Tuesday, June 28, 2016

Anterior thalamic syndrome


Ghika-Schmid F1, Bogousslavsky J. 2000 Aug;48(2):220-7

The acute behavioral syndrome of anterior thalamic infarction: a prospective study of 12 cases.



Using systematic investigations, including neurological and neuropsychological examinations and computed tomography and magnetic resonance imaging analyzed on anatomical maps, we prospectively studied 12 patients (age range, 63+/-19 years) with an isolated anterior thalamic infarct. They had acute, severe, perseverative behavior, which was apparent in thinking, speech, and all memory and executive tasks, combined with increased sensitivity to interference. They also showed superimposition of mental activities normally processed sequentially (e.g., giving biographical information while working on a calculation test), which we called palipsychism (from the Greek palin [again] and the Greek psyche [soul]). In addition, all 12 patients (8 with a left-sided infarct, 4 with a right-sided infarct) had word-finding difficulties, 7 of 12 with impaired naming, 8 of 12 with dysarthria, and 5 of 12 with hypophonia. Comprehension, repetition, written abilities, and reasoning were consistently preserved, but apathy was usual. All patients had anterograde memory impairment, with a delayed recall deficit, primarily verbal in left-sided infarcts and visuospatial in right-sided infarcts. Dysexecutive features such as difficulty in programming motor sequences were always present. Visual neglect or topographic disorientation was found in 3 patients. Magnetic resonance imaging emphasized involvement of the anterior group of thalamic nuclei, the mamillothalamic tract, and the anterior part of the internal medullary lamina, with structural sparing of the dorsomedial and ventrolateral nuclei. Sequential follow-up examinations showed spectacular improvement within a few months, with the only significant persisting abnormalities being memory dysfunction and apathy. The acute behavioral syndrome of anterior thalamic infarction is dominated by palipsychism, which corresponds to an overlap of sequential cognitive processes in two or more domains. Its association with severe perseverative behavior with increased sensitivity to interference, anterograde memory retrieval deficit, intrusions, naming difficulties with dysarthria and hypophonia, and apathy is suggestive of this type of infarct.

Sunday, January 3, 2016

Malignant subtype of Parkinsons

JAMA Neurology august 2015

Importance  There is increasing evidence that Parkinson disease (PD) is heterogeneous in its clinical presentation and prognosis. Defining subtypes of PD is needed to better understand underlying mechanisms, predict disease course, and eventually design more efficient personalized management strategies.

Objectives  To identify clinical subtypes of PD, compare the prognosis and progression rate between PD phenotypes, and compare the ability to predict prognosis in our subtypes and those from previously published clustering solutions.

Design, Setting, and Participants  Prospective cohort study. The cohorts were from 2 movement disorders clinics in Montreal, Quebec, Canada (patients were enrolled during the period from 2005 to 2013). A total of 113 patients with idiopathic PD were enrolled. A comprehensive spectrum of motor and nonmotor features (motor severity, motor complications, motor subtypes, quantitative motor tests, autonomic and psychiatric manifestations, olfaction, color vision, sleep parameters, and neurocognitive testing) were assessed at baseline. After a mean follow-up time of 4.5 years, 76 patients were reassessed. In addition to reanalysis of baseline variables, a global composite outcome was created by merging standardized scores for motor symptoms, motor signs, cognitive function, and other nonmotor manifestations.

Main Outcomes and Measures  Changes in the quintiles of the global composite outcome and its components were compared between different subtypes.

