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2.10 Dementia 99
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because the patients present with headache, ataxia,
trembling limbs, and laughing or crying episodes without a prior reason.
CJD is a neurodegenerative prion disease with four
main forms:
Sporadic CJD (sCJD) : this is the most common
form, with an incidence of 1–1.5 per million of
population.
Familial CJD (fCJD) : this is a rare form due to muta-
tion in the PrP gene.
Iatrogenic CJD (iCJD) : this form is related to neuro-
surgeries with cadaveric-derived dura matter or corneal grafts.
New variant CJD (vCJD) : this form is related to con-
sumption of meat infected with BSE. It is generally
seen in younger patients than the classical CJD.
sCJD is characterized by rapidly progressing dementia, with 50% chance of death within 5 months of
symptom onset. It is typically seen in patients 60–75years old. Other neurological features include cerebellar ataxia, pyramidal and extrapyramidal signs, and
cortical blindness. Death in sCJD patients is most commonly due to pneumonia.
vCJD is linked to consumption of infected cattle
Fig. 2.10.9. Axial FLAIR brains MR-illustration demonstrates
the MR signs of sCJD
meat with BSE. vCJD is seen in younger age than
sCJD, and the neurological symptoms are nonspecifi c,
with patients often showing psychiatric and behavioral
changes. Incubation period of the disease is approximately 10 years. MRI plays an important role in establishing the diagnosis, since defi nite diagnosis of prion
diseases requires pathological sample examination.
Signs on MRI
In sCJD, brain shows hyperintense signal changes in the
caudate head and the putamen on T2W images (Fig. 2.10.9 ).
This sign can be observed in other diseases like carbon
monoxide poisoning, hypoglycemia, hemolytic-uremic
syndrome, and Wilson’s disease.
In vCJD, there are bilateral, almost symmetrical T2 hyperin-
tense lesions found in the pulvinar, the most posterior
thalamic nucleus ( positive pulvinar sign ) (Fig. 2.10.10 ).
Normally, the pulvinar is the most hypointense nuclei of the
deep gray matter on T2W images. Positive pulvinar sign is a
highly sensitive sign of vCJD (Fig. 2.10.10 ).
Fig. 2.10.10. Axial FLAIR brains MR-illustration demonstrates
the bilateral posterior thalamic (pulvinar) hyperintense lesions
in vCJD (positive pulvinar sign)
100 Chapter 2 Neurology
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2.10
For Further Reading
1 . Guermazi A et al Neuroradiological fi ndings in vascular
dementia. Neuroradiology. 2007;49:1–22
2 . Arai K. MRI of progressive supranuclear palsy, corticobasal
degeneration and multiple system atrophy. J Neurol.
2006;253 Suppl 3:III/25–III/29
3 . RajMohan V et al The limbic system. Indian J Psychiatry.
2007;49:132–9
4 . Sy M-S et al Human prion diseases. Med Clin N Am. 2002;
86:551–71
5 . Almer G et al Fatal familial insomnia: a new Austrian fam-
ily. Brain. 1999;122:5–16
6 . Wang Y et al Report on the fi rst Chinese family with
Gerstmann-Str?sler-Scheinker disease manifesting the codon
102 mutation in the prion protein gene. Neuropathology.
2006;26:429–32
7 . Collie DA et al MRI of Creutzfeldt-Jakob disease: imaging
features and recommended MRI protocol. Clin Radiol.
2001;56:726–39
8 . Lucchelli F et al The case of lost Wilma: a clinical report of
Capgras delusion. Neurol Sci. 2007;28:188–95
9 . Uhlenbrock D et al The value of T1-weighted images in the
differentiation between MS, white matter lesions, and subcortical arteriosclerotic encephalopathy. Neuroradiology.
1989;31:203–12
10 . Wodarz R. Watershed infarctions and computed tomog-
raphy. A topographical study in cases with stenosis or
occlusion of the carotid artery. Neuroradiology. 1980;19:
245–8
11 . Bastos Leite AJ et al Thalamic lesions in vascular dementia:
low sensitivity of fl uid-attenuated inversion recovery
(FLAIR) imaging. Stroke. 2004;35:415–9
12 . Drago V et al What’s inside the art? The infl uence of fronto-
temporal dementia in art production. Neurology. 2006;67:
1285–7
13 . Clerici F et al Dementia with Lewy bodies with supranu-
clear gaze palsy: a matter of diagnosis. Neurol Sci. 2005; 26:
358–61
14 . Massano J et al Teaching neuroimage: MRI in multiple sys-
tem atrophy: “hot cross bun” sign and hyperintense rim
bordering the putamina. Neurology. 2008;71:e38
15 . Kwee RM et al Virchow-Robin spaces at MR imaging.
