural interview for diagnosis of Alzheimer dementia and multi-infarct dementia [SIDAM] and Mattis dementia rating scale [MDRS]) were assessed at baseline and at follow-up. At follow-up, the cohort had deteriorated with respect to all clinical scales: Rankin scores (0.3 average change; P=0.001), Barthel index (-5.4; P<0.001), NIHSS scores (1.0; P=0.001), SIDAM scores (-2.1; P=0.004), and MDRS scores (-4.3; P=0.09). The spectrum ranged from marked worsening to some degree of improvement. Seventeen patients experienced a new stroke. Overall, there were 18 strokes within 173 person-years, giving an average incidence rate of stroke of 10.4 per 100 person-years. Age at baseline was found to be a predictor of clinical progression. Sample size estimates show that the number of individuals needed to include in an interventional trial (assumed duration 2 years, assumed treatment effect 40%) is 602 when using stroke occurrence as an outcome measure. A Two-Year Clinical Follow-Up Study in 80 CADASIL Subjects. Progression Patterns and Implications for Clinical Trials. Peters N, Herzog J, Opherk C, Dichgans M. Ludwig-Maximilians-University, Munich, Germany. Stroke. 2004 May 20

Dural AV Fistulas Can Cause Rapid Developing Dementia: Dural fistulas are acquired arteriovenous shunts, accounting for 10-15% of cerebrovascular malformations. Symptoms are commonly tinnitus or intracranial hemorrhage. Rarely, patients with dural fistulas can present with rapid cognitive impairment. The authors report two women with rapidly evolving dementia. Cerebral angiography revealed dural arteriovenous fistula, with retrograde drainage into cortical veins, related to thrombosis of both transverse sinuses. Intra-arterial and intra-venous endovascular approaches failed to cure the fistula. Venous embolization via a transcranial approach was required to occlude the fistula, leading to resolution of the symptoms. Dural arteriovenous fistulas may lead to dementia with diffuse white matter changes related to venous ischemia, and must be considered as a reversible cause of vascular dementia. Dural arteriovenous fistula. A rare cause of treatable dementia. Magot A, Desal H, et al. Nantes, France. Rev Neurol (Paris). 2004 Apr;160(4 Pt 1):425-33

Idiopathic Normal Pressure Hydrocephalus Triad: Dementia, Urinary and Gait Problems: (iNPH) is a syndrome characterized by gait disturbance, dementia, and/or urinary incontinence without causative disorders, and ventricular enlargement due to disturbance of the cerebrospinal fluid (SF) circulation. The number of patients with iNPH will increase with the aging of the population in Japan. However, iNPH is often difficult to differentiate from other senile disorders such as lumbar canal stenosis, parkinsonism, and so on. Clinical guidelines for iNPH are required to improve understanding and provide for patients' quality of life and social care. These guidelines propose three levels of iNPH: possible, probable, and definite. Possible iNPH includes one or more of the classical triad and ventricular dilation in middle aged and elderly patients with closing of the CSF space at high convexity on magnetic resonance imaging. Probable iNPH shows improvement of the symptoms after CSF removal in patients with possible iNPH. Definite iNPH shows clinical improvement after CSF shunt operation. The CSF tap test is a major diagnostic measure because of the simplicity and less invasiveness. Use of the programmable valve is recommended to decrease CSF overdrainage. Clinical guidelines for idiopathic normal pressure hydrocephalus. Ishikawa M; Guideline Committe for Idiopathic Normal Pressure Hydrocephalus, Japanese Society of Normal Pressure Hydrocephalus. Osaka, Japan. Neurol Med Chir (Tokyo). 2004 Apr;44(4):222-3

