Neurological dementias

Author(s):  
Andrew Graham

Dementia in old age is usually due to Alzheimer’s disease, cerebrovascular disease, or mixed pathology. Dementia due to other neurological disorders is uncommon, but important to recognize because management may be very different to that in primary or vascular dementia. This chapter surveys five neurological conditions that may present with dementia in later life: idiopathic normal pressure hydrocephalus (INPH); Huntington’s disease (HD); multiple sclerosis (MS); autoimmune limbic encephalitis (LE); and prion disease. For each disorder it reviews the epidemiology, clinical features, investigations, and treatment, with examples of the characteristic brain imaging changes. Accurate diagnosis of these conditions can be challenging even for physicians with a special interest in dementia, and often requires a neurological referral.

Author(s):  
Andrew Graham

Dementia in old age is usually due to Alzheimer’s disease, cerebrovascular disease, or mixed pathology. Dementia due to other neurological disorders is uncommon, but important to recognise because management may be very different to that in primary or vascular dementia. This chapter surveys five neurological conditions that may present with dementia in later life: idiopathic normal pressure hydrocephalus (INPH); Huntington’s disease (HD); multiple sclerosis (MS); autoimmune limbic encephalitis (LE); and prion disease. For each disorder the epidemiology, clinical features, investigations & treatment are reviewed, with examples of the characteristic brain imaging changes. Accurate diagnosis of these conditions can be challenging even for physicians with a special interest in dementia, and often requires a neurological referral.


Neurosurgery ◽  
2006 ◽  
Vol 59 (3) ◽  
pp. 545-552 ◽  
Author(s):  
Diego Spagnoli ◽  
Lucia Innocenti ◽  
Lorenzo Bello ◽  
Mauro Pluderi ◽  
Susanna Bacigaluppi ◽  
...  

Abstract OBJECTIVE: The influence of cerebrovascular disease (CVD) on the short- and long-term results of surgery was evaluated in a series of consecutive patients with idiopathic normal-pressure hydrocephalus (iNPH). METHODS: Patients with suspected iNPH admitted to our department between June 1996 and June 2003 were evaluated with four clinical and handicap scales. CVD and risk factors for vascular disease were rated. All patients underwent intracranial pressure monitoring via a spinal catheter. Sixty-six patients received a ventriculoperitoneal shunt with a programmable valve. Prospective assessments were programmed at 2 weeks and 3 months after surgery (short-term follow-up). Long-term follow-up evaluations were arranged in June 2004 with patients and/or relatives and health/home care assistants. RESULTS: At the short-term follow-up examination, a significant clinical improvement was globally present in 89% of the patients (P < 0.05). CVD, such as leucoaraiosis or previous strokes, were present in 71% of the patients. Patients both with and without CVD and/or risk factors for vascular disease presented a significant improvement (P < 0.05) after shunting; 85 and 100% of the patients with and without CVD, respectively. At the long-term follow-up examination (mean, 52 ± 24.8 mo), 24% of the patients were dead and 8% had experienced stroke. Globally, 60% of the patients were still improved (P < 0.05); 52 and 79% of the patients with and without CVD, respectively. CONCLUSION: A high success rate in treatment of iNPH is possible in patients with and without CVD. Despite poorer short- and long-term treatment outcome of iNPH patients with CVD, a long-lasting improvement in their quality of life favors surgery.


2020 ◽  
pp. 2642-2647
Author(s):  
Pooja Dassan

Pregnancy can influence the clinical course of an underlying neurological problem or precipitate the first presentation of a neurological disease. This chapter looks at the various ways pregnancy can affect those suffering from epilepsy, multiple sclerosis, or cerebrovascular disease. In epilepsy, sodium valproate is associated with higher risk of major congenital malformations and impaired neuropsychological development than other antiepileptic drugs: lamotrigine or levetiracetam are preferred. Women taking antiepileptic drugs can breastfeed. In multiple sclerosis, the relapse rate is reduced in pregnancy, but substantially increased for three months post-partum. For cerebrovascular disease, stroke syndromes specific to pregnancy include pre-eclampsia, which can lead to posterior reversible vasoconstriction syndrome. Reversible cerebral vasoconstriction syndrome presents after delivery and has many similarities to posterior reversible vasoconstriction syndrome. There are conflicting opinions as to whether pregnancy or delivery increases the risk of cerebral aneurysm or arteriovenous malformation rupture.