RadioGraph. 2007;27:1071–86
2.11 Huntington’s Disease 101
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2.11
Huntington’s Disease
Huntington’s disease (HD) is a chronic, progressive,
autosomal dominant, degenerative disease of the brain
characterized by motor, cognitive, and behavioral
abnormalities.
Patients with HD initially present between 30 and 50
years of age with chorea. Chorea is an involuntary,
jerking, dancing like movement of the distal limbs
(Huntington’s chorea). Chorea increases in severity in
the fi rst few years of life but eventually fades away
again to be replaced by bradykinesia and hypokinesia,
which are the real causes of motor disability in HD. In
advanced stages, patients develop dysarthria, dysphagia, and impairment of gait and balance.
Psychiatric symptoms can be seen in HD, including
depression, personality change, and anxiety. The suicide rate is high, especially in the early stage of the
disease.
There is no treatment for HD, and death usually occurs
10–15 years after manifestations of the symptoms.
Signs on CT and MRI
Both scans typically show bilateral symmetrical or asymmetri-
cal caudate nuclei atrophy causing ballooning of the frontal
horns (Boxcar-shaped frontal horns) (Fig. 2.11.1 ).
Brain cortical and white matter atrophy, especially the frontal
lobes, can be seen in advanced stages of the disease.
Diff erential Diagnoses and Related Diseases
Sydenham Chorea (rheumatic encephalitis) : is a manifes-
tation of a severe form of rheumatic fever. Sydenham chorea (SyC) is characterized clinically by involuntary and
uncoordinated movements, frequent falls, dysarthria, and
multiple weaknesses. There is female gender predominance, and mean age of 11.7 years at the onset of SyC.
The duration of SyC ranges from a week to 2 years with
average duration of 4 months. In rheumatic fever patients,
female gender and the presence of carditis can be the risk
factors for a longer duration of SyC. Interestingly, patients
with previous history of SyC develop psychiatric manifestations later in life, such as obsessive–compulsive disorder, major depressive disorders, or attention defi cits. On
Fig. 2.11.1. Axial FLAIR
MR-illustration ( a ) and
FLAIR MRI ( b ) of patients
with Huntington’s disease
(HD) show bilateral caudate
nucleus head atrophy and the
characteristic boxcar-shaped
frontal horns ( arrowheads )
102 Chapter 2 Neurology
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2.11
MRI, basal ganglia hyperintense lesions may be found in
patients with SyC.
For Further Reading
1 . Terrence CF et al Computed tomography in Huntington’s
disease. Neuroradiology. 1977;13:173–5
2 . Angelini L et al Tourettism as clinical presentation of
Huntington’s disease with onset in childhood. Ital J Neurol
Sci. 1998;19:383–5
3 . Craufurd D. Huntington’s disease. Prenat Diagn. 1996;16:
1237–45
4 . Faustino PC et al Clinical, laboratory, psychiatric and mag-
netic resonance fi ndings in patients with Sydenham chorea. Neuroradiology. 2003;45:456–62
2.12 Heat Stroke (Pancerebellar Syndrome) 103
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2.12
Heat Stroke (Pancerebellar Syndrome)
Heat stroke is a medical emergency characterized by a
core body temperature >40°C or more, hot dry skin,
and neurological disturbance.
Heat stroke may be environmental due to prolonged
exposure to sun heat with hydration, endogenous as in
runners during heavy military exercises (exertional
heat stroke), or a combination of both. Heat stroke may
also develop in other pathological conditions such as
infections, and neuroleptic malignant syndrome
(NMS). NMS is a rare complication of neuroleptic
clinically by hyperpyrexia, muscular rigidity, autonomic dysfunction, altered mental status, and elevation
of serum creatine phosphokinase (CK) levels. Patients
with NMS typically present with fever and muscle
rigidity 24–72 h after the start of treatment with neuroleptic medications; however, NMS may develop weeks
to months later. Cerebellar atrophy can be rarely caused
by NMS.