INPH Triad Helped by Shunts: Thirty men and 13 women with a mean age of 71 underwent surgery and continuous monitoring of intracranial pressure. Clinical improvement occurred in 86% after shunt insertion, 11.6% showed no change, and 2.3% exhibited some worsening. Gait improved in 81.4%, sphincter control in 69.8%, and cognitive dysfunction in 39.5%. There was no treatment-related death. Early or late postsurgical complications occurred in six patients (14%), although all were minor or were satisfactorily resolved. The complete clinical triad, cortical sulci size, and periventricular lucencies were related to outcome, whereas patient age, symptom duration, ventricular dilation, and the degree of presurgical dementia were unrelated to outcome. Is the placement of shunts in patients with idiopathic normal-pressure hydrocephalus worth the risk? Results of a study based on continuous monitoring of intracranial pressure. Poca MA, et al. Spain. J Neurosurg. 2004 May;100(5):855-66

NIFID: Neuronal Intermediate Filament Inclusion Disease: Abnormal neuronal aggregates of alpha-internexin and the three neurofilament (NF) subunits, NF-L, NF-M, and NF-H have recently been identified as the pathological hallmarks of neuronal intermediate filament (IF) inclusion disease (NIFID), a novel neurological disease of early onset with a variable clinical phenotype including frontotemporal dementia, pyramidal and extrapyramidal signs. alpha-Internexin, a class IV IF protein, a major component of inclusions in NIFID, has not previously been identified as a component of the pathological protein aggregates of any other neurodegenerative disease. Therefore, to determine the specificity of this protein, alpha-internexin immunohistochemistry was undertaken on cases of NIFID, non-tau frontotemporal dementias, motor neuron disease, alpha-synucleinopathies, tauopathies, and normal aged control brains. Our results indicate that class IV IF proteins are present within the pleomorphic inclusions of all cases of NIFID. Small subsets of abnormal neuronal inclusions in Alzheimer's disease, Lewy body diseases, and motor neuron disease also contain epitopes of alpha-internexin. Thus, alpha-internexin is a major component of the neuronal inclusions in NIFID and a relatively minor component of inclusions in other neurodegenerative diseases. The discovery of alpha-internexin in neuronal cytoplasmic inclusions implicates novel mechanisms of pathogenesis in NIFID and other neurological diseases with pathological filamentous neuronal inclusions. alpha-Internexin aggregates are abundant in neuronal intermediate filament inclusion disease (NIFID) but rare in other neurodegenerative diseases. Cairns NJ, Uryu K, et al.  University of Pennsylvania School of Medicine.

Pick's Disease: Pick Bodies are Three-Repeat Tau Isoforms: Pick's disease is a dementia characterized by a slowly progressive deterioration of social skills and changes in personality, along with impairment of intellect, memory, and language. Common symptoms include loss of memory, lack of spontaneity, difficulty in thinking or concentrating, and disturbances of speech. Other symptoms include gradual emotional dullness, loss of moral judgment, and progressive dementia. The disease usually affects individuals between the ages of 40 and 60. Pick bodies were found in a family with hereditary Pick's disease with the G272V mutation of the tau protein gene and in several families with other tau mutations in exons 9 and 11-13. The biochemical composition of Pick bodies varies between these mutations. The Pick bodies consisted exclusively of three-repeat (3R) isoforms. Hereditary Pick's disease with the G272V tau mutation shows predominant three-repeat tau pathology. Bronner IF, et al. VU University, Amsterdam, The Netherlands. Brain. 2005 Jul 13

Posterior Cortical Atrophy Starts with Visual Dysfunction: Posterior cortical atrophy (PCA) is an uncommon dementia syndrome with initial manifestations of visual dysfunction and preservation of memory and language until late in the disease.  Fifteen PCA cases and 15 tAD cases, defined by clinical and MRI criteria, were compared. Symptoms of visual disturbance and dyslexia were more commonly reported in PCA cases (p = 0.0001 and p = 0.006), and memory loss was more commonly reported in tAD (p = 0.006). Patients with PCA were less accurate on the Cortical Vision Screening Test (t = 6.0; p < 0.001) and in copying the Rey-Osterreith Complex Figure (t = 6.0; p < 0.001). Memory, evaluated by the Rey Auditory Verbal Learning Test, was impaired in both groups; however, delayed recall was more impaired in the tAD group (t = 2.5; p = 0.03). Posterior cortical atrophy: Clinical presentation and cognitive deficits compared to Alzheimer's disease. Charles RF, et al. Johns Hopkins. Behav Neurol. 2005;16(1):15-23.