Results  The best cluster solution found was based on orthostatic hypotension, mild cognitive impairment, rapid eye movement sleep behavior disorder (RBD), depression, anxiety, and Unified Parkinson's Disease Rating Scale Part II and Part III scores at baseline. Three subtypes were defined as mainly motor/slow progression, diffuse/malignant, and intermediate. Despite similar age and disease duration, patients with the diffuse/malignant phenotype were more likely to have mild cognitive impairment, orthostatic hypotension, and RBD at baseline, and at prospective follow-up, they showed a more rapid progression in cognition (odds ratio [OR], 8.7 [95% CI, 4.0-18.7]; P < .001), other nonmotor symptoms (OR, 10.0 [95% CI, 4.3-23.2]; P < .001), motor signs (OR, 4.1 [95% CI, 1.8-9.1]; P = .001), motor symptoms (OR, 2.9 [95% CI, 1.3-6.2]; P < .01), and the global composite outcome (OR, 8.0 [95% CI, 3.7-17.7]; P < .001).

Conclusions and Relevance  It is recommended to screen patients with PD for mild cognitive impairment, orthostatic hypotension, and RBD even at baseline visits. These nonmotor features identify a diffuse/malignant subgroup of patients with PD for whom the most rapid progression rate could be expected.

View Original Article

Pellagra and spinal myoclonus

Park K, Oeda T, Sawada H. A case of alcoholic pellegra encephalopathy presenting with spinal myoclonus.  Neurology Clinical Practice 5; 472-3.
The authors present a case of alcoholic pellagra  with confusion and myoclonus responding dramatically to administration of niacin1500 mg per day starting 16 days after admission.  Essential points include:
1. Pellagra is rare in US but not in alcoholics
2. Dermatitis may be subtle and not appreciated
3.  Thiamine and niacin levels may be normal
4.  Thiamine may cause worsening due to increased demand for niacin
5.  Myoclonus in context is important to diagnosis, often stimulus sensitive
6.  Severe sensory ataxia, incontinence and dysautonomia also occur and improve with treatment
the 4 D's of pellagra, again, are , diarrhea, dementia, dermatitis and death

Scurvy in neurologic disease

Meisal K, Daggubati S,Josephson SA. .  Scurvy in the 21st century?  Vitamin C deficiency presenting to the neurologist.  Neurol Clin Prac 2015; 5:491-493.
Authors present a series of cases with vitamin C deficiency and review some of the neuro manifestations and non neuro manifestations, ; the former are not widely known. 
Patients with deficiency were caused by various other causes,including autism, poor status without access to produce, usually rural, were not alcohol users, had measurable low vitamin C levels.  Gingival hyperplasia, rash and bleeding were non neurologic manifestations. People bruised,especially on their thighs, Some had other nutritional diseases..  Pain, achiness and weight loss are expected.
Neuro manifestations included positional tremor, neuralgias100 %), focal weakness (50 %)  including footdrop and scapular winging, normal MRI's, long tract signs including hyperreflexia and plantar extensors, fatigue, trouble concentrating, headache, anxiety, and imbalance.
Patients recovered dramatically with treatment.

Wednesday, March 25, 2015

Idiopathic hypertrophic pachymeningitis

  Dumont AS, Clark AW, Sevick RJ, Myles ST. Idiopathic hypertrophic pachymeningitis:  A report of two cases and review of the literature.
Background-- Authors note entity was described by Charcot and Joffroy, and that there are three forms:  spinal, intracranial and craniospinal (latter is rarer). 
Past cases were often attributed to specific etiologies but most recent cases are idiopathic after investigation.  Authors case 1 underwent 2 surgeries for biopsy/decompression , had persistent pain and numbness, but was non progressive for 15 years after one early relapse.  The second one received steroids after biopsy with resolution of symptoms and MRI changes.  Authors argue based on above that the condition is not autoimmune. 
Literature review suggested a worse prognosis for patients with "inflammatory signs" (fever, high sed rate, CRP, or elevated WBC). 
Associations include infections: syphilis, TB, HTLV-1, fungi; may be presenting sign of adjacent ear or sinus infection.  Autoimmune associations include RA, orbital pseudotumor, multifocal fibrosclerosis, MCTD, Wegener's granulomatosis.    The 23 reports run the gamut from marked to slight improvement to deterioration and late recurrence or surgical death. 
For interest, Charcot's clinical descriptors divided the spinal form into distinct stages: first intermittent radicular pain that later became continuous; then muscle weakness and atrophy; then spastic paralysis and loss of sphincter control.  Radicular signs can be confined to the upper extremities and evolve over weeks to months or even a year. 
The cranial form frequently presents with a headache, cranial neuropathies and ataxia. 
Authors emphasize the need for pathologic confirmation.