The most dramatic effect of heat stroke is observed
in the central nervous system, especially the cerebellum. Confusion, delirium, convulsions, myoglobinuria, stupor, or coma are seen in most cases. Downbeat
nystagmus, which is defi ned as a primary position nystagmus with rapid downward phase and slow upward
drift, may be seen with heat stroke cerebellar atrophy.
Direct thermal insult to the brain may lead to intraparenchymal hemorrhage or stroke.
The most common permanent neurological sequela
of heat stroke is pancerebellar syndrome , which is
characterized by cerebellar atrophy causing dysarthria,
irritability, ataxic gait, and poor concentrations.
Classically, the patient presents with cerebellar atrophy symptoms weeks to months after the initial heat
stroke attack. Cerebellar atrophy is caused by marked
degeneration of Purkinje cells with pyknotic nuclei,
chemolytic changes, and swollen dendrites. The cerebellar atrophy is indistinguishable from that seen in
various degenerative diseases affecting the cerebellum
(e.g., alcoholism), so history is very important.
Signs on Brain CT and MRI
The initial CT scan may be normal. Follow-up scans after
weeks or months may show bilateral cerebellar atrophy with
dilatation of the cerebellopontine angles cisterns and the
fourth ventricle. No changes in the cerebral hemispheres or
the brain stem are noticed classically.
Stroke or intraparenchymal hemorrhage may be seen in cases
of direct thermal insult.
Absence of increased intracranial pressure signs.
On postcontrast MRI, patchy enhancement of the cerebellum
hemispheres may be seen bilaterally.
Neuroleptic malignant syndrome : may show hyperintense T2
white matter lesions aff ecting the parieto-occipital area.
Rarely, cerebellar atrophy may be seen.
For Further Reading
1 . Yaqub BA et al Pancerebellar syndrome in heat stroke: clin-
ical course and CT scan fi ndings. Neuroradiology. 1987;
29:294–6
2 . McLaughlin CT et al MR imaging of heat stroke: external
capsule and thalamic T1 shortening and cerebellar injury.
AJNR Am J Neuroradiol. 2003;24:1372–5
3 . Deleu D et al Downbeat nystagmus following classical heat
stroke. Clin Neurol Neurosurg. 2005;108:102–4
4 . Manto M et al Cerebellar gait ataxia following neuroleptic
malignant syndrome. J Neurol. 1996;243:101–6
5 . Becker T et al MRI white matter hyperintensity in neuro-
leptic malignant syndrome (NMS) - a clue to pathogenesis?
J Neurol Transm [GenSect]. 1992;90:151–9
Chapter 3
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Pulmonology
CONTENTS
3.1 Pleural Diseases 106
3.2 Alveolar Lung Diseases 113
3.3 Atelectasis (Lung Collapse) 119
3.4 Sarcoidosis 123
3.5 Emphysema 131
3.6 Idiopathic Interstitial Pneumonias 135
3.7 Histiocytoses 140
3.8 Hemoptysis 146
J. A. Al-Tubaikh: Internal Medicine – An Illustrated Radiological Guide
DOI: 10.1007/978-3-642-03709-2_3, © Springer-Verlag Berlin Heidelberg 2010
105
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3.1
3.1
Pleural Diseases
The pleura are composed of two layers: parietal and
visceral layers, separated by a pleural space. The parietal pleuron is supplied by systemic vessels and drains
into the right atrium via the azygos, hemiazygos, and
internal mammary veins. The visceral pleuron is supplied by bronchial and pulmonary vessels and drains
into the pulmonary veins.
The pleural space normally contains interstitial
fl uid (1–5 mL) that is cleared by the parietal pleural
lymphatic vessels. There is no direct communication
between the visceral pleura lymphatics and the pleural
space.
The Pleura appear normally on radiographs only
when the X-ray beam is tangentially set on the fi lm.
On radiographs, the pleura appear as fi ssures and junctional lines. Fissures are made up of two layers of visceral pleura. The normal parietal pleuron is never
visualized on posteroanterior (PA) radiographs.
Different pathological conditions affecting the
pleura can be diagnosed with confi dence by PA chest
radiographs alone. This topic discusses the main pathological pleural conditions with their typical radiologic
manifestations.