Primary Progressive Aphasia Described: M.-Marsel Mesulam, New England Journal of Medicine 10/16/03 of Northwestern University says primary progressive aphasia, an unusual dementia of unknown cause that is characterized by a relentless loss of language but with memory relatively preserved. It has been reported in several hundred individuals. Alzheimer's patients have forgetfulness, usually accompanied by apathy. They misplace personal objects, repeat questions and forget recent events. However, while these patients may forget people's names, word-finding during conversation is not a major problem. Primary progressive aphasia onsets with word-finding difficulties, abnormal speech patterns and glaring spelling errors. Some cannot find the right words to express their thoughts. Others cannot understand the meaning of words either heard or seen. Still others cannot name objects in their environment. Some can write language; others develop agrammatism, using inappropriate word order and misusing word endings, prepositions, pronouns, conjunctions and verb tenses. Language is the only area of prominent dysfunction for at least the first two years of primary progressive aphasia. Structural brain imaging studies do not reveal a specific lesion, other than atrophy. Language difficulties may be the patient's only symptoms for 10 to 14 years. Other cognitive impairments may emerge, but the language deficit remains the primary feature. Some individuals with primary progressive aphasia maintain and even intensify their involvement in complex hobbies such as gardening, carpentry, sculpting and painting.  Unlike patients with Alzheimer's disease, who cannot retain new information in memory, patients with primary progressive aphasia can recall and evaluate recent events even though they may not be able to express their knowledge verbally. Currently, there is no effective pharmacologic treatment for primary progressive aphasia.

Striatal Hypermetabolic State Mimicked Dementia: Hypermetabolic lesions of the striatum are rare. They usually involve autoimmunity and are associated with choreatic disorders such as Sydenham chorea, lupus, and the antiphospholipid antibody syndrome. A 48-year-old woman was initially seen with a 1-year history of progressive changes in personality and attention. There were minor associated involuntary movements. Negative routine investigation results, including regular autoimmune serologies and magnetic resonance imaging, led to the diagnosis of a frontal dementia. Positron emission tomographic results demonstrated hypermetabolism in the left striatum. Western blot technique results demonstrated plasma antistriatal antibodies. Treatment with corticosteroids induced a resolution of her attentional deficits and a return of her personality to its premorbid state. The repeated positron emission tomographic scan results were near normal, and the titer of antistriatal antibodies was significantly reduced. Dementia-like presentation of striatal hypermetabolic state with antistriatal antibodies responsive to steroids. Leger GC, Johnson N, Horowitz SW, Dale RC, Church AJ, Mesulam MM. Universite de Montreal. Arch Neurol. 2004 May;61(5):754-7

Subcortical Dementia with Globus Pallidus Lesions due to High Altitude Cerebral Edema: Two 63-year-old women developed high altitude cerebral edema complicated by the occurrence of permanent neuropsychiatric sequelae after both developed disturbance of consciousness shortly after their arrival at Cuzco, Peru (3500 m). Both developed persistent neuropsychiatric symptoms after resolution of the acute illness. In one, there was a 1-month lucid interval between remission of high altitude illness and occurrence of the irreversible neuropsychiatric sequelae. CT and MRI disclosed lesions in the globus pallidus bilaterally, suggesting that the neuropsychiatric symptoms in these patients were manifestations of subcortical dementia. The development of high altitude illness was considered to be attributable to mild restrictive lung impairment in case 1 and to a deficient ventilatory response to hypoxia in case 2.  Irreversible subcortical dementia following high altitude illness. Usui C, Inoue Y, Kimura M, Kirino E, Nagaoka S, Abe M, Nagata T, Arai H.  Juntendo University School of Medicine, Tokyo, Japan.