Tuesday, December 9, 2014

CNS metastases in breast cancer patients: prognostic implications of tumor subty

CNS metastases in breast cancer patients: prognostic implications of tumor subtype; Bachmann C, Schmidt S, Staebler A, Fehm T, Fend F, Schittenhelm J, Wallwiener D, Grischke E; Medical Oncology (Northwood; London; England) 32 (1), 400 (Jan 2015)
Development of brain metastases (BM) in breast cancer leads to limited survival. The therapeutical options are limited. There are less data about the risk factors and prognostic importance in BM. Objective is to investigate predictors of central nervous system metastases and outcome after diagnosis of BM according to tumor subtype. Based on medical records, 80 consecutive patients with primary non-metastatic operable breast cancer, treated at Department of Gynecology, University of Tübingen, and who developed BM during follow-up, were retrospectively analyzed. Clinicopathological parameters and their prognostic impact were evaluated. A node involvement (40 %), ER/PR negative (53.75 vs. 61.25 %), triple negative (28.75 %) and HER2+ status (40 %) were associated with BM. BM in breast cancer patients lead to a shortened survival. In cerebral metastatic breast cancer patients with HER2-negative and triple-negative, patients had significant shorter survival after detection of BM compared with HER2-positive and non-triple-negative patients (p = 0.001; p = 0.03). Risk of BM varies significantly by subtype. Understanding the biology of metastases can help categorize patients into prognostically useful categories and tailor treatment regimens for individual patients. Prospective clinical trials would be required for evaluating the potential role of screening for asymptomatic BM and of treatment of triple-negative patients.

Thursday, November 13, 2014

MMN and treatment responsiveness

Slee M, Selvan A, Donaghy M.  Neurology 2007; 69:1680-1687.
Authors look at 47 patients retrospectively and found that strict adherence to consensus criteria was a barrier to treatment.  Neither conduction block nor antibody status is a reliable predictor of treatment responsiveness.
Some of the pearls in the article:  Conduction block was present in 66%; GM1 antibody in a minority (25 %) and was not associated with conduction block. 27 % had lower limb onset, bu tin those patients, arm weakness typically developed subsequently.  Patients may have an acute or subacute onset with spontaneous resolution.  MMN can be PRECIPITATED by the use of steroids, and is usually not helped by steroids.  It "is evident from our study and others (Pakkiam AS, Perry GJ, Multifocal motor neuropathy without overt conduction block. Muscle Nerve 1998; 21: 243-245) that IVIG responsive  MMN occurs regularly without conduction block being demonstrated."  "A redefinition of conduction block which eliminates temporal dispersion as a restrictive factor and which helps predict IVIG responsiveness, (see Ghosh and Busby, JNNP 2005) categorized a further 15 % of our patients with conduction block."  "Our study suggests a decrement if IVIG responsiveness over time."  Using higher doses of 1.92 g/kg/6 weeks does better than Utrecht group of .54 g/kg /mo.  Disability self reports were more accurate than MRC scale scores with overall clinical change.
"Recognition of the clinical picture is the mainstay of diagnosis of MMN outside the researchg setting.  Weakness in nonwasted muscles and differential weakness across a common terminal motor nerve are the cardinal features.  Differential finger extension weakness is a frequent early manifestation likely reflecting vulnerabilities of the terminal branches of the posterior interosseous nerve." 
Blogger note-- this citation is extremely important for dealing with insurance companies to obtain approval for IVIG. 
Take away-- to take care of the patient, don't look at the test, be a doctor and a neurologist