Pleural Eff usion
Pleural effusion is a condition characterized by abnor-
mal fl uid collection between the parietal and visceral
pleura (excess pleural space fl uid). The pleural fl uid
can be water (edematous effusion), blood (hemotho-
rax), pus (empyema), tumor cells (malignant pleural
effusion), or lymph (chylothorx).
Pathologically, pleural effusion is divided into
serous or exudative according to the protein content
after lab analysis. Serous plural effusion contains little
protein content (<2.5 g/dL) and usually arises due to
systemic disease like cardiac failure, nephrotic syndrome, or liver failure. Exudative pleural effusion con-
tains high protein count (>2.5 g/dL) and usually arises
due to infl ammatory or infectious process like tuberculosis, malignancy, and acute pancreatitis.
Disruption of the thoracic duct due to lymphoma or
a tumor can cause lymphatic blockage and leakage
into the pleural space causing chylothorax. Malignant
effusion typically results from metastasizing of the
malignant cells into the pleural cavity via the parietal
pleura lymphatics, and it is often massive.
Bronchopleural fi stula is a condition characterized by
opening of a bronchus into the pleural space. It can
develop occasionally following thoracic surgery, infection, medical intervention, or malignancy. Broncho pleural
fi stula is seen in 2–3% of postpneumonectomy cases.
Signs on Chest Radiographs
Obliteration of the lateral costophrenic angle with a meniscus
like arc at the interface between the fl uid and the chest wall
in PA radiographs (Meniscus sign). (Fig. 3.1.1 )
Obliteration of the posterior costophrenic angle in lateral
radiographs (Fig. 3.1.1 ). This angle is more sensitive to plural
eff usion collection due to gravity eff ect. Up to 50 mL of fl uid is
necessary to obliterate the posterior costophrenic angle, and
200 mL is necessary to obliterate the lateral costophrenic angle.
Subpulmonic pleural eff usion ( SPE ) is a pleural eff usion that
occurs below the lungs at the diaphragmatic surface. SPE does
not obliterate the costophrenic angle, but it distorts the shape
of the diaphragmatic dome giving the impression of raised
hemidiaphragm. You can suspect SPE in the left lung when
the space between the gastric bubble and the lower lung
margins increases up to 3 cm instead of usual few millimeters.
Beside the raised hemidiaphragm, the lung appears to end
early on PA radiographs (Fig. 3.1.2 ).
Encysted ( loculated ) pleural eff usion is a localized encysted fl uid
at the fi ssures between lobes of the lung. It occurs usually at the
right lung’s minor fi ssure, and it has biconvex contour mimicking
a mass (Fig. 3.1.3 ). Very rarely, a benign form of mesothelioma
can grow along the major or minor fi ssures mimicking encysted
pleural eff usion, a condition known as “ pseudotumor .”
Para-pneumonic eff usion is an eff usion that develops adjacent
to pneumonias (empyema). Almost 30% of patients with
pneumonia develop pleural eff usion, and usually resolves
with antibiotic therapy.
Mediastinal pleural eff usion is a fl uid collection around the
mediastinum. It is an unusual condition, and when it occurs, it
forms silhouette sign along the mediastinal borders causing
mediastinal widening. Silhouette sign is a term used to
describe any opacity within the chest radiograph that
obliterates a mediastinal border.
3.1 Pleural Diseases 107
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Fig. 3.1.1. Posteroanterior
( a ) and lateral ( b ) chest
radiographs in two different
patients with pleural effusion
show meniscus sign with
right pleural effusion
obliterating the lateral
costo-phrenic angle
( arrowhead ) in ( a ), and
pleural effusion obliterating
the posterior costo-phrenic
angle in ( b ) ( arrow )
Fig. 3.1.2. Posteroanterior chest radiograph of a patient with
right subpulmonic pleural effusion (SPE) shows raised hemidiaphragm, and the lung seems to end early ( arrowhead )
Fig. 3.1.3. Posteroanterior
( a ) and lateral ( b ) chest
radiographs show right-sided
encysted pleural effusion
( arrowheads )
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3.1
Signs on US
Pleura eff usion appears as anechoic or hypoechoic collection that
lies between the echogenic line of the visceral pleura and lung
(Fig. 3.1.4 ).
Fig. 3.1.4. Transverse ultrasound image shows right-sided pleural effusion ( arrowhead ). The diaphragm can be visualized as a
hyperechoic line separating the right lung base from the liver
( arrow )
a
b
Signs on CT
Serous pleural eff usion is visualized as a crescent peripheral
area with CT water-density. Exodative eff usion can be
hyperdense.