2011 ◽  
Vol 22 (1) ◽  
pp. 101-104 ◽  
Author(s):  
O. Algin ◽  
O. Taskapilioglu ◽  
B. Hakyemez ◽  
M. Parlak

1999 ◽  
Vol 90 (2) ◽  
pp. 221-226 ◽  
Author(s):  
Agnita J. W. Boon ◽  
Joseph T. J. Tans ◽  
Ernst J. Delwel ◽  
Saskia M. Egeler-Peerdeman ◽  
Patrick W. Hanlo ◽  
...  

Object. This study was conducted to determine the prevalence of cerebrovascular disease and its risk factors among patients with normal-pressure hydrocephalus (NPH) and to assess the influence of these factors on the outcome of shunt placement.Methods. A cohort of 101 patients with NPH underwent shunt placement and was followed for 1 year. Gait disturbance and dementia were quantified using an NPH scale and handicap was determined using a modified Rankin scale (mRS). Primary outcome measures consisted of the differences between preoperative and last NPH scale and mRS scores. The presence of risk factors such as hypertension, diabetes mellitus, cardiac disease, peripheral vascular disease, male gender, and advancing age was recorded. Cerebrovascular disease was defined as a history of stroke or a computerized tomography (CT) scan revealing infarcts or moderate-to-severe white matter hypodense lesions.The prevalence of risk factors for cerebrovascular disease was higher in the 45 patients with cerebrovascular disease than the 56 without it. Risk factors did not influence outcome after shunt placement. Intent-to-treat analysis revealed that the mean improvement in the various scales was significantly less for patients with a history of stroke (14 patients), CT scans revealing infarctions (13), or white matter hypodense lesions (32 patients) than for those without cerebrovascular disease. The proportion of patients who responded to shunt placement was also significantly lower among patients with than those without cerebrovascular disease (p = 0.02).Conclusions. The authors identified a subgroup of patients with NPH and cerebrovascular disease who showed disappointing results after shunt placement. Cerebrovascular disease was an important predictor of poor outcome.


Neurosurgery ◽  
1989 ◽  
Vol 25 (5) ◽  
pp. 777-780 ◽  
Author(s):  
Wolfram Fritz ◽  
Henry k Kalbarczyk ◽  
Klaus Schmidt

Abstract We report on transcranial Doppler sonography with carbogen reactivity testing of flow velocity as applied to patients with normal pressure hydrocephalus. A subgroup can be differentiated that shows coexistent cerebrovascular disease, The surgical prognosis in these patients is bad. A flow chart demonstrating the proposed clinical examination is presented.


2020 ◽  
Vol 35 (6) ◽  
pp. 1050-1050
Author(s):  
Wisinger A ◽  
Fink J

Abstract Objective Psychological syndromes like depression and anxiety are common in neurological conditions and lead to poorer quality of life and disability. However, these syndromes are often under-recognized in neurologic patients and are thus under-diagnosed due to overlapping symptomatology. Here, we report on a patient with a complex neurological history as well as clinically significant affective symptoms. Participants The patient is a 69-year-old Caucasian female recently diagnosed with normal pressure hydrocephalus. Relevant medical history was significant for focal epilepsy with complex seizures, obstructive sleep apnea, and cerebrovascular accident. She underwent neuropsychological evaluation as part of a pre-surgical workup for shunt implantation. Results The patient’s neurocognitive profile was marked by significant impairments in most cognitive domains and she was not independent in activities of daily living. On self-report measures of current emotional functioning, she reported clinically severe levels of depression, anxiety, apathy, executive dysfunction, and disinhibition. During the clinical interview, she reported experiencing passive suicidal ideation as well as a significant history of traumatic experiences. Conclusion The psychological symptoms the patient was experiencing undoubtedly impacted her neurocognitive status and functioning abilities, though she was receiving no treatment for these symptoms. Addressing symptoms resulting from psychological syndromes in patients with neurological conditions is an important role for neuropsychologists, as accurate diagnosis of psychological syndromes can inform treatment recommendations and improve patient outcomes. This case illustrates the importance of documenting a thorough clinical history, including past psychiatric history, as well as a detailed examination of self-reported current psychological functioning as part of a comprehensive neuropsychological case formulation.


Neurology ◽  
1980 ◽  
Vol 30 (9) ◽  
pp. 998-998 ◽  
Author(s):  
D. Shukla ◽  
B. M. Singh ◽  
R. J. Strobos

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