Empyema characteristically demonstrates thickened parietal/
visceral pleura (e.g., > 2 mm) with eff usion in between
(split-pleura sign) (Fig. 3.1.5 ). Enhancement of the both
pleura occurs in 80–100% cases after contrast injection.
Multiple gas pockets within the empyema may be seen.
Bronchopleural fi stula: this condition occurs when a bronchus
opens into the pleural space due to lung parenchymal
destruction (e.g., pneumonia with empyema formation). It is
seen as pleural eff usion with air–fl uid level on radiographs or
HRCT (Fig. 3.1.6 ).
Fig. 3.1.5. Posteroanterior chest radiograph ( a ) and axial chest
CT ( b ) of a patient with huge left-sided empyema show split-
pleura sign in ( b ), with thickened, enhanced pleura with effusion
in between ( arrowheads )
Fig. 3.1.6. Axial chest CT shows huge right bronchopulmonary
fi stula
3.1 Pleural Diseases 109
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Diff erential Diagnoses and Related Diseases
Meigs’ syndrome is a disease characterized by
ascites, pleural effusion, and one of the following
ovarian tumors (fi broma, thecoma, granulose cell
tumor, or Brenner’s tumor). In contrast, Pseudo-
Meigs’ syndrome is defi ned as ascites, pleural effusion, and ovarian tumor other than the ones mentioned
previously. Absence of malignant cells from the
ascites or the pleural effusion is mandatory for the
diagnosis of Meigs’ syndrome. Typically, the ascites
and the pleural effusions resolve after tumor resection. Meigs’ syndrome often occurs in postmenopausal women.
Yellow nail syndrome is a rare disease characterized
by extremities lymphadema and thickened, slowlygrowing, yellowish-green nails that are excessively
curved from side to side (Fig. 3.1.7 ). The disease is
commonly accompanied by idiopathic pleural effusion, chronic bronchiectasis, chronic sinusitis, and
lymphadema of the face. Yellow nail syndrome may
be accompanied by rheumatoid arthritis or thyroid
disease. The disease is believed to be caused by hypoplasia, atresia, or varicosity of the lymphatics.
Pneumothorax
Pneumothorax is a condition characterized by presence of air between the parietal and visceral pleura.
There are three types of pneumothoraces:
Primary (spontaneous) pneumothorax : this type
occurs without a defi ned cause, and mainly seen in
young males who are tall, thin, and smokers. Primary
pneumothorax is attributed to rupture of subpleuritic
blebs at lung apices according to some investigators.
Secondary pneumothorax : this type occurs usually
after penetrating trauma, ruptured bulla, or an interventional thoracic procedure (e.g., lung mass biopsy).
Tension Pneumothorax : this type occurs when the air
collection within the subpleural space is large enough
to push the mediastinum to the other side, interfering
with blood circulation within the major vessels.
Up to 40% of pneumothoraces may not be detected by
chest radiographs. CT is 100% sensitive for detection of
pneumothoraces. When pneumothorax opens into the
mediastinum, a pneumomediastium develops. Pneumomediastinum is characterized by the presence of air
around the mediastinal structures.
Fig. 3.1.7. An illustration demonstrates the yellowish-green
nails of the yellow nail syndrome
Sign on Radiograph
A thin visceral pleural line is visible on radiographs. The line is
outlined by air with absence of the peripheral vasculature
laterally, and lung tissue with possible increased density due
to collapse, medially (Fig. 3.1.8 ). Lung apices are the best sites
checked for early detection of pneumothorax.
Deep sulcus sign: the costophrenic angle deepens at the site
of the pneumothorax (Fig. 3.1.9 ). It is seen in pneumothorax
with large air collection.
Tension pneumothorax is seen as complete collapse of the
lung and shift of the trachea and mediastinum to the
contralateral (other side) collapsed lung (Fig. 3.1.10 ).
Pitfall: a skin fold and underlying clothing can mimic a
pneumothorax (Fig. 3.1.11 ). Always correlate the radiological
fi ndings with the patient history and current status.
Pneumomediatinum is detected when the medistinual structures
are surrounded by dark radiolucent line of air (Fig. 3.1.12 ).
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